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  • 1.
    Afram, G.
    et al.
    Karolinska Inst, Med, Stockholm, Sweden..
    Watz, E.
    ONK PAT, Ctr Apheresis, Stockholm, Sweden..
    Remberger, M.
    ONK PAT, Ctr Allogene Stem Cell Transplantat, Immunol, Stockholm, Sweden..
    Axdorph-Nygell, U.
    ONK PAT, Ctr Apheresis, Stockholm, Sweden..
    Sundin, M.
    Karolinska Inst, Pediat Haematol, Stockholm, Sweden..
    Hagglund, Hans
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Mattsson, J.
    Karolinska Inst, Ctr Stem Cell Transplantat, Stockholm, Sweden..
    Uhlin, M.
    Karolinska Inst, Ctr Stem Cell Transplantat, Stockholm, Sweden..
    Extracorporeal photopheresis as treatment for moderate-severe chronic graft-versus-host disease2016In: Bone Marrow Transplantation, ISSN 0268-3369, E-ISSN 1476-5365, Vol. 51, p. S138-S138Article in journal (Other academic)
  • 2.
    Afram, Gabriel
    et al.
    Karolinska Univ Hosp Huddinge, Dept Hematol, Stockholm, Sweden.
    Perez Simon, Jose Antonio
    Univ Seville, CSIC, Hosp Univ Virgen del Rocio, Dept Hematol,Inst Biomed Sevilla IBIS, Seville, Spain.
    Remberger, Mats
    Karolinska Univ Hosp Huddinge, Ctr Allogene Stem Cell Transplantat, Stockholm, Sweden.
    Caballero-Velazquez, Teresa
    Univ Seville, CSIC, Hosp Univ Virgen del Rocio, Dept Hematol,Inst Biomed Sevilla IBIS, Seville, Spain.
    Martino, Rodrigo
    Hosp Santa Creu & Sant Pau, Dept Hematol, Barcelona, Spain.
    Luis Pinana, Jose
    Hosp Santa Creu & Sant Pau, Dept Hematol, Barcelona, Spain;Hosp Clin Univ, Dept Hematol, Valencia, Spain.
    Ringden, Olle
    Karolinska Univ Hosp Huddinge, Ctr Allogene Stem Cell Transplantat, Stockholm, Sweden.
    Esquirol, Albert
    Hosp Santa Creu & Sant Pau, Dept Hematol, Barcelona, Spain.
    Lopez-Corral, Lucia
    Hosp Univ Salamanca IBSAL, Dept Hematol, Salamanca, Spain.
    Garcia, Irene
    Hosp Santa Creu & Sant Pau, Dept Hematol, Barcelona, Spain.
    Lopez-Godino, Oriana
    Hosp Univ Salamanca IBSAL, Dept Hematol, Salamanca, Spain.
    Sierra, Jordi
    Hosp Santa Creu & Sant Pau, Dept Hematol, Barcelona, Spain.
    Caballero, Dolores
    Hosp Univ Salamanca IBSAL, Dept Hematol, Salamanca, Spain.
    Ljungman, Per
    Vazquez, Lourdes
    Hosp Univ Salamanca IBSAL, Dept Hematol, Salamanca, Spain.
    Hägglund, Hans
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Reduced intensity conditioning increases risk of severe cGVHD: identification of risk factors for cGVHD in a multicenter setting2018In: Medical Oncology, ISSN 1357-0560, E-ISSN 1559-131X, Vol. 35, no 6, article id 79Article in journal (Refereed)
    Abstract [en]

    Chronic graft-versus-host disease (cGVHD) remains a major cause of morbidity and mortality after allogeneic hematopoietic stem cell transplantation (HSCT). Aim is to identify risk factors for the development of cGVHD in a multicenter setting. Patients transplanted between 2000 and 2006 were analyzed (n = 820). Donors were HLA-identical siblings (57%), matched unrelated donors (30%), and HLA-A, B or DR antigen mismatched (13%). Reduced intensity conditioning (RIC) was given to 65% of patients. Overall incidence of cGVHD was 46% for patients surviving more than 100 days after HSCT (n = 747). Older patient age [HR 1.15, p < 0.001], prior acute GVHD [1.30, p = 0.024], and RIC [1.36, p = 0.028] increased overall cGVHD. In addition, RIC [4.85, p < 0.001], prior aGVHD [2.14, p = 0.001] and female donor to male recipient [1.80, p = 0.008] increased the risk of severe cGVHD. ATG had a protective effect for both overall [0.41, p < 0.001] and severe cGVHD [0.20, p < 0.001]. Relapse-free survival (RFS) was impaired in patients with severe cGVHD. RIC, prior aGVHD, and female-to-male donation increase the risk of severe cGVHD. ATG reduces the risk of all grades of cGVHD without hampering RFS. GVHD prophylaxis may be tailored according to the risk profile of patients.

  • 3.
    Afram, Gabriel
    et al.
    Karolinska Univ Lab, Hematol Ctr, Stockholm, Sweden;Karolinska Inst, Div Hematol, Dept Med, Stockholm, Sweden.
    Watz, Emma
    Karolinska Univ Lab, Dept Clin Immunol & Transfus Med, Stockholm, Sweden;Karolinska Inst, Div Transplantat Surg, Dept Clin Sci Intervent & Technol, Stockholm, Sweden.
    Remberger, Mats
    Karolinska Univ Hosp, Ctr Allogene Stem Cell Transplantat, Stockholm, Sweden;Karolinska Inst, Dept Oncol Pathol, Stockholm, Sweden.
    Nygell, Ulla Axdorph
    Karolinska Univ Lab, Hematol Ctr, Stockholm, Sweden;Karolinska Univ Lab, Dept Clin Immunol & Transfus Med, Stockholm, Sweden;Karolinska Inst, Div Transplantat Surg, Dept Clin Sci Intervent & Technol, Stockholm, Sweden.
    Sundin, Mikael
    Karolinska Univ Hosp, Astrid Lindgren Childrens Hosp, Hematol Immunol SCT Sect, Stockholm, Sweden;Karolinska Inst, Div Pediat, Dept Clin Sci Intervent & Technol, Stockholm, Sweden.
    Hägglund, Hans
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Mattsson, Jonas
    Karolinska Univ Hosp, Ctr Allogene Stem Cell Transplantat, Stockholm, Sweden;Karolinska Inst, Dept Oncol Pathol, Stockholm, Sweden.
    Uhlin, Michael
    Karolinska Univ Lab, Dept Clin Immunol & Transfus Med, Stockholm, Sweden;Karolinska Inst, Div Transplantat Surg, Dept Clin Sci Intervent & Technol, Stockholm, Sweden.
    Higher response rates in patients with severe chronic skin graft-versus-host disease treated with extracorporeal photopheresis2019In: Central European Journal of Immunology, ISSN 1426-3912, E-ISSN 1644-4124, Vol. 44, no 1, p. 84-91Article in journal (Refereed)
    Abstract [en]

    Introduction: Different forms of graft-versus-host disease (GVHD) remain a major cause of morbidity and mortality after allogeneic hematopoietic stem cell transplantation (HSCT). The prognosis for steroid-refractory chronic GVHD (cGVHD) remains poor. Our aim was to evaluate extracorporeal photopheresis (ECP) treatment in cGVHD patients with different organ involvement to detect subgroups of patients with the best response.

    Material and methods: Thirty-four patients who underwent HSCT and developed moderate (n = 7) or severe (n = 27) steroid-refractory or steroid-dependent cGVHD treated with ECP were included in the analysis. A matched cGVHD control patient group untreated with ECP was collected for comparison.

