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  • 1. Abelsson, Johanna
    et al.
    Andreasson, Bjorn
    Samuelsson, Jan
    Hultcrantz, Malin
    Ejerblad, Elisabeth
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Johansson, Berit
    Emanuel, Robyn
    Mesa, Ruben
    Johansson, Peter
    Patients with polycythemia vera have worst impairment of quality of life among patients with newly diagnosed myeloproliferative neoplasms2013In: Leukemia and Lymphoma, ISSN 1042-8194, E-ISSN 1029-2403, Vol. 54, no 10, p. 2226-2230Article in journal (Refereed)
    Abstract [en]

    The quality of life (QoL) at the time of diagnosis of myeloproliferative neoplasm (MPN) has, to date, not been studied. One hundred and seventy-nine patients with MPN: 80 with essential thrombocythemia (ET), 73 with polycythemia vera (PV), 22 with primary myelofibrosis (PMF) and four with MPN undifferentiated, were included in this study. European Organisation for Research and Treatment of Cancer Quality of Life Questionnaire Core 30 (EORTC-QLQC30) and the MPN-Symptom Assessment Form (MPN-SAF) were used to evaluate QoL. Fatigue was the most reported symptom in these patients. Patients with PV reported significantly higher mean scores for inactivity, dizziness, cough, itching, depression and lower total QoL compared to patients with ET. Patients with PV had significantly more headache and itching compared to patients with PMF. When the newly diagnosed patients with MPN were compared with a cohort of patients with MPN with mean disease duration of 7.8 years, the differences were most striking for patients with PMF, with significantly more fatigue, abdominal discomfort, concentration problems, insomnia, fever, weight loss and lower overall QoL developed over time.

  • 2. Andersen, Christen L.
    et al.
    McMullin, Mary F.
    Ejerblad, Elisabeth
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Zweegman, Sonja
    Harrison, Claire
    Fernandes, Savio
    Bareford, David
    Knapper, Steven
    Samuelsson, Jan
    Loefvenberg, Eva
    Linder, Olle
    Andreasson, Bjorn
    Ahlstrand, Erik
    Jensen, Morten K.
    Bjerrum, Ole W.
    Vestergaard, Hanne
    Larsen, Herdis
    Klausen, Tobias W.
    Mourits-Andersen, Torben
    Hasselbalch, Hans C.
    A phase II study of vorinostat (MK-0683) in patients with polycythaemia vera and essential thrombocythaemia2013In: British Journal of Haematology, ISSN 0007-1048, E-ISSN 1365-2141, Vol. 162, no 4, p. 498-508Article in journal (Refereed)
    Abstract [en]

    Inhibition of histone deacetylases may be an important target in patients with myeloproliferative neoplasms. This investigator-initiated, non-randomized, open-label phase II multi-centre study included 63 patients (19 essential thrombocythaemia, 44 polycythaemia vera) from 15 centres. The primary objective was to evaluate if vorinostat was followed by a decline in clonal myeloproliferation as defined by European Leukaemia Net. Thirty patients (48%) completed the intervention period (24 weeks of therapy). An intention-to-treat response rate of 35% was identified. Pruritus was resolved [19% to 0% (P=0.06)] and the prevalence of splenomegaly was lowered from 50% to 27% (P=0.03). Sixty-five per cent of the patients experienced a decrease in JAK2 V617F allele burden (P=0.006). Thirty-three patients (52% of patients) discontinued study drug before end of intervention due to adverse events (28 patients) or lack of response (5 patients). In conclusion, vorinostat showed effectiveness by normalizing elevated leucocyte and platelet counts, resolving pruritus and significantly reducing splenomegaly. However, vorinostat was associated with significant side effects resulting in a high discontinuation rate. A lower dose of vorinostat in combination with conventional and/or novel targeted therapies may be warranted in future studies.

  • 3. Andersen, Christen Lykkegaard
    et al.
    Bjorn, Mads Emil
    McMullin, Mary Frances
    Harrison, Claire
    Samuelsson, Jan
    Ejerblad, Elisabeth
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Zweegman, Sonja
    Fernandes, Savio
    Bareford, David
    Knapper, Steven
    Lofvenberg, Eva
    Linder, Olle
    Andreasson, Bjorn
    Ahlstrand, Erik
    Jensen, Morten Krogh
    Bjerrum, Ole Weis
    Vestergaard, Hanne
    Larsen, Herdis
    Klausen, Tobias Wirenfeldt
    Mourits-Andersen, Torben
    Skov, Vibe
    Thomassen, Mads
    Kruse, Torben
    Gronbaek, Kirsten
    Hasselbalch, Hans Carl
    Circulating YKL-40 in patients with essential thrombocythemia and polycythemia vera treated with the novel histone deacetylase inhibitor vorinostat2014In: Leukemia Research, ISSN 0145-2126, E-ISSN 1873-5835, Vol. 38, no 7, p. 816-821Article in journal (Refereed)
    Abstract [en]

    YKL-40 regulates vascular endothelial growth factors and induces tumor proliferation. We investigated YKL-40 before and after treatment with vorinostat in 31 polycythemia vera (PV) and 16 essential thrombocythemia (ET) patients. Baseline PV patient levels were 2 times higher than in healthy controls (P<0.0001) and 1.7 times higher than in ET (P = 0.02). A significant correlation between YKL-40 at baseline and neutrophils, CRP, LDH, JAK2V617F and platelets in PV patients was observed, as well as a significantly greater reduction of YKL-40 levels in PV patients responding to therapy. YKL-40 might be a novel marker of disease burden and progression in myeloproliferative neoplasms. (C) 2014 Elsevier Ltd. All rights reserved.

