Pathophysiology, diagnosis, and treatment of paroxysmal nocturnal hemoglobinuria: a review.


:Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired disorder of the hematopoietic stem cell that makes blood cells more sensitive to the action of complement. Patients experience intravascular hemolysis, smooth muscle dystonia, renal failure, arterial and pulmonary hypertension, recurrent infectious diseases and an increased risk of notably dreadful thrombotic complications. The diagnosis is made by flow cytometry. Efforts have been recently performed to improve the sensitivity and the standardization of this technique. PNH is frequently associated with aplastic anemia or low-risk myelodysplasia and may be asymptomatic. Management of the classical form of PNH has been dramatically revolutionized by the development of eculizumab, which brings benefits in terms of hemolysis, quality of life, renal function, thrombotic risk, and life expectancy. Prophylaxis and treatment of arterial and venous thrombosis currently remain a challenge in PNH.


Eur J Haematol


Devalet B,Mullier F,Chatelain B,Dogné JM,Chatelain C




Has Abstract


2015-09-01 00:00:00












  • Treatment of consecutive patients with chronic myeloid leukaemia in the cooperating centres from the Czech Republic and the whole of Slovakia after 2000--a report from the population-based CAMELIA Registry.

    abstract:BACKGROUND:Most results on the treatment of chronic myeloid leukaemia (CML) with imatinib were obtained from clinical trials that may differ from the routine practice. We report the results of treatment of consecutive patients with CML at ten major centres during 2000-2008. PATIENTS AND METHODS:Data reporting was retr...

    journal_title:European journal of haematology

    pub_type: 杂志文章,多中心研究


    authors: Faber E,Mužík J,Koza V,Demečková E,Voglová J,Demitrovičová L,Chudej J,Markuljak I,Cmunt E,Kozák T,Tóthová E,Jarošová M,Dušek L,Indrák K

    更新日期:2011-08-01 00:00:00

  • Genetic variations in multiple myeloma I: effect on risk of multiple myeloma.

    abstract::Few risk factors have been established for the plasma cell disorder multiple myeloma, but some of these like African American ethnicity and a family history of B-cell lymphoproliferative diseases suggest a genetic component for the disease. Genetic variation represents the genetic basis of variability in a population....

    journal_title:European journal of haematology

    pub_type: 杂志文章,评审


    authors: Vangsted A,Klausen TW,Vogel U

    更新日期:2012-01-01 00:00:00

  • Analysis of low and high grade B-cell lymphoma subtypes using semi-nested PCR and two primer sets.

    abstract::Immunoglobulin gene rearrangements in B-cell lymphoma subtypes may not always be detected by PCR if only one primer set is applied. We therefore analysed a range of low and high grade B-cell lymphoma subtypes for monoclonality using PCR, to determine appropriate primer selection strategies for routine diagnostic use. ...

    journal_title:European journal of haematology

    pub_type: 杂志文章


    authors: Essop MF,Blakolmer K,Close PM,Manuel YE,Cornelius S

    更新日期:1997-09-01 00:00:00

  • Outcomes of relapsed or refractory acute myeloid leukemia patients failing venetoclax-based salvage therapies.

    abstract:OBJECTIVES AND METHODS:We conducted a retrospective analysis to evaluate the outcomes of 28 heavily pretreated (median 3 (2-6) treatment lines, sixteen (57%) allotransplanted) relapsed/refractory acute myeloid leukemia patients who had failed salvage venetoclax-based therapies. RESULTS:The median age was 59 years (20-...

    journal_title:European journal of haematology

    pub_type: 杂志文章


    authors: Zucenka A,Pileckyte R,Trociukas I,Peceliunas V,Vaitekenaite V,Maneikis K,Davainis L,Zvirblis T,Stoskus M,Gineikiene E,Norvilas R,Dirse V,Surauciute A,Zucenkiene E,Griskevicius L

    更新日期:2021-01-01 00:00:00

  • FDA's and EMA's approval of brentuximab vedotin for advanced Hodgkin lymphoma: Another player in the town?

    abstract::ECHELON-1 study is a randomized open-labeled controlled trial investigating whether addition of brentuximab vedotin to chemotherapy offers benefit over the standard chemotherapy regimen in advanced Hodgkin lymphoma. After a median follow-up of 24.6 months, it has met its primary endpoint the reduction of modified prog...