    Results: Compared to the control group and the stable/progressive disease (SD/PD) patients, individuals with complete/partial remission have higher overall survival and lower transplant-related mortality. Furthermore, patients with complete and partial remission (CR/PR) had significantly higher levels of albumin and platelets after ECP treatment compared to patients with stable or progressive cGVHD (SD/PD). Corticosteroid treatment and other immunosuppressive agents could successfully be tapered in the CR/PR group compared to the SD/PD patients. In this study patients with skin cGVHD are those with the highest rate of CR/PR after ECP treatment.

    Conclusions: Our results suggest that ECP treatment is safe and effective for patients with predominantly skin, oral and liver cGVHD.

  • 4.
    Ajeganova, Sofia
    et al.
    Karolinska Inst, Dept Med Huddinge, S-14186 Stockholm, Sweden..
    Tesfa, Daniel
    Karolinska Inst, Ctr Hematol & Regenerat Med, Dept Med Huddinge, S-14186 Stockholm, Sweden.;Roche AB, Med Affairs, S-10074 Stockholm, Sweden..
    Hägglund, Hans
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Fadeel, Bengt
    Karolinska Inst, Inst Environm Med, Unit Mol Toxicol, S-17177 Stockholm, Sweden..
    Vedin, Inger
    Karolinska Inst, Ctr Hematol & Regenerat Med, Dept Med Huddinge, S-14186 Stockholm, Sweden..
    Zignego, Anna Linda
    Univ Florence, Ctr Syst Manifestat Hepatitis Viruses, Dept Internal Med, I-50134 Florence, Italy..
    Palmblad, Jan
    Karolinska Inst, Ctr Hematol & Regenerat Med, Dept Med Huddinge, S-14186 Stockholm, Sweden..
    Effect of FCGR polymorphism on the occurrence of late-onset neutropenia and flare-free survival in rheumatic patients treated with rituximab2017In: Arthritis Research & Therapy, ISSN 1478-6354, E-ISSN 1478-6362, Vol. 19, article id 44Article in journal (Refereed)
    Abstract [en]

    Background: The causes and mechanisms of late-onset neutropenia (LON) following rituximab treatment in patients with rheumatic diseases are not known. In this study, we aimed to investigate the role of established Fc gamma receptor gene (FCGR) polymorphisms and B-cell-activating factor (BAFF) gene promoter polymorphisms for the development of LON and for the efficacy of rituximab in patients with rheumatic diseases. Methods: A single-center case-control retrospective study was nested in a cohort of 214 consecutive patients with rheumatic diseases treated with rituximab. Eleven patients presented with LON. Fifty non-LON control subjects were matched by diagnosis, age, sex, and treatments. Single-nucleotide polymorphisms of FCGR (FCGR2A 131H/R, FCGR2B 232I/T, FCGR3A 158V/F) and BAFF promoter polymorphism -871C/T were analyzed with polymerase chain reaction-based techniques, and serum immunoglobulin M (IgM) and BAFF levels were analyzed by enzyme-linked immunosorbent assay. Flare-free survival was related to LON occurrence and polymorphisms. Results: The FCGR3A V allele, but not other FCGR polymorphisms, correlated with the occurrence of LON; each V allele conferred a fourfold increased OR for LON (p = 0.017). FCGR3A 158V/V and presentation with LON were associated with a longer flare-free survival (p = 0.023 and p = 0.031, respectively). FCGR3A 158V/V was related to lower IgM levels (p = 0.016). Serum BAFF levels showed no relationship with LON and BAFF -871C/T promoter polymorphism. There was a tendency toward longer flare-free survival in patients with the BAFF -871T/T allotype compared with the C/T or C/C allotypes (p = 0.096). Conclusions: The results of the present study suggest that presentation with LON may be a result of the intrinsic efficacy of rituximab in patients with rheumatic diseases. LON could indicate a longer biological and therapeutic activity of rituximab modulated by a certain genotypic polymorphism: the high-affinity FCGR3A V allele. This genotype and the occurrence of LON are both related to longer flare-free survival, suggestive of common mechanisms for LON and duration of response to rituximab. The role of the BAFF -871C/T promoter polymorphism in LON occurrence is unclear.

  • 5. Broesby-Olsen, Sigurd
    et al.
    Dybedal, Ingunn
    Gülen, Theo
    Kielsgaard Kristensen, Thomas
    Boe Møller, Michael
    Ackermann, Leena
    Sääf, Maria
    Karlsson, Maria A
    Agertoft, Lone
    Brixen, Kim
    Hermann, Pernille
    Stylianou, Eva
    Mortz, Charlotte G
    Torfing, Trine
    Havelund, Troels
    Sander, Birgitta
    Bergström, Anna
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Dermatology and Venereology.
    Bendix, Marie
    Garvey, Lene H
    Weis Bjerrum, Ole
    Valent, Peter
    Bindslev-Jensen, Carsten
    Nilsson, Gunnar
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Vestergaard, Hanne
    Hägglund, Hans
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Multidisciplinary Management of Mastocytosis: Nordic Expert Group Consensus2016In: Acta Dermato-Venereologica, ISSN 0001-5555, E-ISSN 1651-2057, Vol. 96, no 5Article in journal (Refereed)
    Abstract [en]

    Mastocytosis is a heterogeneous group of diseases defined by an increased number and accumulation of mast cells, and often also by signs and symptoms of mast cell activation. Disease subtypes range from indolent to rare aggressive forms. Mastocytosis affects people of all ages and has been considered rare; however, it is probably underdiagnosed with potential severe implications. Diagnosis can be challenging and symptoms may be complex and involve multiple organ-systems. In general it is advised that patients should be referred to centres with experience in the disease offering an individualized, multidisciplinary approach. We present here consensus recommendations from a Nordic expert group for the diagnosis and general management of patients with mastocytosis.

  • 6.
    Burman, Joachim
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Neurology.
    Tolf, Andreas
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Neurology.
    Hägglund, Hans
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Askmark, Håkan
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Neurology.
    Autologous haematopoietic stem cell transplantation for neurological diseases2018In: Journal of Neurology, Neurosurgery and Psychiatry, ISSN 0022-3050, E-ISSN 1468-330X, Vol. 89, no 2, p. 147-155Article, review/survey (Refereed)
    Abstract [en]

    Neuroinflammatory diseases such as multiple sclerosis, neuromyelitis optica, chronic inflammatory demyelinating polyneuropathy and myasthenia gravis are leading causes of physical disability in people of working age. In the last decades significant therapeutic advances have been made that can ameliorate the disease course. Nevertheless, many affected will continue to deteriorate despite treatment, and the costs associated with disease-modifying drugs constitute a significant fiscal burden on healthcare in developed countries. Autologous haematopoietic stem cell transplantation is a treatment approach that aims to ameliorate and to terminate disease activity. The erroneous immune system is eradicated using cytotoxic drugs, and with the aid of haematopoietic stem cells a new immune system is rebuilt. As of today, more than 1000 patients with multiple sclerosis have been treated with this procedure. Available data suggest that autologous haematopoietic stem cell transplantation is superior to conventional treatment in terms of efficacy with an acceptable safety profile. A smaller number of patients with other neuroinflammatory conditions have been treated with promising results. Herein, current data on clinical effect and safety of autologous haematopoietic stem cell transplantation for neurological disease are reviewed.

  • 7. Gülen, T
    et al.
    Hägglund, Hans
    astocytosis Centre Karolinska, Karolinska University Hospital, Karolinska Institutet, Stockholm, Sweden.
    Dahlén, B
    Nilsson, G
    High prevalence of anaphylaxis in patients with systemic mastocytosis: a single-centre experience2014In: Clinical and Experimental Allergy, ISSN 0954-7894, E-ISSN 1365-2222, Vol. 44, no 1, p. 121-129Article in journal (Refereed)
    Abstract [en]

    BACKGROUND: Systemic mastocytosis (SM) is a clonal mast cells disorder characterized by the proliferation, accumulation and activation of mast cells in extracutaneous tissues. The clinical picture is heterogeneous and may range from asymptomatic to potentially fatal anaphylactic reactions due to excessive mast cell mediator release.