  • 4.
    Berggren, Daniel Moreno
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences.
    Folkvaljon, Y.
    Univ Uppsala Hosp, Reg Canc Ctr, Uppsala, Sweden..
    Engvall, M.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Immunology, Genetics and Pathology.
    Sundberg, J.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Lehman, Sören
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Lambe, M.
    Univ Uppsala Hosp, Reg Canc Ctr, Uppsala, Sweden.;Karolinska Inst, Dept Med Epidemiol & Biostat, Stockholm, Sweden..
    Antunovic, P.
    Linkoping Univ Hosp, Dept Hematol, Linkoping, Sweden..
    Garelius, H.
    Sahlgrens Univ Hosp, Dept Med, Sect Haematol & Coagulat, Gothenburg, Sweden..
    Hellström-Lindberg, E.
    Karolinska Univ Hosp, Ctr Hematol & Regenerat Med, Stockholm, Sweden..
    Jadersten, M.
    Karolinska Univ Hosp, Ctr Hematol & Regenerat Med, Stockholm, Sweden..
    Lorenz, F.
    Umea Univ, Dept Med Biosci, Umea, Sweden..
    Nilsson, L.
    Skane Univ Hosp, Dept Med, Lund, Sweden..
    Ejerblad, Elisabeth
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Validation Of Prognostic Scoring Systems For Myelodysplastic Syndromes In The Swedish Mds-Register2016In: Haematologica, ISSN 0390-6078, E-ISSN 1592-8721, Vol. 101, p. 243-243Article in journal (Other academic)
  • 5.
    Berggren, Daniel Moreno
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Folkvaljon, Yasin
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Surgical Sciences.
    Engvall, Marie
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Immunology, Genetics and Pathology, Medicinsk genetik och genomik.
    Sundberg, Johan
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Lambe, Mats
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Surgical Sciences. Karolinska Inst, Dept Med Epidemiol & Biostat, Stockholm, Sweden.
    Antunovic, Petar
    Linkoping Univ Hosp, Dept Haematol, Linkoping, Sweden.
    Garelius, Hege
    Sahlgrens Univ Hosp, Sect Haematol & Coagulat, Dept Med, Gothenburg, Sweden.
    Lorenz, Fryderyk
    Umea Univ, Dept Med Biosci, Umea, Sweden.
    Nilsson, Lars
    Skane Univ Hosp, Dept Haematol Oncol & Radiat Phys, Lund, Sweden.
    Rasmussen, Bengt
    Orebro Univ Hosp, Sch Med Sci, Orebro, Sweden.
    Lehmann, Sören
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Hellstrom-Lindberg, Eva
    Karolinska Inst, Karolinska Univ Hosp, Dept Med Huddinge, Ctr Haematol & Regenerat Med, Stockholm, Sweden.
    Jadersten, Martin
    Karolinska Inst, Karolinska Univ Hosp, Dept Med Huddinge, Ctr Haematol & Regenerat Med, Stockholm, Sweden.
    Ejerblad, Elisabeth
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Prognostic scoring systems for myelodysplastic syndromes (MDS) in a population-based setting: a report from the Swedish MDS register2018In: British Journal of Haematology, ISSN 0007-1048, E-ISSN 1365-2141, Vol. 181, no 5, p. 614-627Article in journal (Refereed)
    Abstract [en]

    The myelodysplastic syndromes (MDS) have highly variable outcomes and prognostic scoring systems are important tools for risk assessment and to guide therapeutic decisions. However, few population-based studies have compared the value of the different scoring systems. With data from the nationwide Swedish population-based MDS register we validated the International Prognostic Scoring System (IPSS), revised IPSS (IPSS-R) and the World Health Organization (WHO) Classification-based Prognostic Scoring System (WPSS). We also present population-based data on incidence, clinical characteristics including detailed cytogenetics and outcome from the register. The study encompassed 1329 patients reported to the register between 2009 and 2013, 14% of these had therapy-related MDS (t-MDS). Based on the MDS register, the yearly crude incidence of MDS in Sweden was 2<bold></bold>9 per 100000 inhabitants. IPSS-R had a significantly better prognostic power than IPSS (P<0<bold></bold>001). There was a trend for better prognostic power of IPSS-R compared to WPSS (P=0<bold></bold>05) and for WPSS compared to IPSS (P=0<bold></bold>07). IPSS-R was superior to both IPSS and WPSS for patients aged 70years. Patients with t-MDS had a worse outcome compared to de novo MDS (d-MDS), however, the validity of the prognostic scoring systems was comparable for d-MDS and t-MDS. In conclusion, population-based studies are important to validate prognostic scores in a real-world' setting. In our nationwide cohort, the IPSS-R showed the best predictive power.