    journal_title:European journal of haematology

    pub_type: 杂志文章,评审


    authors: Milunović V,Mišura Jakobac K,Kursar M,Mandac Rogulj I,Ostojić Kolonić S

    更新日期:2019-09-01 00:00:00

  • Free iron catalyzes oxidative damage to hematopoietic cells/mesenchymal stem cells in vitro and suppresses hematopoiesis in iron overload patients.

    abstract:OBJECTIVES:Transfusional iron overload is of major concern in hematological disease. Iron-overload-related dyserythropoiesis and reactive oxygen species (ROS)-related damage to hematopoietic stem cell (HSC) function are major setbacks in treatment for such disorders. We therefore aim to investigate the effect of iron o...

    journal_title:European journal of haematology

    pub_type: 杂志文章


    authors: Lu W,Zhao M,Rajbhandary S,Xie F,Chai X,Mu J,Meng J,Liu Y,Jiang Y,Xu X,Meng A

    更新日期:2013-09-01 00:00:00

  • CD1-reactive leukemic cells in bone marrow: presence of Langerhans cell marker on leukemic monocytic cells.

    abstract::Langerhans cells originate in bone marrow and probably belong to the monocyte-macrophage lineage. CD1 is a specific marker of Langerhans cells. By immunofluorescence and immunoelectron microscopy, CD1a antigen and myeloid markers (CD11, CD13, CD14, CD15, CD33, HLA-DR) were studied in 53 cases of acute myeloid leukemia...

    journal_title:European journal of haematology

    pub_type: 杂志文章


    authors: Misery L,Campos L,Dezutter-Dambuyant C,Guyotat D,Treille D,Schmitt D,Thivolet J

    更新日期:1992-01-01 00:00:00

  • Survivin expression, apoptosis and proliferation in chronic myelomonocytic leukemia.

    abstract::We analyzed the expression of the inhibitor of apoptosis survivin by immunocytochemistry in bone marrow cells from patients with chronic myelomonocytic leukemia (CMML) to evaluate possible abnormalities in comparison with other myelodysplastic (MDS) and myeloproliferative syndromes, and to investigate a possible corre...

    journal_title:European journal of haematology

    pub_type: 杂志文章


    authors: Invernizzi R,Travaglino E,Benatti C,Malcovati L,Della Porta M,Cazzola M,Ascari E

    更新日期:2006-06-01 00:00:00

  • Prognostic value of serum M-protein doubling time at escape from plateau of multiple myeloma. The Finnish Leukaemia Group.

    abstract::A long plateau phase is one of the strongest signs predicting long survival in multiple myeloma. The kinetics of escape from the plateau is, however, poorly known, and so is its influence on subsequent survival. During a 9-yr follow-up of 432 myeloma patients the serum M-protein doubling time at first relapse was meas...

    journal_title:European journal of haematology

    pub_type: 杂志文章


    authors: Oivanen TM

    更新日期:1996-09-01 00:00:00

  • Effectiveness of propagermanium treatment in multiple myeloma patients.

    abstract::Interferon (IFN) is one of several drugs effective in treating multiple myeloma (MM), and propagermanium is an IFN inducer. We report on 10 MM patients who were treated with propagermanium at doses from 10 to 40 mg. Two patients achieved complete remission (CR), two patients achieved partial remission (PR), and the co...

    journal_title:European journal of haematology

    pub_type: 临床试验,杂志文章


    authors: Tsutsumi Y,Tanaka J,Kanamori H,Musashi M,Minami H,Fukushima A,Yamato H,Ehira N,Kawamura T,Obara S,Ogura N,Asaka M,Imamura M,Masauzi N

    更新日期:2004-12-01 00:00:00

  • Primary pancreatic lymphoma: Clinical presentation, diagnosis, treatment, and outcome.

    abstract::Primary pancreatic lymphoma (PPL) is a rare disease representing 0.1% of malignant lymphomas, which lacks well-defined diagnostic and therapeutic protocols. OBJECTIVES:To describe PPL clinical, diagnostic and histological characteristics, together with therapy and outcome, in a relatively large series of patients. M...

