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












  • Role of allogeneic stem cell transplantation in mantle cell lymphoma.

    abstract::Despite a wide spectrum of treatment options, mantle cell lymphoma (MCL) remains a challenging hematologic malignancy to manage. Advances in front-line therapy, including the monoclonal antibody rituximab and increasing use of cytarabine, have improved remission rates. Autologous hematopoietic cell transplantation (HC...

    journal_title:European journal of haematology

    pub_type: 杂志文章,评审


    authors: Cohen JB,Burns LJ,Bachanova V

    更新日期:2015-04-01 00:00:00

  • Hydrocortisone modulates colony-stimulating activity produced by human bone marrow-derived adherent cells.

    abstract::In an attempt to clarify the significance of hydrocortisone (HC) in human long-term bone marrow cultures, the production of colony-stimulating activity (CSA) and colony-enhancing activity (CEA) by human bone marrow-derived adherent cells (MDAC) and the modulation by HC were examined. The CSA production by MDAC was dem...

    journal_title:European journal of haematology

    pub_type: 杂志文章


    authors: Hirata J,Kaneko S,Nishimura J,Motomura S,Ibayashi H

    更新日期:1988-03-01 00:00:00

  • Disseminated intravascular coagulation in acute leukemia: clinical and laboratory features at presentation.

    abstract:BACKGROUND:Although there are two major scoring systems for the clinical diagnosis of disseminated intravascular coagulation (DIC), the validity of these systems for leukemia-associated DIC remains to be confirmed. METHODS:By analyzing 125 newly diagnosed acute leukemia patients, we investigated clinical and laborator...

    journal_title:European journal of haematology

    pub_type: 杂志文章


    authors: Yanada M,Matsushita T,Suzuki M,Kiyoi H,Yamamoto K,Kinoshita T,Kojima T,Saito H,Naoe T

    更新日期:2006-10-01 00:00:00

  • ZAP-70 mRNA quantification in B-cell chronic lymphocytic leukaemia.

    abstract:OBJECTIVE:The mutational status of the immunoglobulin (Ig) V(H) gene in B-cell chronic lymphocytic leukaemia (B-CLL) identifies two subgroups of patients with significantly different outcomes. We investigated the association of ZAP-70 expression with IgVH mutational status in B-CLL by quantifying ZAP-70 mRNA, to evalua...

    journal_title:European journal of haematology

    pub_type: 临床试验,杂志文章


    authors: Catherwood MA,Matthews C,Niblock R,Dobbin E,Morris TC,Alexander HD

    更新日期:2006-04-01 00:00:00

  • Fibroblast colony-forming cells with high sensitivity to serum mitogen(s) exist in bone marrow of patients with chronic myelocytic leukemia.

    abstract::Marrow fibroblast colony formation was studied in patients with myeloproliferative disorders, using human serum or platelet-derived growth factor plus plasma-derived serum as growth-stimulating factors. Colony numbers negatively correlated with the patient's age, but were not different from those of controls. However,...

    journal_title:European journal of haematology

    pub_type: 杂志文章


    authors: Kimura A,Katoh O,Hyodo H,Kusumi S,Kuramoto A

    更新日期:1990-05-01 00:00:00

  • Challenges of multiple mutations in individual patients with haemophilia.

    abstract::Multiple mutations in the same gene within a haemophilia family are being increasingly reported and raise many issues with regard to the specificity of the mutations in causing the disease. In a proportion of families with multiple mutations, discordant phenotypic severity is often observed among the affected members....

    journal_title:European journal of haematology

    pub_type: 杂志文章,评审


    authors: Shetty S,Bhave M,Ghosh K

    更新日期:2011-03-01 00:00:00

  • A comparison of Campath and Thymoglobulin as part of the conditioning before allogeneic hematopoietic stem cell transplantation.

    abstract:BACKGROUND:In vivo T-cell depletion with anti-thymocyte globulin is a commonly used strategy for the prevention of graft-versus-host disease (GVHD) and to avoid rejection after hematopoietic stem cell transplantation (HSCT). METHODS:We compared 36 patients given Campath (alemtuzumab) as part of the conditioning with a...