  • 6.
    Birgegård, Gunnar
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Samuelsson, Jan
    Univ Hosp Linkoping, Dept Hematol, Linkoping, Sweden.
    Ahlstrand, Erik
    Orebro Univ, Fac Med & Hlth, Dept Med, Orebro, Sweden.
    Ejerblad, Elisabeth
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Enevold, Christian
    Copenhagen Univ Hosp, Inst Inflammat Res, Copenhagen, Denmark.
    Ghanima, Waleed
    Ostfold Hosp, Dept Res, Sarpsborg, Norway.
    Hasselbalch, Hans
    Zealand Univ Hosp, Dept Hematol, Roskilde, Denmark.
    Nielsen, Claus H.
    Zealand Univ Hosp, Dept Hematol, Roskilde, Denmark.
    Knutsen, Håvar
    Ulleval Hosp, Dept Hematol, Oslo, Norway.
    Pedersen, Ole B.
    Naestved Hosp, Dept Clin Immunol, Naestved, Denmark.
    Sörensen, Anders
    Copenhagen Univ Hosp, Inst Inflammat Res, Copenhagen, Denmark;Zealand Univ Hosp, Dept Hematol, Roskilde, Denmark.
    Andreasson, Björn
    NU Hosp Grp, Hematol Sect, Specialist Med, Uddevalla, Sweden.
    Inflammatory functional iron deficiency common in myelofibrosis, contributes to anaemia and impairs quality of life. From the Nordic MPN study Group2019In: European Journal of Haematology, ISSN 0902-4441, E-ISSN 1600-0609, Vol. 102, no 3, p. 235-240Article in journal (Refereed)
    Abstract [en]

    Objectives: The study investigates the hypothesis that inflammation in myelofibrosis (MF) like in myeloma and lymphoma, may disturb iron distribution and contribute to anaemia.

    Methods: A cross-sectional study of 80 MF and 23 ET patients was performed.

    Results: About 35% of anaemic MF patients had functional iron deficiency (FID) with transferrin saturation <20 and normal or elevated S-ferritin (<500 mu g/L). In ET, FID was rare. In MF patients with FID, 70.6% were anaemic, vs 29.4% in patients without FID (P = 0.03). Hepcidin was significantly higher in MF patients with anaemia, including transfusion-dependent patients, 50.6 vs 24.4 mu g/L (P = 0.01). There was a significant negative correlation between Hb and inflammatory markers in all MF patients: IL-2, IL-6 and TNF-alpha, (P < 0.01-0.03), LD (P = 0.004) and hepcidin (P = 0.03). These correlations were also seen in the subgroup of anaemic MF patients (Table ). Tsat correlated negatively with CRP (P < 0.001). Symptom burden was heavier in MF patients with FID, and MPN-SAF quality of life scores correlated with IL-6 and CRP.

    Conclusions: The inflammatory state of MF disturbs iron turnover, FID is common and contributes to anaemia development and impairment of QoL. Anaemic MF patients should be screened for FID.

  • 7.
    Creignou, M.
    et al.
    Karolinska Inst Stockholm, Huddinge Ctr Regenerat Med & Hematol, Dept Med, Stockholm, Sweden..
    Ejerblad, Elisabeth
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Antunovic, P.
    Linkoping Univ Hosp, Dept Hematol, Linkoping, Sweden..
    Garelius, H.
    Sahlgrens Univ Hosp, Dept Internal Med, Sect Hematol & Coagulat, Gothenburg, Sweden..
    Lorenz, F.
    Norrland Univ Hosp, Dept Med, Umea, Sweden..
    Nilsson, L.
    Lund Univ Hosp, Dept Med, Lund, Sweden..
    Rasmussen, B.
    Orebro Univ Hosp, Dept Med, Orebro, Sweden..
    Walldin, G.
    Karolinska Inst Stockholm, Huddinge Ctr Regenerat Med & Hematol, Dept Med, Stockholm, Sweden..
    Jansson, M.
    Karolinska Inst Stockholm, Huddinge Ctr Regenerat Med & Hematol, Dept Med, Stockholm, Sweden..
    Karimi, M.
    Karolinska Inst Stockholm, Huddinge Ctr Regenerat Med & Hematol, Dept Med, Stockholm, Sweden..
    Hellstrom-Lindberg, E.
    Karolinska Inst Stockholm, Huddinge Ctr Regenerat Med & Hematol, Dept Med, Stockholm, Sweden..
    Jadersten, M.
    Karolinska Inst Stockholm, Huddinge Ctr Regenerat Med & Hematol, Dept Med, Stockholm, Sweden..
    Targeted Sequencing of A Cohort Of 385 Patients With Myelodysplastic Syndromes: A Multicenter, Population-Based Study From Sweden2017In: Leukemia Research, ISSN 0145-2126, E-ISSN 1873-5835, Vol. 55, p. S138-S139Article in journal (Other academic)
  • 8.
    Ejerblad, Elisabeth
    et al.
    Uppsala University, Medicinska vetenskapsområdet, Faculty of Medicine, Department of Medical Sciences. Verksamhetsområde hematologi.
    Fored, CM
    Lindblad, P
    Fryzek, J
    McLaughlin, JK
    Nyren, O
    Obesity and Risk for Chronic Renal Failure.2006In: J Am Soc Nephrol, ISSN 1046-6673Article in journal (Refereed)
  • 9.
    Ejerblad, Elisabeth
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Kvasnicka, Hans M
    Thiele, Jürgen
    Andreasson, Björn
    Björkholm, Magnus
    Löfvenberg, Eva
    Markevärn, Berit
    Merup, Mats
    Nilssson, Lars
    Palmblad, Jan
    Samuelsson, Jan
    Birgegård, Gunnar
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Diagnosis according to World Health Organization determines the long-term prognosis in patients with myeloproliferative neoplasms treated with anagrelide: Results of a prospective long-term follow-up2013In: Hematology, ISSN 1024-5332, E-ISSN 1607-8454, Vol. 18, no 1, p. 8-13Article in journal (Refereed)
    Abstract [en]

    OBJECTIVES:

    During long term follow-up of a cohort of patients with essential thrombocythemia (ET) and polycythemia vera (PV) a higher than expected incidence of myelofibrosis (MF) was noted. In order to test if the explanation could be found in the diagnostic criteria a re-evaluation of diagnosis using the 2008 WHO diagnostic criteria for ET and MF was performed.