    journal_title:European journal of haematology

    pub_type: 杂志文章


    authors: Facchinelli D,Sina S,Boninsegna E,Borin A,Tisi MC,Piazza F,Scapinello G,Maiolo E,Hohaus S,Zamò A,Merli M,Stefani PM,Mellone F,Basso M,Sartori R,Rusconi C,Parisi A,Manfrin E,Krampera M,Ruggeri M,Visco C,Tecchio C

    更新日期:2020-10-01 00:00:00

  • Deafness from eighth cranial nerve involvement in a patient with large-cell transformation of mycosis fungoides.

    abstract::Central nervous system (CNS) involvement by mycosis fungoides (MF) is rare and is usually seen in advanced stages with lymph node or visceral involvement. We describe a patient with advanced stage MF in large-cell transformation who presented with profound hearing loss after chemotherapy. Despite an initial differenti...

    journal_title:European journal of haematology

    pub_type: 杂志文章


    authors: Chua SL,Seymour JF,Prince HM

    更新日期:2000-05-01 00:00:00

  • The hematologic consequences of obesity.

    abstract::The prevalence of obesity is increasing and progressively influencing physician-patient interactions. While there is a sizable amount of data demonstrating that obesity is a state of low-grade inflammation, to our knowledge, there is no single review summarizing its effects on hematologic parameters and thrombotic ris...

    journal_title:European journal of haematology

    pub_type: 杂志文章,评审


    authors: Purdy JC,Shatzel JJ

    更新日期:2020-12-03 00:00:00

  • BKV-specific T cells in the treatment of severe refractory haemorrhagic cystitis after HLA-haploidentical haematopoietic cell transplantation.

    abstract:BACKGROUND:Haemorrhagic cystitis caused by BK virus (BKV) is a known complication of allogeneic haematopoietic cell transplantation (HCT) and is relatively common following HLA-haploidentical transplantation. Adoptive immunotransfer of virus-specific T cells from the donor is a promising therapeutic approach, although ...

    journal_title:European journal of haematology



    authors: Pello OM,Innes AJ,Bradshaw A,Finn SA,Uddin S,Bray E,Olavarria E,Apperley JF,Pavlů J

    更新日期:2017-06-01 00:00:00

  • Effect of cytokines on the toxicity of cytostatic drugs on leukemic cells in vitro and in vivo.

    abstract::Most cytostatic drugs exert their effect on cells in active cell cycle. To improve the effect of cytostatic drugs we have tried, prior to treatment in vitro, to recruit tumor cells from G0 with growth factors. Leukemic cells from the bone marrows of 26 patients with AML and CML in blast crisis were incubated with G-CS...

    journal_title:European journal of haematology

    pub_type: 杂志文章


    authors: Sundman-Engberg B,Tidefelt U,Paul C

    更新日期:1996-01-01 00:00:00

  • Response rate and survival after conventional chemotherapy for multiple myeloma by hospitals with different inclusion rates of patients to the trials. A Finnish Leukemia Group study.

    abstract::The purpose of this study was to analyse the influence of the hospital size, measured as the number of annual patient enrolments in the Finnish Leukaemia Group trials in the period 1979-85, on response rate and survival after conventional chemotherapy for multiple myeloma. The 432 myeloma patients for this study were ...

    journal_title:European journal of haematology

    pub_type: 临床试验,杂志文章,多中心研究


    authors: Oivanen T,Kellokumpu-Lehtinen P,Koivisto AM,Koivunen E

    更新日期:1999-10-01 00:00:00

  • Treatment of 34 patients with myelodysplastic syndromes with 13-CIS retinoic acid.

    abstract::Thirty-four patients with myelodysplastic syndromes, 23 men and 11 women, aged between 47 and 80 years, with all types of myelodysplastic syndromes were treated with 13-cis-retinoic acid. The dose of retinoic acid ranged between 10 and 60 mg/m2/daily and was administered in combination with vitamin E to diminish side ...

    journal_title:European journal of haematology

    pub_type: 临床试验,杂志文章


    authors: Bourantas KL,Tsiara S,Christou L

    更新日期:1995-10-01 00:00:00

  • Effective treatment and prophylaxis of hyperuricemia and impaired renal function in tumor lysis syndrome with low doses of rasburicase.