    OBJECTIVE: The aim of this study was to investigate the prevalence and trigger factors of anaphylactic reactions among adult SM patients. We also explored the clinical spectrum of mast cell mediator-related symptoms in patients with SM.

    METHODS: This descriptive study was performed among 84 consecutive adult (≥ 18 years) patients those were diagnosed with SM according to WHO criteria. Sixty-six of the patients also underwent a comprehensive allergy work-up.

    RESULTS: Sixty of 84 patients with SM (71%) had bone marrow mast cell aggregates and fulfilled the major criteria for SM and 76 patients (91%) had indolent disease. Simultaneous occurrence of cutaneous mastocytosis was observed in 59 patients (70%). Thirty-six patients (43%) had had at least one episode of an anaphylactic reaction. The clinical courses of the reactions were usually severe and patients often presented with syncope attacks (72%). Most patients reacted after hymenoptera venom stings (19/36; 53%). In 39% (14/36), a clear aetiology could not be determined. While males and females were equally frequent among the patients with SM, anaphylaxis patients showed a male predominance (61%). Anaphylactic reactions occurred more frequently in patients without cutaneous engagement. The rate of allergy sensitization was significantly higher in SM patients with anaphylaxis as compared with non-anaphylaxis SM patients, 70% vs. 23%, respectively (P = 0.0002).

    CONCLUSIONS AND CLINICAL RELEVANCE: Anaphylaxis is more prevalent in patients with SM, predominantly in patients with atopic SM. Hymenoptera venom-induced and idiopathic anaphylaxis were the most frequent elicitors. Our findings implicate that all mastocytosis patients with anaphylaxis should undergo detailed allergological assessment before considering treatment and preventive measures.

  • 8. Gülen, T
    et al.
    Hägglund, Hans
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Dahlén, B
    Nilsson, Gunnar
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Mastocytosis: the puzzling clinical spectrum and challenging diagnostic aspects of an enigmatic disease2016In: Journal of Internal Medicine, ISSN 0954-6820, E-ISSN 1365-2796, Vol. 279, no 3, p. 211-228Article, review/survey (Refereed)
    Abstract [en]

    Mastocytosis is a complex disorder characterized by the accumulation of abnormal mast cells (MC) in the skin, bone marrow and/or other visceral organs. The clinical manifestations result from MC-derived mediators and, less frequently, from destructive infiltration of MCs. Patients suffer from a variety of symptoms including pruritus, flushing and life-threatening anaphylaxis. Whilst mastocytosis is likely to be suspected in a patient with typical skin lesions [i.e. urticaria pigmentosa (UP)], the absence of cutaneous signs does not rule out the diagnosis of this disease. Mastocytosis should be suspected in cases of recurrent, unexplained or severe insect-induced anaphylaxis or symptoms of MC degranulation without true allergy. In rare cases, unexplained osteoporosis or unexplained haematological abnormalities can be underlying feature of mastocytosis, particularly when these conditions are associated with elevated baseline serum tryptase levels. The diagnosis is based on the World Health Organization criteria, in which the tryptase level, histopathological and immunophenotypic evaluation of MCs and molecular analysis are crucial. A somatic KIT mutation, the most common of which is D816V, is usually detectable in MCs and their progenitors. Once a diagnosis of systemic mastocytosis (SM) is made, it is mandatory to assess the burden of the disease, its activity, subtype and prognosis, and the appropriate therapy. Mastocytosis comprises seven different categories that range from indolent forms, such as cutaneous and indolent SM, to progressive forms, such as aggressive SM and MC leukaemia. Although prognosis is good in patients with indolent forms of the disease, patients with advanced categories have a poor prognosis.

  • 9. Gülen, T
    et al.
    Hägglund, Hans
    Mastocytosis Centre Karolinska, Karolinska University Hospital and Karolinska Institutet, Stockholm.
    Sander, B
    Dahlén, B
    Nilsson, Gunnar
    Department of Medicine Solna, Clinical Immunology and Allergy Research Unit, Karolinska Institutet, Stockholm.
    The presence of mast cell clonality in patients with unexplained anaphylaxis2014In: Clinical and Experimental Allergy, ISSN 0954-7894, E-ISSN 1365-2222, Vol. 44, no 9, p. 1179-1187Article in journal (Refereed)
    Abstract [en]

    BACKGROUND: The mechanisms by which mast cells in patients with unexplained anaphylaxis (UEA) are triggered remain elusive. Onset of episodes is unpredictable and often recurrent. The substantial overlap between the clinical manifestations of UEA and clonal mast cell disorders (CMD) suggests an association between these rare disorders. The two forms of CMD characterized to date are systemic mastocytosis (SM) and monoclonal mast cell activation syndrome (MMAS).

    OBJECTIVE: To examine the hypothesis that the pathogenesis of UEA reflects the presence of aberrant subpopulations of mast cells.

    METHODS: Thirty (14 men, 16 women) patients (≥ 18 years) suffering from UEA and with no signs of cutaneous mastocytosis were recruited. Patients underwent an initial complete allergy work-up to confirm the diagnosis of UEA. Level of baseline serum tryptase (sBT) and total IgE were determined. In addition, a bone marrow examination was performed on all 30 patients to investigate possible underlying CMD.

    RESULTS: Fourteen (47%) of our cases (nine men, five women) were diagnosed with CMD: 10 with SM and four with MMAS. Four of the 10 patients with SM had mast cell aggregates in their bone marrow. All patients with SM exhibited a sBT level > 11.4 ng/mL, whereas this level was elevated in only two of those with MMAS and four with UAE but not diagnosed with CMD. Total IgE levels were lower in the group of patients with CMD (P < 0.03).

    CONCLUSION AND CLINICAL RELEVANCE: The pathogenic mechanism underlying UEA could be explained by the presence of immunophenotypically aberrant mast cells with clonal markers in 47% of our subjects, indicating that clonal mast cell disorders are present in a substantial subset of these patients. Thus, the presence of CMD should be considered in patients with UEA if they have an elevated level of sBT (≥ 11.4 ng/mL) and cardiovascular symptoms such as syncope.

  • 10. Gülen, Theo
    et al.
    Hägglund, Hans
    Department of Hematology, Karolinska University Hospital Huddinge, Stockholm, Sweden.
    Dahlén, Sven-Erik
    Sander, Birgitta
    Dahlén, Barbro
    Nilsson, Gunnar
    Clinical Immunology and Allergy Research Unit, Department of Medicine Solna, Karolinska Institutet, Stockholm, Sweden.
    Flushing, fatigue, and recurrent anaphylaxis: a delayed diagnosis of mastocytosis2014In: The Lancet, ISSN 0140-6736, E-ISSN 1474-547X, Vol. 383, no 9928, p. 1608-Article in journal (Refereed)
  • 11.
    Hägglund, Hans
    et al.
    Department of Hematology, Karolinska University Hospital Huddinge.
    Sander, Birgitta
    Gülen, Theo
    Lindelöf, Bernt
    Nilsson, Gunnar
    Clinical Immunology and Allergy Unit, Department of Medicine, Karolinska Institutet and Karolinska University Hospital Solna, SE-171 76 Stockholm, and Mastocytosis Center Karolinska, Karolinska University Hospital and Karolinska Institutet, Stockholm, Sweden.
    Increased risk of malignant melanoma in patients with systemic mastocytosis?2014In: Acta Dermato-Venereologica, ISSN 0001-5555, E-ISSN 1651-2057, Vol. 94, no 5, p. 583-584Article in journal (Refereed)
    Abstract [en]

    Mastocytosis, a group of rare disorders that occur in bothchildren and adults, is characterised by abnormal growthand pathological accumulation of mast cells in one or moreorgans, most commonly the skin (1). Urticaria pigmentosa(UP) is the most common cutaneous variant. In cases ofextracutaneous involvement, systemic mastocytosis (SM)can be diagnosed on the basis of the criteria formulatedby the WHO. The course of SM in most patients (90%) isindolent, with more aggressive presentation in only a few.The incidence of cutaneous melanoma is increasingand although this malignancy and mastocytosis originatefrom 2 different types of cells (melanocytes from theneural crest and mast cells from haematopoetic stem cells,respectively) they share certain similarities, includingexpression of the transcription factors MITF and STAT3,and dependence of the growth factor receptor KIT and itsligand stem cell factor for their growth and development(2, 3). We have found 5 published case reports that suggesta relationship between these 2 pathologies. In the first,published in 1979, a patient with nodular mastocytosis de-veloped both melanocytoma and mastocytoma (4). In thesecond, UP and SM preceded a metastatic melanoma (5)and the third involved combined mastocytoma-junctionalnaevus (6). In the fourth case, malignant melanoma wasdiagnosed prior to SM (7). And finally, a patient withtelangiectasia macularis eruptive perstans (TEMP), arare form of cutaneous mastocytosis, was found to havea malignant melanoma (8).Here, we describe our 4 additional cases and discusspossible associations between these 2 diseases.