    METHODS:

    This prospective study of 60 patients with ET and PV was set up in 1998 to evaluate the long-term efficacy and tolerability of anagrelide treatment. Bone marrow trephine biopsies were requested from study start, after 2 and 7 years of follow-up. A blinded re-evaluation of the bone marrow trephines was performed. The 2008 WHO bone marrow criteria were used for diagnosis and fibrosis grading.

    RESULTS:

    Of 40 patients with an initial diagnosis of ET, 21 were confirmed as 'true ET' whereas 17 were reclassified as primary myelofibrosis (PMF) (12 PMF-0, 3 PMF-1, 2 PMF-2) and 2 as myeloproliferative neoplasms of uncertain origin. After 7 years of follow-up, 19 of 21 patients with 'true ET' were alive, none had transformed to MF, leukemia, or myelodysplastic syndrome. In contrast, 4/17 patients reclassified as PMF had died, two patients transformed to myelodysplastic syndrome and 7 patients progressed to overt MF.

    DISCUSSION:

    We conclude that a blinded re-evaluation of bone marrow trephines from study start and after 7 years of follow-up using 2008 World Health Organization criteria was able to differentiate between true ET and PMF with a marked difference in follow-up outcome.

  • 10.
    Larfors, Gunnar
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Moreno Berggren, Daniel
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Garelius, Hege
    Sahlgrens Univ Hosp, Sect Haematol & Coagulat, Dept Specialist Med, Gothenburg, Sweden..
    Jadersten, Martin
    Karolinska Univ Hosp, Karolinska Inst, Dept Med Huddinge, Ctr Haematol & Regenerat Med, Stockholm, Sweden..
    Nilsson, Lars
    Skane Univ Hosp, Dept Haematol Oncol & Radiat Phys, Lund, Sweden..
    Rasmussen, Bengt
    Örebro Univ Hosp, Sch Med Sci, Örebro, Sweden..
    Ejerblad, Elisabeth
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Income, education and their impact on treatments and survival in patients with myelodysplastic syndromes2021In: European Journal of Haematology, ISSN 0902-4441, E-ISSN 1600-0609, Vol. 107, no 2, p. 219-228Article in journal (Refereed)
    Abstract [en]

    Objectives To assess whether socioeconomic indices such as income and educational level can explain part of the variation in survival among patients with myelodysplastic syndromes, and further to assess whether these factors influence care and treatment decisions. Methods Population-based cohort study on 2945 Swedish patients diagnosed between 2009 and 2018 and included in the Swedish MDS Register. Relative mortality was assessed by Cox regression, whereas treatment differences were assessed by Poisson regression. Regarding mortality, patients were also compared to a matched comparison group from the general population. Results Mortality was 50% higher among patients in the lowest income category compared to the highest and 40% higher in patients with mandatory school education only compared to those with college or university education. Treatment with hypomethylating agents and allogeneic stem cell transplantation, as well as investigation with cytogenetic diagnostics were also linked to income and education. The findings were not explained by differences in risk class or comorbidity at the time of diagnosis. Conclusions Income and education are linked to survival among patients with myelodysplastic syndromes. Socioeconomic status also seems to influence treatment intensity as patients with less income and education to a lesser degree receive hypomethylating agents and transplants.

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  • 11.
    Larfors, Gunnar
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Moreno Berggren, Daniel
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Garelius, Hege
    Sahlgrens Univ Hosp, Dept Specialist Med, Sect Haematol & Coagulat, Gothenburg, Sweden..
    Nilsson, Lars
    Skane Univ Hosp, Dept Haematol Oncol & Radiat Phys, Lund, Sweden..
    Rasmussen, Bengt
    Örebro Univ, Fac Med & Hlth, Dept Med, Örebro, Sweden..
    Hellström-Lindberg, Eva
    Karolinska Univ Hosp, Karolinska Inst, Dept Med Huddinge, Ctr Haematol & Regenerat Med, Stockholm, Sweden..
    Ejerblad, Elisabeth
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    MDS-Comorbidity Index using register data has prognostic impact in Swedish MDS patients2023In: Leukemia Research, ISSN 0145-2126, E-ISSN 1873-5835, Vol. 134, article id 107386Article in journal (Refereed)
    Abstract [en]

    Comorbidities influence the mortality in patients with myelodysplastic syndromes, and a growing body of evidence suggest that comorbidity history should be used in addition to established prognostic indices. A comorbidity index specific for MDS, the MDS-CI, was introduced a decade ago. In this study we aim to construct an MDS-CI version based on diagnoses from register data only, to expand its use beyond the clinical setting to retrospective and register based studies. We further test this version on a Swedish population-based MDS cohort of 2947 patients, and compare its prognostic accuracy to that of Charlson Comorbidity Index. Our register based MDS-CI divided patients into three risk groups of similar proportions as have been published for the original MDS-CI. Compared to low risk patients, intermediate and high risk patients had 50 % and 70 % higher mortality, respectively. The prognostic value of MDS-CI was equal to that of Charlson comorbidity index. Adding MDS-CI to the established prognostic factors IPSS-R and age increased the prognostic accuracy. In summary, we demonstrate that MDS-CI can be adequately estimated from diagnoses recorded in registers only, and that it is a useful tool in any future study on myelodysplastic syndromes with a need to adjust for comorbidities.