    abstract:BACKGROUND:Tumor lysis syndrome (TLS) is a complication that can cause renal failure by precipitation of uric acid (UA) and phosphate crystals in renal tubules. Rasburicase proved to be effective in rapidly reducing UA levels. Costs of rasburicase average up to 4500 euros. To assess if lower doses of rasburicase are ef...

    journal_title:European journal of haematology

    pub_type: 杂志文章


    authors: Hummel M,Reiter S,Adam K,Hehlmann R,Buchheidt D

    更新日期:2008-04-01 00:00:00

  • Hypocholesterolemia in adult patients with thalassemia: a link with the severity of genotype in thalassemia intermedia patients.

    abstract:OBJECTIVES:Hypocholesterolemia has been previously described in patients affected by thalassemia. In this study we retrospectively evaluated the cholesterol level in two groups of patients affected by either thalassemia major (TM) or thalassemia intermedia (TI), with the aim of establishing factors correlated to hypoch...

    journal_title:European journal of haematology

    pub_type: 杂志文章


    authors: Ricchi P,Ammirabile M,Spasiano A,Costantini S,Di Matola T,Cinque P,Pagano L,Prossomariti L

    更新日期:2009-03-01 00:00:00

  • Regrowth of granulocyte-macrophage progenitor cells (GM-CFC) in suspension cultures of bone marrow depleted of GM-CFC with 4-hydroperoxycyclophosphamide (4-HC).

    abstract::The hematopoietic capacity of bone marrow treated with 4-hydroperoxycyclophosphamide (4-HC) was studied using long-term suspension cultures. It was shown that, in the presence of a previously established marrow adherent cell layer (ACL), the 4-HC treated bone marrow, deprived of GM-CFC, generates GM-CFC in long-term s...

    journal_title:European journal of haematology

    pub_type: 杂志文章


    authors: Beran M,Zander AR,Andersson BS,McCredie KB

    更新日期:1987-08-01 00:00:00

  • MicroRNAs as components of regulatory networks controlling erythropoiesis.

    abstract::Over the last two decades, the role of microRNAs has been extensively investigated, and it has become clear that these small non-coding RNAs play an essential role in several biological processes including erythropoiesis and that their dysregulation is associated with pathologies. Recent technical innovations have con...

    journal_title:European journal of haematology

    pub_type: 杂志文章,评审


    authors: Azzouzi I,Schmugge M,Speer O

    更新日期:2012-07-01 00:00:00

  • Protein deficiency balance as a predictor of clinical outcome in hereditary spherocytosis.

    abstract::Vertical and horizontal interactions between membrane constituents account for integrity, strength and deformability of the erythrocyte. Disruption of vertical interactions caused by membrane protein deficiencies in hereditary spherocytosis (HS), favor membrane vesiculation with development of spherocytic cells. Our a...

    journal_title:European journal of haematology

    pub_type: 杂志文章


    authors: Rocha S,Rebelo I,Costa E,Catarino C,Belo L,Castro EM,Cabeda JM,Barbot J,Quintanilha A,Santos-Silva A

    更新日期:2005-05-01 00:00:00

  • Bone marrow histopathology in primary myelofibrosis: clinical and haematologic correlations and prognostic evaluation.

    abstract::In 51 patients with primary myelofibrosis the initial bone marrow biopsy findings were evaluated by morphometric methods, correlated with the patients' main clinical and haematologic data and analysed for prognostic significance. The high variability of the marrow features was the most striking finding of the morpholo...

    journal_title:European journal of haematology

    pub_type: 杂志文章


    authors: Pereira A,Cervantes F,Brugues R,Rozman C

    更新日期:1990-02-01 00:00:00

  • Comparison of poly- and monoclonal antibodies for determination of B-cell clonal excess in an routine clinical laboratory.

    abstract::Flow cytometry (FCM) has gained wide use in the determination of clonality in B-cell lymphoproliferative diseases and many methodological variations exist. We have compared the suitability of a) dual fluorochrome (FITC/PE)-labelled monoclonal antibodies, b) single fluorochrome (FITC)-labelled monoclonal antibodies and...