  • 12. Håkansson, Irene
    et al.
    Sandstedt, Anna
    Lundin, Fredrik
    Askmark, Håkan
    Pirskanen, Ritva
    Carlson, Kristina
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Piehl, Fredrik
    Hägglund, Hans
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Successful autologous haematopoietic stem cell transplantation for refractory myasthenia gravis - a case report2017In: Neuromuscular Disorders, ISSN 0960-8966, E-ISSN 1873-2364, Vol. 27, no 1, p. 90-93Article in journal (Refereed)
    Abstract [en]

    Myasthenia gravis (MG) is an autoimmune disease, with immune reactivity against the post-synaptic endplate of the neuromuscular junction. Apart from symptomatic treatment with choline esterase blockers, many patients also require immunomodulatory treatment. Despite existing treatment options, some patients are treatment refractory. We describe a patient with severe MG refractory to corticosteroids, four oral immunosuppressants, cyclophosphamide, rituximab and bortezomib who was treated with autologous haematopoietic stem cell transplantation. Two years after this, the patient has significantly improved in objective tests and in quality of life and leads an active life. Diplopia is her only remaining symptom and she is completely free of medication for MG. We believe that autologous haematopoietic stem cell transplantation can be an effective therapeutic option for carefully selected cases of severe, treatment refractory MG.

  • 13.
    Källström, Miikka
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences.
    Soveri, Inga
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Renal Medicine.
    Oldgren, Jonas
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Medicinska och farmaceutiska vetenskapsområdet, centrumbildningar mm, UCR-Uppsala Clinical Research Center. Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Cardiology.
    Laukkanen, Jari
    Faculty of Sport and Health Sciences and Central Finland Health Care District, Department of Internal Medicine, University of Jyväskylä, Jyväskylä, Finland; Institute of Public Health and Clinical Nutrition, University of Eastern Finland, Kuopio, Finland.
    Ichiki, Tomoko
    Cardiovascular Medicine, Mayo Clinic and Cardiology, International University of Health and Welfare, Rochester, Minnesota; Cardiology, International University of Health and Welfare, Narita, Japan.
    Tei, Chuwa
    Waon Therapy Research Institute, Tokyo, Japan.
    Timmerman, Mark
    Department of Family Medicine, River Valley Clinic, Spring Green, Wisconsin.
    Berglund, Lars
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Medicinska och farmaceutiska vetenskapsområdet, centrumbildningar mm, UCR-Uppsala Clinical Research Center. Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Public Health and Caring Sciences, Geriatrics.
    Hägglund, Hans
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Effects of sauna bath on heart failure: A systematic review and meta-analysis2018In: Clinical Cardiology, ISSN 0160-9289, E-ISSN 1932-8737, Vol. 41, no 11, p. 1491-1501Article, review/survey (Refereed)
    Abstract [en]

    BACKGROUND:

    Sauna bath has potential as a lifestyle treatment modality for heart failure (HF). It is important to analyze the current evidence to help suggest paths of future study and potential for clinical application.

    HYPOTHESIS:

    Sauna bath has a positive effect on HF patients.

    METHODS:

    PubMed, Cochrane Library, and CINAHL databases were searched to identify randomized and nonrandomized controlled studies to compare effects of sauna bath with no sauna bath. Studies were searched for both infrared sauna bath and Finnish sauna bath. The strength of evidence was rated using a modified GRADE approach. Out of 1444 studies, nine met the inclusion criteria and were included in this review. Seven of these nine studies were included in the meta-analysis. Only studies with infrared sauna bath met the inclusion criteria.

    RESULTS:

    In the meta-analysis, exposure to an infrared sauna bath in 60°C for 15 minutes, followed by a 30-minute rest in warm environment, five times a week for 2 to 4 weeks, was associated with a significant reduction in B-type natriuretic peptide, cardiothoracic ratio, and an improvement in left-ventricular ejection fraction. There was no significant effect on left-ventricular end-diastolic diameter, left atrial diameter, systolic blood pressure, or diastolic blood pressure. The strength of evidence varied from moderate to insufficient.

    CONCLUSION:

    Infrared sauna bath was associated with short-term improvement in cardiac function. More evidence is needed about long-term effects of sauna bath and the effects of a Finnish sauna on cardiovascular health among patients with HF or other cardiovascular diseases.

  • 14. Lyberg, Katarina
    et al.
    Ali, Hani Abdulkadir
    Grootens, Jennine
    Kjellander, Matilda
    Tirfing, Malin
    Arock, Michel
    Hägglund, Hans
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Nilsson, Gunnar
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Ungerstedt, Johanna
    Histone deacetylase inhibitor SAHA mediates mast cell death and epigenetic silencing of constitutively active D816V KIT in systemic mastocytosis.2017In: OncoTarget, ISSN 1949-2553, E-ISSN 1949-2553, Vol. 8, no 6, p. 9647-9659Article in journal (Refereed)
    Abstract [en]

    Systemic mastocytosis (SM) is a clonal bone marrow disorder, where therapeutical options are limited. Over 90% of the patients carry the D816V point mutation in the KIT receptor that renders this receptor constitutively active. We assessed the sensitivity of primary mast cells (MC) and mast cell lines HMC1.2 (D816V mutated), ROSA (KIT WT) and ROSA (KIT D816V) cells to histone deacetylase inhibitor (HDACi) treatment. We found that of four HDACi, suberoyl anilide hydroxamic acid (SAHA) was the most effective in killing mutated MC. SAHA downregulated KIT, followed by major MC apoptosis. Primary SM patient MC cultured ex vivo were even more sensitive to SAHA than HMC1.2 cells, whereas primary MC from healthy subjects were less affected. There was a correlation between cell death and SM disease severity, where cell death was more pronounced in the case of aggressive SM, with almost 100% cell death among MC from the mast cell leukemia patient. Additionally, ROSA (KIT D816V) was more affected by HDACi than ROSA (KIT WT) cells. Using ChIP qPCR, we found that the level of active chromatin mark H3K18ac/H3 decreased significantly in the KIT region. This epigenetic silencing was seen only in the KIT region and not in control genes upstream and downstream of KIT, indicating that the downregulation of KIT is exerted by specific epigenetic silencing. In conclusion, KIT D816V mutation sensitized MC to HDACi mediated killing, and SAHA may be of value as specific treatment for SM, although the specific mechanism of action requires further investigation.