    journal_title:European journal of haematology

    pub_type: 杂志文章


    authors: Norlin AC,Remberger M

    更新日期:2011-01-01 00:00:00

  • Cost analysis of CHOP (-like) chemotherapy regimens for patients with newly diagnosed aggressive non-Hodgkin's lymphoma.

    abstract::Many cost analyses of stem-cell transplantations are available, which is in sharp contrast to the level of cost analyses on first-line chemotherapy for aggressive non-Hodgkin's lymphoma (NHL). Given the scarcity of cost analyses of first-line chemotherapy for NHL, it is difficult to assess the economic impact of upcom...

    journal_title:European journal of haematology

    pub_type: 杂志文章


    authors: van Agthoven M,Faber LM,Uyl-de Groot CA,Sonneveld P,Verdonck LF,Willemze R,Kluin-Nelemans JC,Löwenberg B,Huijgens PC

    更新日期:2002-10-01 00:00:00

  • Paraneoplastic cerebellar degeneration and nephrotic syndrome preceding Hodgkin's disease: case report and review of the literature.

    abstract::A patient presented with symptoms of cerebellar degeneration and nephrotic syndrome. A work-up at that time failed to reveal an underlying disease; however, 20 months later Hodgkin's disease was diagnosed. Hodgkin's lymphadenopathy developed 2 wk after prednisone therapy for the nephrotic syndrome had been discontinue...

    journal_title:European journal of haematology

    pub_type: 杂志文章,评审


    authors: Spyridonidis A,Fischer KG,Glocker FX,Fetscher S,Klisch J,Behringer D

    更新日期:2002-05-01 00:00:00

  • An Arg545----Cys545 substitution mutation of the von Willebrand factor in type IIB von Willebrand's disease.

    abstract::Type IIB is a special variant of von Willebrand's disease, characterized by an abnormal von Willebrand factor which shows an increased interaction with platelets. This interaction sometimes causes platelet aggregation and thrombocytopenia in vivo. It involves the glycoprotein-Ib (GPIb) receptor on platelets and corres...

    journal_title:European journal of haematology

    pub_type: 杂志文章


    authors: Donnér M,Andersson AM,Kristoffersson AC,Nilsson IM,Dahlbäck B,Holmberg L

    更新日期:1991-11-01 00:00:00

  • Intensive remission induction therapy for chronic myeloid leukemia in blast phase with a goal of post-remission bone marrow transplant--a pilot study.

    abstract::An intensive protocol utilizing mitoxantrone, high-dose cytarabine, vincristine, etoposide and methylprednisolone as induction therapy for chronic myeloid leukemia in blast transformation is described. Fourteen patients were treated, with a remission/second chronic phase achieved in 64%. None of the 3 patients older t...

    journal_title:European journal of haematology

    pub_type: 杂志文章


    authors: Lipton JH,Messner HA,Curtis JE,Atkins HL,Minden MD

    更新日期:1996-07-01 00:00:00

  • Incidence of bacterial and fungal infections in newly diagnosed acute myeloid leukaemia patients younger than 65 yr treated with induction regimens including fludarabine: retrospective analysis of 224 cases.

    abstract:OBJECTIVES:Infections are the major cause of morbidity and mortality in patients with acute myeloid leukaemia (AML). They primarily occur during the first course of induction chemotherapy and may increase the risk of leukaemia relapse, due to a significant delay in consolidation therapy. The intensification of inductio...

    journal_title:European journal of haematology

    pub_type: 杂志文章,多中心研究


    authors: Malagola M,Peli A,Damiani D,Candoni A,Tiribelli M,Martinelli G,Piccaluga PP,Paolini S,De Rosa F,Lauria F,Bocchia M,Gobbi M,Pierri I,Zaccaria A,Zuffa E,Mazza P,Priccolo G,Gugliotta L,Bonini A,Visani G,Skert C,Ber