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  • 12. Lenhoff, S.
    et al.
    Dreimane, A.
    Ejerblad, Elisabeth
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Isaksson, C.
    Ljungman, P.
    Brune, M.
    Recent results of allogeneic stem cell transplantation for myelofibrosis using reduced intensity conditioning - the Swedish experience2015In: Bone Marrow Transplantation, ISSN 0268-3369, E-ISSN 1476-5365, Vol. 50, p. S493-S494Article in journal (Other academic)
  • 13. Lindgren, M.
    et al.
    Samuelsson, J.
    Nilsson, L.
    Knutsen, H.
    Ghanima, W.
    Astrom, M.
    Ejerblad, Elisabeth
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Hugosson, A.
    Westin, J.
    Palmqvist, L.
    Andreasson, B.
    Influence of IL-28B and IL-6 Genetic Polymorphism on IFN-ALFA Treatment Outcome in Patients with Polycythemia Vera and Essential Trombocythemia2014In: Haematologica, ISSN 0390-6078, E-ISSN 1592-8721, Vol. 99, no S1, p. 130-131Article in journal (Other academic)
  • 14. Lindgren, Marie
    et al.
    Samuelsson, Jan
    Nilsson, Lars
    Knutsen, Havar
    Ghanima, Waleed
    Astrom, Maria
    Ejerblad, Elisabeth
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Hugosson, Anna
    Johansson, Peter L.
    Andreasson, Bjorn
    A Retrospective Cohort Study of Interferon-alpha Therapy in Myeloproliferative Neoplasms; Adverse Events, Thromboembolic Incidence and Causes of Termination of Therapy2014In: Blood, ISSN 0006-4971, E-ISSN 1528-0020, Vol. 124, no 21Article in journal (Other academic)
  • 15.
    Lindholm, C.
    et al.
    Karolinska Inst, Ctr Hematol & Regenerat Med, Dept Med Huddinge, Stockholm, Sweden.;Karolinska Univ Hosp, Dept Hematol, Stockholm, Sweden.
    Olofsson, E.
    Karolinska Inst, Ctr Hematol & Regenerat Med, Dept Med Huddinge, Stockholm, Sweden.;Karolinska Univ Hosp, Dept Hematol, Stockholm, Sweden.
    Creignou, M.
    Karolinska Inst, Ctr Hematol & Regenerat Med, Dept Med Huddinge, Stockholm, Sweden.;Karolinska Univ Hosp, Dept Hematol, Stockholm, Sweden.
    Nilsson, L.
    Skane Univ Hosp, Dept Hematol Oncol & Radiat Phys, Lund, Sweden.
    Garelius, H. Gravdahl
    Sahlgrens Univ Hosp, Sect Hematol & Coagulat, Med, Gothenburg, Sweden.
    Cammenga, J.
    Linköping Univ Hosp, Dept Hematol, BKV, Linköping, Sweden.;Linköping Univ Hosp, CKOC, Linköping, Sweden.;Linköping Univ, Linköping, Sweden.
    Ljungman, P.
    Karolinska Univ Hosp Huddinge, Dept Cellular Therapy & Allogene Stem Cell Transp, Stockholm, Sweden.;Karolinska Inst, Dept Med Huddinge, Div Hematol, Stockholm, Sweden.
    Ejerblad, Elisabeth
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Tobiasson, M.
    Karolinska Inst, Ctr Hematol & Regenerat Med, Dept Med Huddinge, Stockholm, Sweden.;Karolinska Univ Hosp, Dept Hematol, Stockholm, Sweden.
    Failure to reach hematopoietic allogenic stem cell transplantation in patients with myelodysplastic syndromes planned for transplantation: a population-based study2022In: Bone Marrow Transplantation, ISSN 0268-3369, E-ISSN 1476-5365, Vol. 57, no 4, p. 598-606Article in journal (Refereed)
    Abstract [en]

    The only potential cure for patients with myelodysplastic syndrome (MDS) is allogeneic hematopoietic stem cell transplantation (HCT). However, a proportion of patients who are HCT candidates do not finally get transplanted. This population-based study aimed to characterize HCT candidates were attempting to reach HCT fail and to identify causes and risk factors for failure. Data were collected from (1) the national Swedish registry, enrolling 291 transplant candidates between 2009-2018, and (2) Karolinska University Hospital, enrolling 131 transplantation candidates between 2000 and 2018. Twenty-five % (nation-wide) and 22% (Karolinska) failed to reach HCT. Reasons for failure to reach HCT were progressive and refractory disease (47%), no donor identified (22%), identification of comorbidity (18%), and infectious complications (14%). Factors associated with failure to reach HCT were IPSS-R cytogenetic risk-group very poor, mixed MDS/MPN disease, low blast count (0-4.9%), and low hemoglobin levels (<= 7.9 g/dL). Transplanted patients had a longer overall survival (OS) compared to patients who failed to reach transplantation (83 months versus 14 months; p < 0.001). The survival advantage was seen for the IPSS-R risk groups intermediate, high, and very high. This study demonstrated that a high proportion of HCT-candidates fail to reach HCT and underlines the difficulties associated with bridging MDS patients to HCT.