  • 15.
    Machaczka, M.
    et al.
    Karolinska Inst, Dept Med Huddinge, Stockholm, Sweden.;Karolinska Univ Hosp, Hematol Ctr Karolinska, Stockholm, Sweden.
    Hägglund, Hans
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology. Karolinska Inst, Dept Med Huddinge, Uppsala, Sweden.
    Staver, E.
    Karolinska Inst, Dept Med Huddinge, Stockholm, Sweden.
    Joks, M.
    Poznan Univ Med Sci, Dept Hematol & Bone Marrow Transplantat, Poznan, Poland.
    Hassan, M.
    Karolinska Inst, Dept Lab Med, Clin Res Ctr, Expt Canc Med, Stockholm, Sweden; Karolinska Univ Hosp, Dept Clin Res Ctr, Stockholm, Sweden.
    Wahlin, B. E.
    Karolinska Inst, Dept Med Huddinge, Stockholm, Sweden; Karolinska Univ Hosp, Hematol Ctr Karolinska, Stockholm, Sweden.
    Nygell, U. Axdorph
    Karolinska Inst, Dept Oncol & Pathol, Stockholm, Sweden; Karolinska Univ Hosp, Dept Clin Immunol & Transfus Med, Stockholm, Sweden.
    G-CSF mobilized peripheral blood stem cell collection for allogeneic transplantation in healthy donors: Analysis Of Factors Affecting Yield2017In: Bone Marrow Transplantation, ISSN 0268-3369, E-ISSN 1476-5365, Vol. 52, no Supplement: 1, p. S139-S139Article in journal (Other academic)
  • 16.
    Machaczka, Maciej
    et al.
    Karolinska Univ Hosp, Karolinska Inst, Dept Med Huddinge, Stockholm, Sweden.;Karolinska Univ Hosp, Hematol Ctr Karolinska, M54, SE-14186 Stockholm, Sweden..
    Hägglund, Hans
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology. Karolinska Univ Hosp, Karolinska Inst, Dept Med Huddinge, Stockholm, Sweden.;Karolinska Univ Hosp, Hematol Ctr Karolinska, Stockholm, Sweden..
    Staver, Emma
    Karolinska Univ Hosp, Karolinska Inst, Dept Med Huddinge, Stockholm, Sweden.;Karolinska Univ Hosp, Hematol Ctr Karolinska, M54, SE-14186 Stockholm, Sweden..
    Joks, Monika
    Poznan Univ Med Sci, Dept Hematol & Bone Marrow Transplantat, Poznan, Poland..
    Hassan, Moustapha
    Karolinska Univ Hosp, Karolinska Inst, Dept Lab Med, Expt Canc Med,Clin Res Ctr, Stockholm, Sweden.;Karolinska Univ Hosp, Clin Res Ctr, Stockholm, Sweden..
    Wahlin, Bjorn Engelbrekt
    Karolinska Univ Hosp, Karolinska Inst, Dept Med Huddinge, Stockholm, Sweden.;Karolinska Univ Hosp, Hematol Ctr Karolinska, M54, SE-14186 Stockholm, Sweden..
    Nygell, Ulla Axdorph
    Karolinska Univ Hosp, Karolinska Inst, Dept Med Huddinge, Stockholm, Sweden.;Karolinska Univ Hosp, Hematol Ctr Karolinska, M54, SE-14186 Stockholm, Sweden.;Karolinska Univ Hosp, Karolinska Inst, Dept Oncol & Pathol, Stockholm, Sweden.;Karolinska Univ Hosp, Dept Clin Immunol & Transfus Med, Stockholm, Sweden..
    G-CSF mobilized peripheral blood stem cell collection for allogeneic transplantation in healthy donors: Analysis of factors affecting yield2017In: Journal of clinical apheresis, ISSN 0733-2459, E-ISSN 1098-1101, Vol. 32, no 6, p. 384-391Article in journal (Refereed)
    Abstract [en]

    Mobilized PBSC are the main source for allogeneic HSCT. We aimed to evaluate factors that affect CD34+ cell yield including the donor's age, gender, BSA, processed blood volume and the method of G-CSF dose calculation. Data from 170 healthy donors were analyzed. The concentration of CD34+ cells in the peripheral blood (PB) and the processed volume of blood were significantly correlated to CD34+ cells yield (P<.00005 and P<.001, respectively). The G-CSF dose per m(2) was significantly correlated to the concentration of CD34+ cells in the PB (P=.0003) and in the product (P=.01). Smaller BSA and less processed volume were found among female donors, who were given lesser G-CSF dose perm(2), and showed lower yield compared to men. However, multivariate analysis of the yield showed that only the concentration of CD34+ cells in the PB and the processed volume remained independent significant.

  • 17.
    Pahnke, Simon
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Egeland, Torstein
    Univ Oslo, Oslo Univ Hosp, Inst Immunol, Rikshosp, Oslo, Norway;Univ Oslo, Inst Clin Med, Oslo, Norway.
    Halter, Jorg
    Univ Hosp Basel, Dept Hematol, Basel, Switzerland;Univ Basel, Basel, Switzerland.
    Hägglund, Hans
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Shaw, Bronwen E.
    Med Coll Wisconsin, Ctr Int Blood & Bone Marrow Transplant Res, Milwaukee, WI 53226 USA.
    Woolfrey, Ann E.
    Fred Hutchinson Canc Res Ctr, Div Clin Res, 1124 Columbia St, Seattle, WA 98104 USA;Univ Washington, Dept Med, Seattle, WA USA.
    Szer, Jeff
    Royal Melbourne Hosp, Victorian Comprehens Canc Ctr, Integrated Haematol Dept, Melbourne, Vic, Australia.
    Current use of biosimilar G-CSF for haematopoietic stem cell mobilisation2019In: Bone Marrow Transplantation, ISSN 0268-3369, E-ISSN 1476-5365, Vol. 54, no 6, p. 858-866Article in journal (Refereed)
    Abstract [en]

    Despite biosimilars of the granulocyte-colony stimulating factor (G-CSF) filgrastim being approved by the European Medicines Agency since 2008, there is still some debate regarding their use in related and unrelated healthy haematopoietic stem cell donors. We present a review of published experiences using biosimilar filgrastim for healthy donor mobilisation as well as the results of a survey by the World Marrow Donor Association (WMDA) of its current use by register-associated transplant and collection centres for both related and unrelated donors. A total of 1287 healthy donors and volunteers are included in the reviewed studies. The pharmacokinetics and pharmacodynamics studies show a high degree of similarity to the reference product Neupogen. Mobilisation of CD34 + cells as well as reported adverse events are also found to be comparable, although there is still a lack of long-term follow up for both Neupogen and filgrastim biosimilars. No evidence is found of a higher risk of filgrastim antibody formation using filgrastim biosimilars. Based on this increased experience, the WMDA therefore recommend that Stem Cell Donor Registries can use filgrastim biosimilars for the mobilisation of peripheral blood progenitor cells in healthy donors, provided that they are approved by national and/or regional agencies.

  • 18.
    Pahnke, Simon
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Larfors, Gunnar
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Axdorph-Nygell, Ulla
    Karolinska University Hospital, Stockholm, Sweden .
    Fischer-Nielsen, Anne
    Copenhagen University Hospital, Copenhagen, Denmark.
    Haastrup, Eva
    Copenhagen University Hospital, Copenhagen, Denmark.
    Heldal, Dag
    Oslo University Hospital, Oslo, Norway.
    Itälä-Remes, Maija
    Turku University Hospital, Turku, Finland.
    Johansson, Jan-Erik
    University of Gothenburg, Gothenburg, Sweden.
    Kauppila, Marjut
    Turku University Hospital, Turku, Finland.
    Lenhoff, Stig
    Skåne University Hospital, Lund, Sweden.
    Ljungman, Per
    Karolinska Institutet, Stockholm, Sweden.
    Niittyvuopio, Riita
    Helsinki University Hospital, Helsinki, Finland.
    Sandstedt, Anna
    Linköping University Hospital, Linköping, Sweden.
    Hägglund, Hans
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Short-term side effects and attitudes towards second donation: A comparison of related and unrelated haematopoietic stem cell donors2018In: Journal of clinical apheresis, ISSN 0733-2459, E-ISSN 1098-1101, Vol. 33, no 3, p. 226-235Article in journal (Refereed)
    Abstract [en]

    The Nordic Register of Haematopoietic Stem Cell Donors (NRHSD) has registered related and unrelated donors from 10 transplant centres in Sweden, Norway, Finland and Denmark since 1998. We present a prospective, observational study of 1,957 donors, focusing mainly on the differences between related and unrelated donors. Related donors are reported to have more comorbidities, but similar side effects compared with unrelated donors. Side effects after BM or PBSC donation are generally of short duration and in this study no deaths, myocardial infarctions, splenic ruptures, or thromboembolic events are reported. Interestingly, related donors express more hesitancy towards donating again when asked 1 month after donation.