    更新日期:2008-11-01 00:00:00

  • Accumulation of gamma-globin mRNA in human erythroid cells treated with angelicin.

    abstract::The aim of the present study was to determine whether angelicin is able to increase the expression of gamma-globin genes in human erythroid cells. Angelicin is structurally related to psoralens, a well-known chemical class of photosensitizers used for their antiproliferative activity in treatment of different skin dis...

    journal_title:European journal of haematology

    pub_type: 杂志文章


    authors: Lampronti I,Bianchi N,Borgatti M,Fibach E,Prus E,Gambari R

    更新日期:2003-09-01 00:00:00

  • A novel gene STORP (STOmatin-Related Protein) is localized 2 kb upstream of the promyelocytic gene on chromosome 15q22.

    abstract::We generated a 100-kb map of the region 5' of the PML (promyelocytic leukemia) gene on human chromosome 15q22 and identified a new gene provisionally named STORP for stomatin-related protein. The STORP gene is positioned 2 kb upstream of the PML gene in a head-to-head configuration, and contains 7 exons spanning a gen...

    journal_title:European journal of haematology

    pub_type: 杂志文章


    authors: Gilles F,Glenn M,Goy A,Remache Y,Zelenetz AD

    更新日期:2000-02-01 00:00:00

  • Effects of interleukin-3 following chemotherapy of non-Hodgkin's lymphoma. A prospective, controlled phase I/II study.

    abstract::The effect of rhIL-3 was investigated in 32 patients with newly diagnosed non-Hodgkin lymphoma in a phase I/II trial. All patients received 6 cycles of standard CHOP chemotherapy, and each patient was his own control where rhIL-3 was given as a daily s.c. injection for 14 days (day 2-15) in cycle 2 and 4, while cycle ...

    journal_title:European journal of haematology

    pub_type: 临床试验,杂志文章


    authors: Hovgaard DJ,Nissen NI

    更新日期:1995-02-01 00:00:00

  • Hepcidin-25 vs. conventional clinical biomarkers in the diagnosis of functional iron deficiency.

    abstract:OBJECTIVES:As hepcidin-25 is considered as a key regulator of human iron homoeostasis, this study aimed to compare this parameter with conventional biomarkers and diagnostic tools of iron deficiency (ID). METHODS:In total, 233 hospitalised adult patients, who underwent routine blood testing for ID, were included. All ...

    journal_title:European journal of haematology

    pub_type: 杂志文章


    authors: Enko D,Wagner H,Kriegshäuser G,Kimbacher C,Stolba R,Worf E,Halwachs-Baumann G

    更新日期:2015-12-01 00:00:00

  • Engraftment of human myelodysplastic syndrome derived cell line in transgenic severe combined immunodeficient (TG-SCID) mice expressing human GM-CSF and IL-3.

    abstract::A transgenic SCID (TG-SCID) mouse expressing the human cytokines interleukin-3 (IL-3) and granulocyte-macrophage colony-stimulating factor (GM-CSF) has been generated with the aim of making a model system allowing the in vivo proliferation of human hematopoietic cells. Using TG-SCID mice expressing high levels (30-35 ...

    journal_title:European journal of haematology

    pub_type: 杂志文章


    authors: Kim DK,Kojima M,Fukushima T,Miyasaka M,Nakauchi H

    更新日期:1998-08-01 00:00:00

  • Prognostic role of immunohistochemical analysis of 5 mc in myelodysplastic syndromes.

    abstract:BACKGROUND:Aberrant DNA methylation at CpG islands within promoters is increasingly recognised as a common event in human cancers and has been associated with the silencing of important tumour suppressor genes. Epigenetic therapy using hypomethylating agents has demonstrated clinical effectiveness; the drugs azacitidin...

    journal_title:European journal of haematology

    pub_type: 杂志文章


    authors: Poloni A,Goteri G,Zizzi A,Serrani F,Trappolini S,Costantini B,Mariani M,Olivieri A,Catarini M,Centurioni R,Alesiani F,Giantomassi F,Stramazzotti D,Biagetti S,Alfonsi S,Berardinelli E,Leoni P