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  • 16.
    McPherson, S.
    et al.
    Queens Univ Belfast, Blood Canc Res Grp, Ctr Canc Res & Cell Biol, Belfast, Antrim, North Ireland.
    McCourt, P.
    Queens Univ Belfast, Blood Canc Res Grp, Ctr Canc Res & Cell Biol, Belfast, Antrim, North Ireland.
    Ejerblad, Elisabeth
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Zweegman, S.
    Vrije Univ Amsterdam, Med Ctr, Dept Haematol, Amsterdam, Netherlands.
    Harrison, C.
    Guys & St Thomas NHS Fdn Trust, Dept Haematol, London, England.
    Fernandes, S.
    Russells Hall Hosp, Dept Haematol, Dudley, England.
    Knapper, S.
    Cardiff Univ, Dept Haematol, Cardiff, S Glam, Wales.
    Samuelsson, J.
    Stockholm South Hosp, Dept Internal Med, Stockholm, Sweden.
    Linder, O.
    Orebro Univ Hosp, Dept Haematol, Orebro, Sweden.
    Andreasson, B.
    NU Hosp Org, Dept Haematol, Uddevalla Hosp, Uddevalla, Sweden.
    Ahlstrand, E.
    Orebro Univ Hosp, Dept Haematol, Orebro, Sweden.
    Jensen, M.
    Herlev Hosp, Dept Haematol, Herlev, Denmark.
    Bjerrum, O.
    Rigshosp, Dept Haematol, Copenhagen Univ Hosp, Copenhagen, Denmark.
    Vestergaard, H.
    Odense Univ Hosp, Dept Haematol, Odense, Denmark.
    Larsen, H.
    Viborg Hosp, Dept Haematol, Dept Internal Med, Viborg, Denmark.
    Mourits-Andersen, T.
    Esbjerg Cent Hosp, Dept Haematol, Esbjerg, Denmark.
    Hasselbalch, H.
    Roskilde Hosp, Dept Haematol, Roskilde, Denmark.
    Andersen, C.
    Roskilde Hosp, Dept Haematol, Roskilde, Denmark.
    McMullin, M. F.
    Queens Univ Belfast, Sch Med Dent & Biomed Sci, Ctr Med Educ, Belfast, Antrim, North Ireland.
    Mills, K.
    Queens Univ Belfast, Blood Canc Res Grp, Ctr Canc Res & Cell Biol, Belfast, Antrim, North Ireland.
    METHYLATION AGE IN MPN PATIENTS AS A CORRELATE FOR DISEASE STATUS, ALLELE BURDEN AND THERAPEUTIC RESPONSE2017In: Haematologica, ISSN 0390-6078, E-ISSN 1592-8721, Vol. 102, no Suppl. 2, p. 278-279, article id P697Article in journal (Other academic)
  • 17.
    Moreno Berggren, Daniel
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Garelius, Hege
    Willner Hjelm, Petter
    Nilsson, Lars
    Rasmussen, Bengt
    Weibull, Caroline E.
    Lambe, Mats
    Lehmann, Sören
    Hellström-Lindberg, Eva
    Jädersten, Martin
    Ejerblad, Elisabeth
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Therapy-related MDS dissected based on primary disease and treatment—a nationwide perspective2023In: Leukemia, ISSN 0887-6924, E-ISSN 1476-5551, Vol. 37, no 5, p. 1103-1112Article in journal (Refereed)
  • 18.
    Moreno Berggren, Daniel
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Kjellander, Matilda
    Backlund, Ellen
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Engvall, Marie
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Immunology, Genetics and Pathology, Medicinsk genetik och genomik.
    Garelius, Hege
    Lorenz, Fryderyk
    Nilsson, Lars
    Rasmussen, Bengt
    Lehmann, Sören
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Hellström-Lindberg, Eva
    Jädersten, Martin
    Ungerstedt, Johanna
    Ejerblad, Elisabeth
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Prognostic scoring systems and comorbidities in chronic myelomonocytic leukaemia: a nationwide population-based study2021In: British Journal of Haematology, ISSN 0007-1048, E-ISSN 1365-2141, Vol. 192, no 3, p. 474-483Article in journal (Refereed)
    Abstract [en]

    Outcomes in chronic myelomonocytic leukaemia (CMML) are highly variable and may be affected by comorbidity. Therefore, prognostic models and comorbidity indices are important tools to estimate survival and to guide clinicians in individualising treatment. In this nationwide population‐based study, we assess comorbidities and for the first time validate comorbidity indices in CMML. We also compare the prognostic power of: the revised International Prognostic Scoring System (IPSS‐R), CMML‐specific prognostic scoring system (CPSS), MD Anderson Prognostic Scoring System (MDAPS) and Mayo score. In this cohort of 337 patients with CMML, diagnosed between 2009 and 2015, the median overall survival was 21·3 months. Autoimmune conditions were present in 25% of the patients, with polymyalgia rheumatica and Hashimoto’s thyroiditis being most common. Of the tested comorbidity indices: the Charlson Comorbidity Index (CCI), Haematopoietic cell transplantation‐specific Comorbidity Index (HCT‐CI) and Myelodysplastic Syndrome‐Specific Comorbidity Index (MDS‐CI), CCI had the highest C‐index (0·62) and was the only comorbidity index independently associated with survival in multivariable analyses. When comparing the prognostic power of the scoring systems, the CPSS had the highest C‐index (0·69). In conclusion, using ‘real‐world’ data we found that the CCI and CPSS have the best prognostic power and that autoimmune conditions are overrepresented in CMML.