  • 19. Ruutu, Tapani
    et al.
    Juvonen, Eeva
    Remberger, Mats
    Remes, Kari
    Volin, Liisa
    Mattsson, Jonas
    Nihtinen, Anne
    Hägglund, Hans
    Centre for Allogeneic Stem Cell Transplantation, Departments of Clinical Immunology and Medicine, Karolinska Hospital, Huddinge University Hospital, Huddinge, Sweden.
    Ringdén, Olle
    Improved survival with ursodeoxycholic acid prophylaxis in allogeneic stem cell transplantation: long-term follow-up of a randomized study2014In: Biology of blood and marrow transplantation, ISSN 1083-8791, E-ISSN 1523-6536, Vol. 20, no 1, p. 135-138Article in journal (Refereed)
    Abstract [en]

    We report the long-term results of a prospective randomized study on the use of ursodeoxycholic acid (UDCA) for prevention of hepatic complications after allogeneic stem cell transplantation. Two hundred forty-two patients, 232 with malignant disease, were randomized to receive (n = 123) or not to receive (n = 119) UDCA from the beginning of the conditioning until 90 days post-transplantation. The results were reported after 1-year follow-up. UDCA administration reduced significantly the proportion of patients developing high serum bilirubin levels as well as the incidence of severe acute graft-versus-host disease (GVHD), liver GVHD, and intestinal GVHD. In the UDCA prophylaxis group, nonrelapse mortality (NRM) was lower and overall survival better than in the control group. After a 10-year follow-up, the difference in the survival and NRM in favor of the UDCA-treated group, seen at 1 year, was maintained (survival 48% versus 38%, P = .037; NRM 28% versus 41%, P = .01). A landmark analysis in patients surviving at 1 year post-transplantation showed no significant differences between the study groups in the long-term follow-up in chronic GVHD, relapse rate, NRM, disease-free survival, or overall survival. These long-term results continue to support the useful role of UDCA in the prevention of transplant-related complications in allogeneic transplantation.

  • 20.
    Ustun, Celalettin
    et al.
    Univ Minnesota, Div Hematol Oncol & Transplantat, Minneapolis, MN 55455 USA..
    Gotlib, Jason
    Stanford Univ, Div Hematol, Stanford, CA USA..
    Popat, Uday
    Univ Texas MD Anderson Canc Ctr, Dept Stem Cell Transplantat, Houston, TX USA..
    Artz, Andrew
    Univ Chicago, Dept Med, Hematol Oncol Sect, Chicago, IL USA..
    Litzow, Mark
    Mayo Clin, Dept Internal Med, Div Hematol, Minneapolis, MN USA..
    Reiter, Andreas
    Univ Med Ctr Mannheim, Dept Hematol & Oncol, Mannheim, Germany..
    Nakamura, Ryotaro
    City Hope Med Ctr, Dept Hematol Hematopoiet Cell Transplantat, Duarte, CA USA..
    Kluin-Nelemans, Hanneke C.
    Univ Groningen, Univ Med Ctr Groningen, Dept Hematol, Groningen, Netherlands..
    Verstovsek, Srdan
    Univ Texas MD Anderson Canc Ctr, Dept Leukemia, Houston, TX USA..
    Gajewskil, James
    Oregon Hlth & Sci Univ, Dept Hematol, Portland, OR USA..
    Perales, Miguel-Angel
    Mem Sloan Kettering Canc Ctr, Dept Med, Div Hematol Oncol, New York, NY USA..
    George, Tracy
    Univ New Mexico, Dept Pathol, Albuquerque, NM USA..
    Shore, Tsiporah
    Weill Cornell Med Coll, Div Hematol & Oncol, New York, NY USA..
    Sperr, Wolfgang
    Med Univ Vienna, Dept Internal Med 1, Div Hematol & Hemostaseol & Ludwig Boltzmann Clus, Vienna, Austria..
    Saber, Wael
    Med Coll Wisconsin, Ctr Int Blood & Marrow Transplant Res, Dept Med, Milwaukee, WI USA..
    Kota, Vamsi
    Emory Univ, Winship Canc Inst, Dept Hematol & Oncol, Div Hematol, Atlanta, GA USA..
    Yavuz, Akif Selim
    Istanbul Univ, Istanbul Med Sch, Div Hematol, Istanbul, Turkey..
    Pullarkat, Vinod
    Rogosheske, John
    Univ Minnesota, Div Hematol Oncol & Transplantat, Minneapolis, MN 55455 USA..
    Hogan, William
    Mayo Clin, Dept Internal Med, Div Hematol, Minneapolis, MN USA..
    Van Besien, Koen
    Weill Cornell Med Coll, Dept Med, New York, NY USA..
    Hägglund, Hans
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Damaj, Gandhi
    Univ Basse Normandie, Univ Hosp, Sch Med, Inst Hematol,Dept Hematol, Caen, France..
    Arock, Michel
    Ecole Normale Super, CNRS UMR 8113, Cellular & Mol Oncol Unit, Cachan, France.;Univ Pitie Salpetriere, Ctr Hosp, Hematol Lab, Paris, France..
    Horny, Hans-Peter
    LMU, Inst Pathol, Munich, Germany..
    Metcalfe, Dean D.
    NIAID, Mast Cell Biol Sect, Lab Allerg Dis, NIH, Bethesda, MD USA..
    Deeg, H. Joachim
    Univ Washington, Fred Hutchinson Canc Res Ctr, Seattle, WA USA.;Univ Washington, Sch Med, Seattle, WA USA..
    Devine, Steven
    Ohio State Univ, Dept Med, Div Hematol, Columbus, OH 43210 USA.;Ohio State Univ, Ctr Comprehens Canc, Columbus, OH 43210 USA..
    Weisdorfl, Daniel
    Univ Minnesota, Div Hematol Oncol & Transplantat, Minneapolis, MN 55455 USA..
    Akin, Cem
    Harvard Med Sch, Brigham & Womens Hosp, Div Rheumatol Immunol & Allergy, Boston, MA USA..
    Valent, Peter
    Consensus Opinion on Allogeneic Hematopoietic Cell Transplantation in Advanced Systemic Mastocytosis2016In: Biology of blood and marrow transplantation, ISSN 1083-8791, E-ISSN 1523-6536, Vol. 22, no 8, p. 1348-1356Article in journal (Other academic)
  • 21. Valent, P
    et al.
    Sotlar, K
    Sperr, W R
    Escribano, L
    Yavuz, S
    Reiter, A
    George, T I
    Kluin-Nelemans, H C
    Hermine, O
    Butterfield, J H
    Hägglund, Hans
    Hematology Center Karolinska, Karolinska University Hospital, Stockholm, Sweden.
    Ustun, C
    Hornick, J L
    Triggiani, M
    Radia, D
    Akin, C
    Hartmann, K
    Gotlib, J
    Schwartz, L B
    Verstovsek, S
    Orfao, A
    Metcalfe, D D
    Arock, M
    Horny, H-P
    Refined diagnostic criteria and classification of mast cell leukemia (MCL) and myelomastocytic leukemia (MML): a consensus proposal2014In: Annals of Oncology, ISSN 0923-7534, E-ISSN 1569-8041, Vol. 25, no 9, p. 1691-1700Article in journal (Refereed)
    Abstract [en]