    更新日期:2013-09-01 00:00:00

  • Congenital haemolytic anaemia in a low birth weight infant due to congenital stomatocytosis.

    abstract::A baby girl born at 31 weeks gestation showed severe haemolytic anaemia and hyperbilirubinaemia which led to exchange transfusion within the first 12 hours of life. There was no blood group incompatibility between mother and child but there was a marked stomatocytosis of the baby's red blood cells. Family history reve...

    journal_title:European journal of haematology

    pub_type: 杂志文章


    authors: Huppi PS,Ott P,Amato M,Schneider H

    更新日期:1991-07-01 00:00:00

  • Dose-escalated CHOP and tailored intensification with IFE according to early response and followed by BEAM/autologous stem-cell transplantation in poor-risk aggressive B-cell lymphoma: a prospective study from the GEL-TAMO Study Group.

    abstract:OBJECTIVES:The role of high-dose therapy and autologous stem-cell transplantation (HDT/ASCT) in the up-front treatment of poor-risk aggressive lymphoma is still unknown. We conducted a prospective multi-centre trial with dose-escalated CHOP (MegaCHOP) and tailored intensification prior to HDT/ASCT according to early re...

    journal_title:European journal of haematology

    pub_type: 杂志文章,多中心研究,随机对照试验


    authors: Arranz R,Conde E,Grande C,Mateos MV,Gandarillas M,Albo C,Lahuerta JJ,Fernández-Rañada JM,Hernández MT,Alonso N,García Vela JA,Garzón S,Rodríguez J,Caballero D,Grupo Español de Linfomas\/Trasplante Autólogo de Médula Osea (GEL

    更新日期:2008-03-01 00:00:00

  • A case of nodular sclerosis Hodgkin's lymphoma repeatedly relapsing in the context of composite plasma cell-hyaline vascular Castleman's disease: successful response to rituximab and radiotherapy.

    abstract::We report the case of an Epstein-Barr virus (EBV)- and human immunodeficiency virus-serum negative patient suffering from repeatedly relapsing classical Hodgkin's Lymphoma (cHL) associated with a histological picture of plasma cell-hyaline vascular (PC-HV) form of Castleman's disease (CD). The CD30- and CD15-positive,...

    journal_title:European journal of haematology

    pub_type: 杂志文章


    authors: Falchi L,Capello D,Palumbo B,Rauco A,Emili R,Cianciulli M,Pace R,Capparella V,Liberati F,Liberati AM

    更新日期:2007-11-01 00:00:00

  • Discrepant ratios of arterial vs. venous thrombosis in hemophilias A and B as compared to FVII deficiency.

    abstract:BACKGROUND:The occurrence of a thrombotic event in congenital bleeding disorders has drawn considerable attention in recent years. Both patients with hemophilia and patients with von Willebrand disease and even those with rare coagulation disorders have been shown to present occasional thrombotic events. Little is know...

    journal_title:European journal of haematology

    pub_type: 杂志文章


    authors: Girolami A,Berti de Marinis G,Bertozzi I,Peroni E,Tasinato V,Lombardi AM

    更新日期:2013-08-01 00:00:00

  • An unusual case of CD4+ CD7+ CD56+ acute leukemia with overlapping features of type 2 dendritic cell (DC2) and myeloid/NK cell precursor acute leukemia.

    abstract::Type 2 dendritic cell (DC2) acute leukemia has been recently described. We report here an unusual case of a 17-yr-old adolescent with overlapping features of DC2 and myeloid/NK cell precursor acute leukemia as defined by Suzuki et al. The patient presented with lymphadenopathy and hepatosplenomegaly without extranodal...

    journal_title:European journal of haematology

    pub_type: 杂志文章


    authors: Anargyrou K,Paterakis G,Boutsis D,Politou M,Papadhimitriou SI,Siakandaris M,Vassiliadis J,Androulakis A,Meletis J,Rombos J,Tassiopoulou A,Vaiopoulos G