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  • 19.
    Rasmussen, Bengt
    et al.
    Örebro Univ, Sch Med Sci, Örebro, Sweden.
    Gohring, Gudrun
    Hannover Med Sch, Dept Human Genet, Hannover, Germany.
    Bernard, Elsa
    Mem Sloan Kettering Canc Ctr, Dept Epidemiol & Biostat, Computat Oncol Serv, New York, NY 10021 USA.
    Nilsson, Lars
    Skane Univ Hosp, Dept Hematol Oncol & Radiat Phys, Lund, Sweden.
    Tobiasson, Magnus
    Karolinska Inst, Karolinska Univ Hosp, Dept Med Huddinge, Ctr Hematol & Regenerat Med, Stockholm, Sweden.
    Jadersten, Martin
    Karolinska Inst, Karolinska Univ Hosp, Dept Med Huddinge, Ctr Hematol & Regenerat Med, Stockholm, Sweden.
    Garelius, Hege
    Sahlgrens Univ Hosp, Dept Med, Sect Hematol & Coagulat, Gothenburg, Sweden.
    Dybedal, Ingunn
    Oslo Univ Hosp, Dept Hematol, Oslo, Norway.
    Gronbaek, Kirsten
    Copenhagen Univ Hosp, Dept Hematol, Rigshosp, Copenhagen, Denmark.
    Ejerblad, Elisabeth
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Lorenz, Fryderyk
    Umeå Univ, Dept Med Biosci, Umeå, Sweden.
    Flogegard, Max
    Falun Gen Hosp, Dept Internal Med, Falun, Sweden.
    Marcher, Claus Werenberg
    Odense Univ Hosp, Dept Hematol, Odense, Denmark.
    Öster Fernström, Annette
    Karolinska Inst, Ctr Hematol & Regenerat Med HERM, Dept Med Huddinge, Stockholm, Sweden..
    Cavelier, Lucia
    Uppsala University, Science for Life Laboratory, SciLifeLab. Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Immunology, Genetics and Pathology, Medicinsk genetik och genomik.
    Papaemmanuil, Elli
    Mem Sloan Kettering Canc Ctr, Dept Epidemiol & Biostat, Computat Oncol Serv, New York, NY 10021 USA.
    Ebeling, Freja
    Helsinki Univ Cent Hosp, Dept Med, Div Hematol, Helsinki, Finland.
    Kittang, Astrid Olsnes
    Haukeland Hosp, Dept Med, Div Hematol, Bergen, Norway.
    Norgaard, Jan Maxwell
    Aarhus Univ Hosp, Dept Hematol, Aarhus, Denmark.
    Saft, Leonie
    Karolinska Univ Hosp & Inst, Div Hematopathol, Dept Clin Pathol, Stockholm, Sweden.
    Mollgard, Lars
    Sahlgrens Univ Hosp, Dept Med, Sect Hematol & Coagulat, Gothenburg, Sweden.
    Hellstrom-Lindberg, Eva
    Karolinska Inst, Karolinska Univ Hosp, Dept Med Huddinge, Ctr Hematol & Regenerat Med, Stockholm, Sweden.
    "Randomized phase II study of azacitidine +/- lenalidomide in higher-risk myelodysplastic syndromes and acute myeloid leukemia with a karyotype including Del(5q)"2022In: Leukemia, ISSN 0887-6924, E-ISSN 1476-5551, Vol. 36, no 5, p. 1436-1439Article in journal (Other academic)
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    FULLTEXT01
  • 20.
    Wedge, Eileen
    et al.
    Copenhagen Univ Hosp, Dept Hematol, Juliane Maries Vej 6, DK-2100 Copenhagen, Denmark.;Univ Copenhagen, Fac Hlth & Med Sci, Biotech Res & Innovat Ctr, Copenhagen, Denmark.;Univ Copenhagen, Danish Stem Cell Ctr Danstem, Copenhagen, Denmark..
    Hansen, Jakob Werner
    Copenhagen Univ Hosp, Dept Hematol, Juliane Maries Vej 6, DK-2100 Copenhagen, Denmark.;Univ Copenhagen, Fac Hlth & Med Sci, Biotech Res & Innovat Ctr, Copenhagen, Denmark.;Univ Copenhagen, Danish Stem Cell Ctr Danstem, Copenhagen, Denmark..
    Dybedal, Ingunn
    Oslo Univ Hosp, Dept Hematol, Oslo, Norway..
    Creignou, Maria
    Karolinska Inst, Ctr Hematol & Regenerat Med, Stockholm, Sweden.;Karolinska Univ Hosp, Dept Hematol, Stockholm, Sweden..
    Ejerblad, Elisabeth
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.
    Lorenz, Fryderyk
    Univ Hosp Umeå, Dept Med, Umeå, Sweden..
    Werlenius, Olle
    Sahlgrens Univ Hosp, Sect Hematol & Coagulat, Gothenburg, Sweden..
    Ungerstedt, Johanna
    Karolinska Inst, Ctr Hematol & Regenerat Med, Stockholm, Sweden.;Karolinska Univ Hosp, Dept Hematol, Stockholm, Sweden..
    Holm, Mette Skov
    Aarhus Univ Hosp, Dept Hematol, Aarhus, Denmark..
    Nilsson, Lars
    Skane Univ Hosp, Dept Med, Lund, Sweden..
    Kittang, Astrid Olsnes
    Haukeland Hosp, Dept Med, Sect Hematol, Bergen, Norway..
    Antunovic, Peter
    Linköping Univ Hosp, Dept Hematol, Linköping, Sweden..
    Rohon, Peter
    Univ Hosp Olomouc, Dept Hematooncol, Olomouc, Czech Republic..
    Andersen, Mette Klarskov
    Copenhagen Univ Hosp, Dept Clin Genet, Copenhagen, Denmark..
    Papaemmanuil, Elli
    Mem Sloan Kettering Canc Ctr, Computat Oncol Serv, 1275 York Ave, New York, NY 10021 USA.;Mem Sloan Kettering Canc Ctr, Ctr Hematol Malignancies, 1275 York Ave, New York, NY 10021 USA..
    Bernard, Elsa
    Mem Sloan Kettering Canc Ctr, Computat Oncol Serv, 1275 York Ave, New York, NY 10021 USA.;Mem Sloan Kettering Canc Ctr, Ctr Hematol Malignancies, 1275 York Ave, New York, NY 10021 USA..
    Jädersten, Martin
    Karolinska Inst, Ctr Hematol & Regenerat Med, Stockholm, Sweden.;Karolinska Univ Hosp, Dept Hematol, Stockholm, Sweden..
    Hellström-Lindberg, Eva
    Karolinska Inst, Ctr Hematol & Regenerat Med, Stockholm, Sweden.;Karolinska Univ Hosp, Dept Hematol, Stockholm, Sweden..
    Gronbaek, Kirsten
    Copenhagen Univ Hosp, Dept Hematol, Juliane Maries Vej 6, DK-2100 Copenhagen, Denmark.;Univ Copenhagen, Fac Hlth & Med Sci, Biotech Res & Innovat Ctr, Copenhagen, Denmark.;Univ Copenhagen, Danish Stem Cell Ctr Danstem, Copenhagen, Denmark..
    Ljungman, Per
    Karolinska Inst, Ctr Hematol & Regenerat Med, Stockholm, Sweden.;Karolinska Univ Hosp, Dept Hematol, Stockholm, Sweden..
    Friis, Lone Smidstrup
    Copenhagen Univ Hosp, Dept Hematol, Juliane Maries Vej 6, DK-2100 Copenhagen, Denmark..
    Allogeneic Hematopoietic Stem Cell Transplantation for Chronic Myelomonocytic Leukemia: Clinical and Molecular Genetic Prognostic Factors in a Nordic Population2021In: Transplantation and Cellular Therapy, ISSN 2666-6375, E-ISSN 2666-6367, Vol. 27, no 12, p. 991.e1-991.e9Article in journal (Refereed)
    Abstract [en]