    Mast cell leukemia (MCL), the leukemic manifestation of systemic mastocytosis (SM), is characterized by leukemic expansion of immature mast cells (MCs) in the bone marrow (BM) and other internal organs; and a poor prognosis. In a subset of patients, circulating MCs are detectable. A major differential diagnosis to MCL is myelomastocytic leukemia (MML). Although criteria for both MCL and MML have been published, several questions remain concerning terminologies and subvariants. To discuss open issues, the EU/US-consensus group and the European Competence Network on Mastocytosis (ECNM) launched a series of meetings and workshops in 2011-2013. Resulting discussions and outcomes are provided in this article. The group recommends that MML be recognized as a distinct condition defined by mastocytic differentiation in advanced myeloid neoplasms without evidence of SM. The group also proposes that MCL be divided into acute MCL and chronic MCL, based on the presence or absence of C-Findings. In addition, a primary (de novo) form of MCL should be separated from secondary MCL that typically develops in the presence of a known antecedent MC neoplasm, usually aggressive SM (ASM) or MC sarcoma. For MCL, an imminent prephase is also proposed. This prephase represents ASM with rapid progression and 5%-19% MCs in BM smears, which is generally accepted to be of prognostic significance. We recommend that this condition be termed ASM in transformation to MCL (ASM-t). The refined classification of MCL fits within and extends the current WHO classification; and should improve prognostication and patient selection in practice as well as in clinical trials.

  • 22.
    Valent, Peter
    et al.
    Med Univ Vienna, Dept Internal Med 1, Div Hematol & Hemostaseol, Vienna, Austria;Med Univ Vienna, Ludwig Boltzmann Inst Hematol & Oncol, Vienna, Austria.
    Elberink, Joanna N. G. Oude
    Univ Groningen, Univ Med Ctr Groningen, Dept Allergol, Groningen, Netherlands.
    Gorska, Aleksandra
    Med Univ Gdansk, Dept Allergol, Gdansk, Poland.
    Lange, Magdalena
    Med Univ Gdansk, Dept Dermatol Venereol & Allergol, Gdansk, Poland.
    Zanotti, Roberta
    Verona Univ Hosp, Dept Med, Sect Hematol, Verona, Italy.
    van Anrooij, Bjorn
    Univ Groningen, Univ Med Ctr Groningen, Dept Allergol, Groningen, Netherlands.
    Bonifacio, Massimiliano
    Verona Univ Hosp, Dept Med, Sect Hematol, Verona, Italy.
    Bonadonna, Patrizia
    Med Univ Vienna, Ludwig Boltzmann Inst Hematol & Oncol, Vienna, Austria;Verona Univ Hosp, Allergy Unit, Verona, Italy.
    Gleixner, Karoline V.
    Med Univ Vienna, Dept Internal Med 1, Div Hematol & Hemostaseol, Vienna, Austria;Med Univ Vienna, Ludwig Boltzmann Inst Hematol & Oncol, Vienna, Austria.
    Hadzijusufovic, Emir
    Med Univ Vienna, Dept Internal Med 1, Div Hematol & Hemostaseol, Vienna, Austria;Univ Vet Med Vienna, Clin Internal Med & Infect Dis, Dept Compan Anim & Horses, Vienna, Austria.
    Perkins, Cecelia
    Stanford Univ, Dept Med, Sch Med, Div Hematol,Stanford Canc Inst, Stanford, CA 94305 USA.
    Hartmann, Karin
    Univ Cologne, Dept Dermatol, Cologne, Germany;Univ Lubeck, Dept Dermatol, Lubeck, Germany.
    Illerhaus, Anja
    Univ Cologne, Dept Dermatol, Cologne, Germany.
    Merante, Serena
    Univ Pavia, Dept Mol Med, Pavia, Italy;Univ Pavia, Dept Hematol Oncol, Pavia, Italy;Fdn IRCCS Policlin San Matteo, Pavia, Italy.
    Elena, Chiara
    Univ Pavia, Dept Mol Med, Pavia, Italy;Univ Pavia, Dept Hematol Oncol, Pavia, Italy;Fdn IRCCS Policlin San Matteo, Pavia, Italy.
    Shoumariyeh, Khalid
    Univ Freiburg, Fac Med, Dept Hematol Oncol & Stem Cell Transplantat, Med Ctr, Freiburg, Germany.
    von Bubnoff, Nikolas
    Univ Freiburg, Fac Med, Dept Hematol Oncol & Stem Cell Transplantat, Med Ctr, Freiburg, Germany;German Canc Consortium DKTK Partner Site Freiburg, Freiburg, Germany.
    Parente, Roberta
    Univ Salerno, Div Allergy & Clin Immunol, Salerno, Italy.
    Triggiani, Massimo
    Univ Salerno, Div Allergy & Clin Immunol, Salerno, Italy.
    Schwaab, Juliana
    Heidelberg Univ, Univ Med Mannheim, Med Klin, Hamatol & Onkol 3, Mannheim, Germany.
    Jawhar, Mohamad
    Heidelberg Univ, Univ Med Mannheim, Med Klin, Hamatol & Onkol 3, Mannheim, Germany.
    Caroppo, Francesca
    Univ Padua, Dept Med, Pediat Dermatol Unit, Padua, Italy.
    Fortina, Anna Belloni
    Univ Padua, Dept Med, Pediat Dermatol Unit, Padua, Italy.
    Brockow, Knut
    Tech Univ Munich, Dept Dermatol & Allergy Biederstein, Munich, Germany.
    Fuchs, David
    Johannes Kepler Univ Linz, Kepler Univ Hosp, Dept Internal Med 3, Hematol & Oncol, Linz, Austria.
    Greul, Rosemarie
    Johannes Kepler Univ Linz, Kepler Univ Hosp, Dept Internal Med 3, Hematol & Oncol, Linz, Austria.
    Yavuz, Akif Selim
    Istanbul Univ, Istanbul Med Sch, Div Hematol, Istanbul, Turkey.
    Doubek, Michael
    Univ Hosp Brno, Brno, Czech Republic.
    Mattsson, Mattias
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology. Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Immunology, Genetics and Pathology, Experimental and Clinical Oncology.
    Hägglund, Hans
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Panse, Jens
    Univ Hosp RWTH Aachen, Dept Oncol Haematol Haemostaseol & Stem Cell Tran, Aachen, Germany.
    Sabato, Vito
    Univ Antwerp, Dept Immunol Allergol Rheumatol, Fac Med & Hlth Sci, Antwerp, Belgium;Antwerp Univ Hosp, Antwerp, Belgium.
    Aberer, Elisabeth
    Med Univ Graz, Dept Dermatol & Venereol, Graz, Austria.
    Al-Ali, Haifa Kathrin
    Univ Hosp Leipzig, Leipzig, Germany.
    Morren, Marie-Anne
    Univ Hosp Leuven, Dept Dermatol, Leuven, Belgium.
    Varkonyi, Judit
    Semmelweis Univ, Dept Hematol, Budapest, Hungary.
    Zink, Alexander
    Tech Univ Munich, Dept Dermatol & Allergy Biederstein, Munich, Germany.
    Niedoszytko, Marek
    Med Univ Gdansk, Dept Allergol, Gdansk, Poland.
    Niederwieser, Dietger
    Univ Hosp Leipzig, Leipzig, Germany.
    Malcovati, Luca
    Univ Pavia, Dept Mol Med, Pavia, Italy.
    Reiter, Andreas
    Heidelberg Univ, Univ Med Mannheim, Med Klin, Hamatol & Onkol 3, Mannheim, Germany.
    Kennedy, Vanessa
    Stanford Univ, Dept Med, Sch Med, Div Hematol,Stanford Canc Inst, Stanford, CA 94305 USA.
    Gotlib, Jason
    Stanford Univ, Dept Med, Sch Med, Div Hematol,Stanford Canc Inst, Stanford, CA 94305 USA.
    Lortholary, Olivier
    Paris Descartes Univ, Ctr Natl Reference Mastocytoses, Necker Pasteur Ctr Infect Dis & Trop Med, Inst Imagine, Paris, France;Paris Descartes Univ, Ctr Natl Reference Mastocytoses, Necker Enfants Malad, Inst Imagine, Paris, France.
    Hermine, Olivier
    Univ Paris 05, Hop Necker, APHP,Ctr Reference Mastocytoses, Imagine Inst,INSERM,U1123,Sorbonne Paris Cite,Dep, Paris, France.
    Arock, Michel
    Hop La Pitie Salpetriere, Lab Hematol, Paris, France.
    Kluin-Nelemans, Hanneke
    Univ Groningen, Univ Med Ctr Groningen, Dept Hematol, Groningen, Netherlands.
    Sperr, Wolfgang R.
    Med Univ Vienna, Dept Internal Med 1, Div Hematol & Hemostaseol, Vienna, Austria;Med Univ Vienna, Ludwig Boltzmann Inst Hematol & Oncol, Vienna, Austria.
    The Data Registry of the European Competence Network on Mastocytosis (ECNM): Set Up, Projects, and Perspectives2019In: Journal of Allergy and Clinical Immunology: In Practice, ISSN 2213-2198, E-ISSN 2213-2201, Vol. 7, no 1, p. 81-87Article, review/survey (Refereed)
    Abstract [en]