    journal_title:European journal of haematology

    pub_type: 杂志文章


    authors: Agrawal YP,Hämäläinen E,Mahlamäki EK,Aho H,Nousianinen T,Lahtinen R,Penttilä IM

    更新日期:1992-01-01 00:00:00

  • The frequently low cobalamin levels in dementia usually signify treatable metabolic, neurologic and electrophysiologic abnormalities.

    abstract::Cobalamin levels are frequently low in patients with dementia, but it is unclear if they represent definable deficiency and what the mechanisms are. Therefore, patients being evaluated for dementia who had low cobalamin levels but no obvious evidence of deficiency were studied hematologically, neurologically and with ...

    journal_title:European journal of haematology

    pub_type: 杂志文章


    authors: Carmel R,Gott PS,Waters CH,Cairo K,Green R,Bondareff W,DeGiorgio CM,Cummings JL,Jacobsen DW,Buckwalter G

    更新日期:1995-04-01 00:00:00

  • Point mutation in the band 4.2 gene associated with autosomal recessively inherited erythrocyte band 4.2 deficiency.

    abstract::A patient who represented acute hemolytic crisis was studied. Analysis of the erythrocyte membrane proteins by SDS-PAGE revealed a deficiency of band 4.2. In the family, the sister of the patient who had been clinically normal was also shown to be deficient in band 4.2. Binding studies showed that the propositus' memb...

    journal_title:European journal of haematology

    pub_type: 杂志文章


    authors: Iwamoto S,Kajii E,Omi T,Kamesaki T,Akifuji Y,Ikemoto S

    更新日期:1993-05-01 00:00:00

  • Allogeneic bone marrow vs. peripheral blood stem cell transplantation: a long-term retrospective single-center analysis in 329 patients.

    abstract:OBJECTIVES:Granulocyte colony-stimulating factor-mobilized peripheral blood hematopoietic stem cell transplantation (HSCT) provides a valuable and increasingly used alternative to bone marrow transplantation (BMT). This retrospective study aimed at determining whether the stem cell source is predictive for outcome, rel...

    journal_title:European journal of haematology

    pub_type: 杂志文章


    authors: Auberger J,Clausen J,Kircher B,Kropshofer G,Lindner B,Nachbaur D

    更新日期:2011-12-01 00:00:00

  • Rapid infusion of rituximab over 60 min.

    abstract::The use of rituximab is increasing and regular administration over 2 to 3 h requires considerable healthcare resources and is inconvenient for patients. There is interest in reducing rituximab administration times and although infusion of rituximab over 90 min is safe, there is limited data on the safety of 60 min inf...

    journal_title:European journal of haematology

    pub_type: 杂志文章


    authors: Tuthill M,Crook T,Corbet T,King J,Webb A

    更新日期:2009-04-01 00:00:00

  • Dual rearrangement of immunoglobulin and T-cell receptor gene in a case of T-cell hairy-cell leukemia.

    abstract::We report a case of T-cell hairy-cell leukemia with a dual rearrangement of Ig- and T-cell receptor genes. The cytochemical, transmission electron microscopy, and surface antigens data (CD3+, CD8+, CD11+, HLA-DR+, CD19-, CD20-) were consistent with a T-cell hairy-cell leukemia. Molecular analysis according to Southern...

    journal_title:European journal of haematology

    pub_type: 杂志文章


    authors: Palumbo AP,Corradini P,Battaglio S,Omedè P,Coda R,Boccadoro M,Pileri A

    更新日期:1991-02-01 00:00:00

  • Chemotherapy followed by modified donor lymphocyte infusion as a treatment for relapsed acute leukemia after haploidentical hematopoietic stem cell transplantation without in vitro T-cell depletion: superior outcomes compared with chemotherapy alone and a

    abstract::We retrospectively compared the antileukemic effects of chemotherapy alone and chemotherapy followed by modified donor lymphocyte infusion (DLI) in 82 patients with relapsed acute leukemia after haploidentical hematopoietic stem cell transplantation (HSCT) without in vitro T-cell depletion. We also investigated progno...

    journal_title:European journal of haematology

    pub_type: 杂志文章


    authors: Yan CH,Wang JZ,Liu DH,Xu LP,Chen H,Liu KY,Huang XJ

    更新日期:2013-10-01 00:00:00