    更新日期:2003-10-01 00:00:00

  • Long-term complication in follicular lymphoma: assessing the risk of secondary neoplasm in 242 patients treated or not with 90-yttrium-ibritumomab-tiuxetan.

    abstract:BACKGROUND:Non-Hodgkin lymphoma patients have a 25% increased risk of secondary primary neoplasms (SPNs). Regarding the controversy about the increased risk of SPN in patients exposed to radioimmunotherapy (RIT), we have analyzed this issue in a cohort of follicular lymphoma (FL) patients treated with/without RIT. PAT...

    journal_title:European journal of haematology

    pub_type: 杂志文章


    authors: Andrade-Campos MM,Liévano P,Espinosa-Lara N,Soro-Alcubierre G,Grasa-Ulrich JM,López-Gómez L,Baringo T,Giraldo P

    更新日期:2016-12-01 00:00:00

  • Hodgkin's disease and age.

    abstract::506 unselected, previously untreated patients with Hodgkin's disease were treated at the Finsen Institute between 1969 and 1983. The prognostic significance of age, sex, stage, systemic symptoms, histologic subtype, number of involved nodal regions, total tumour burden (peripheral + intrathoracic nodal tumour burden, ...

    journal_title:European journal of haematology

    pub_type: 杂志文章


    authors: Specht L,Nissen NI

    更新日期:1989-08-01 00:00:00

  • Human immunodeficiency virus-associated malignant lymphoma in eastern Denmark diagnosed from 1990-1996: clinical features, histopathology, and association with Epstein-Barr virus and human herpesvirus-8.

    abstract::The clinicopathological features of human immunodeficiency virus (HIV)-associated lymphoma were investigated in a retrospective study of 85 adult patients in eastern Denmark diagnosed during the period 1990-1996. The possible pathogenetic role of Epstein-Barr virus (EBV) and human herpesvirus 8 (HHV-8) in these tumour...

    journal_title:European journal of haematology

    pub_type: 杂志文章


    authors: Hansen PB,Penkowa M,Kirk O,Skinhøj P,Pedersen C,Lisse I,Kiss K,Zhou X,Hamilton-Dutoit SJ

    更新日期:2000-06-01 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

  • Labelling autologous platelets with 111In tropolonate for platelet kinetic studies: limitations imposed by thrombocytopenia.

    abstract::An in vitro method of radiolabelling platelets with 111In tropolonate in plasma has been devised enabling imaging and cell kinetic studies to be performed in patients with thrombocytopenia (TP) using autologous, rather than donor, platelets. Platelets from 10 TP patients, with platelet counts ranging from 4-91 x 10(9)...

    journal_title:European journal of haematology

    pub_type: 杂志文章


    authors: Danpure HJ,Osman S,Peters AM

    更新日期:1990-10-01 00:00:00

  • Aberrant promoter methylation of Dab2 gene in myelodysplastic syndrome.

    abstract:OBJECTIVES:Human Disabled-2 (Dab2), a putative tumor suppressor gene, is frequently down-regulated in human tumors. This study aims to explore the association between Dab2 methylation status and expression in newly diagnosed myelodysplastic syndrome (MDS) patients and patients who received 5-aza-2'-deoxycytidine (decit...

    journal_title:European journal of haematology

    pub_type: 杂志文章


    authors: Yang Y,Zhang Q,Xu F,Chang C,Li X

    更新日期:2012-12-01 00:00:00

  • Peripheral blood accessory cells modulate committed colony-forming units but not 5-week cobblestone-area-forming cell outgrowth from CD34+ cells.

    abstract::While cellular modulation in vitro of committed hematopoietic stem cell (HSC) growth has been known for some time, less is known about the effect of accessory cells (AC) on the growth of more immature HSC. We have examined the effect of peripheral blood (PB) AC on hematopoiesis by coculturing enriched PB CD34+ cells (...

    journal_title:European journal of haematology

    pub_type: 杂志文章


    authors: Olesen G,Tønder H,Palshof T,Hokland P

    更新日期:2002-02-01 00:00:00