    Mastocytosis is a unique hematologic neoplasm with complex biology and pathology and a variable clinical course. The disease can essentially be divided into cutaneous mastocytosis (CM) and systemic mastocytosis (SM). In adults, SM is diagnosed in most cases and manifests as either indolent or advanced disease. Patients with advanced SM have an unfavorable prognosis with reduced survival. However, so far, little is known about the prevalence of various categories of SM and about prognostic factors. In an attempt to learn more about the behavior and evolution of various forms of CM and SM, the European Competence Network on Mastocytosis (ECNM) initiated a mastocytosis registry in 2012. In this article, the set up and start phase of this registry are described. Until 2018, more than 3000 patients from 12 countries and 25 centers have been enrolled. In a majority of all patients, robust follow-up data and relevant clinical end points are available. Using this data set, a series of registry projects have been launched, with the aim to validate previously identified diagnostic and prognostic variables and to identify new disease-related and patient-related parameters in various forms of mastocytosis. Moreover, the core data set of the registry will be useful to establish multiparametric scoring systems through which prognostication and individualized management of patients with mastocytosis should improve in the foreseeable future. (C) 2019 American Academy of Allergy, Asthma & Immunology

  • 23.
    Worel, Nina
    et al.
    Med Univ Vienna, Dept Blood Grp Serol & Transfus Med, A-1090 Vienna, Austria..
    Buser, Andreas
    Swiss Red Cross, Ctr Blood Transfus, Basel, Switzerland.;Univ Basel Hosp, Dept Hematol, CH-4031 Basel, Switzerland..
    Greinix, Hildegard T.
    Med Univ Graz, Div Hematol, Graz, Austria..
    Hagglund, Hans
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Navarro, Willis
    Natl Marrow Donor Program, Minneapolis, MN USA..
    Pulsipher, Michael A.
    Univ Utah, Div Hematol & Hematol Malignancies, Huntsman Canc Inst, Primary Childrens Hosp, Salt Lake City, UT USA..
    de Faveri, Grazia Nicoloso
    Swiss Blood Stem Cell, Bern, Switzerland..
    Bengtsson, Mats
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Immunology, Genetics and Pathology, Clinical Immunology. Swedish Bone Marrow Donors, Tobias Registry, Uppsala, Sweden..
    Billen, Annelies
    UCL Canc Inst, London, England..
    Espino, German
    Univ Hosp Caja del Seguro Social, Hematol & Bone Marrow Transplantat Sect, Dept Internal Med, Panama City, Panama..
    Fechter, Mirjam
    Leiden Univ, Med Ctr, Europdonor Fdn Leiden, Leiden, Netherlands..
    Giudice, Valeria
    S Orsola Malpighi Univ Hosp, Dept Immunohematol & Transfus Med, Bologna, Italy..
    Hoelig, Kristina
    Tech Univ Dresden, Univ Hosp Carl Gustav Cams, Dept Internal Med 1, D-01062 Dresden, Germany..
    Kanamori, Heiwa
    Kanagawa Canc Ctr, Dept Hematol, Yokohama, Kanagawa, Japan..
    Kodera, Yoshihisa
    Aichi Med Univ, Asia Pacific Blood & Marrow Transplantat Grp, Sch Med, Nagakute, Aichi 48011, Japan.;Aichi Med Univ, Dept Promot Blood & Marrow Transplantat, Sch Med, Nagakute, Aichi 48011, Japan..
    Leitner, Gerda
    Med Univ Vienna, Dept Blood Grp Serol & Transfus Med, A-1090 Vienna, Austria..
    Netelenbos, Tanja
    Leiden Univ, Dept Immunohematol & Blood Transfus, Med Ctr, Leiden, Netherlands..
    Niedervvieser, Dietger
    Univ Hosp Leipzig, Dept Hematol, Leipzig, Germany..
    van Walraven, Suzanna M.
    Leiden Univ, Med Ctr, Europdonor Fdn Leiden, Leiden, Netherlands.;World Marrow Donor Assoc, Eth Working Grp, Leiden, Netherlands.;Oxford Univ Hosp NHS Trust, British Bone Marrow Donor Registry, Oxford, England..
    Rocha, Vanderson
    NHS BT, Cord Blood Banks, Oxford, England..
    Torosian, Tigran
    Fdn DKMS Polska, Warsaw, Poland..
    Vergueiro, Carmen
    FCM Santa Casa de Selo Paulo, Disciplina Hematol & Oncol, Sao Paulo, Brazil..
    Weisdorf, Daniel
    Univ Minnesota, Bone Marrow Transplant Program, Minneapolis, MN 55455 USA..
    Yabe, Hiromasa
    Tokai Univ, Sch Med, Dept Cell Transplantat & Regenerat Med, Tokyo 151, Japan..
    Halter, Joerg P.
    Univ Basel Hosp, Dept Hematol, CH-4031 Basel, Switzerland..
    Suitability Criteria for Adult Related Donors: A Consensus Statement from the Worldwide Network for Blood and Marrow Transplantation Standing Committee on Donor Issues2015In: Biology of blood and marrow transplantation, ISSN 1083-8791, E-ISSN 1523-6536, Vol. 21, no 12, p. 2052-2060Article in journal (Refereed)
    Abstract [en]

    The number of allogeneic hematopoietic stem cell (HSC) transplants performed globally each year continues to increase. Advances in HLA typing, better supportive care, and administration of reduced-intensity conditioning regimens allow treatment of older patients with older sibling donors. Pretransplant donor assessment and testing are very important processes affecting the quality and safety of donation. For unrelated HSC donors detailed recommendations for health assessment have been published, allowing donation only if they are unrestrictedly healthy. Eligibility criteria for related donors are less strict and vary significantly between centers. In situations where a family donor does not meet the suitability criteria for unrelated donors, involved physicians often struggle with the decision whether the matched relative is suitable for donation or not. On behalf of the Worldwide Network for Blood and Marrow Transplantation Standing Committee on Donor Issues, we intended to develop a consensus document with recommendations for donor workup and final clearance of family donors who would not be able to serve as unrelated donors because of their age or pre-existing diseases. This article covers different topics intending to support decision-making, with the goal of minimizing medical risk to the donor and protection of the recipient from transmissible diseases.

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