    Chronic myelomonocytic leukemia (CMML) is an aggressive disease in which survival after allogeneic hematopoietic stem cell transplantation (HCT) remains relatively poor. An assessment of prognostic factors is an important part of treatment decision making and has the potential to be greatly improved by the inclusion of molecular genetics. However, there is a significant knowledge gap in the interpretation of mutational patterns. This study aimed to describe outcomes of allogeneic HCT in patients with CMML in relation to clinical and molecular genetic risk factors. This retrospective study included 64 patients with CMML who underwent allogeneic HCT between 2008 and 2018, with a median follow-up of 5.4 years. Next-generation sequencing using targeted myeloid panels was carried out on saved material from 51 patients from the time of transplantation. Kaplan-Meier and Cox regression were used for analysis of overall survival (OS), and cumulative incidence with competing risks and Fine and Gray models were used for analysis of relapse and nonrelapse mortality (NRM). Mutations were detected in 48 patients (94%), indicating high levels of minimal residual disease (MRD) positivity at transplantation, even among those in complete remission (CR) (n = 14), 86% of whom had detectable mutations. The most frequently mutated genes were ASXL1 (37%), TET2 (37%), RUNX1 (33%), SRSF2 (26%), and NRAS (20%). Risk stratification using the CMML-specific Prognostic Scoring System molecular score (CPSS-Mol) resulted in 45% of patients moving to a higher risk-group compared with risk stratification using the CPSS. High leucocyte count (>= 13 x 10(9)/L), transfusion requirement, and previous intensive chemotherapy were associated with higher incidence of relapse. Being in CR was not linked to better outcomes. Neither ASXL1 nor RUNX1 mutation was associated with a difference in OS, relapse, or NRM, despite being high risk in the nontransplantation setting. TET2 mutations were associated with a significantly higher 3-year OS (73% versus 40%; P =.039). Achieving MRD-negative CR was rare in this CMML cohort, which may explain why we did not observe better outcomes for those in CR. This merits further investigation. Our analyses suggest that the negative impact of ASXL1 and RUNX1 mutations can be overcome by allogeneic HCT; however, risk stratification is complex in CMML and requires larger cohorts and multivariate models, presenting an ongoing challenge in this rare disease.

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