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

abstract：OBJECTIVES:As new, effective therapies emerge for acute lymphoblastic leukaemia (ALL), the results of clinical trials need to relate to standard of care. METHODS:We used the population-based Swedish ALL Registry to evaluate characteristics, treatment and long-term outcome in 933 patients with diagnosis between 1997 an...

journal_title：European journal of haematology

authors： Lennmyr E,Karlsson K,Ahlberg L,Garelius H,Hulegårdh E,Izarra AS,Joelsson J,Kozlowski P,Moicean A,Tomaszewska-Toporska B,Lübking A,Hallböök H,Swedish Adult Acute Lymphoblastic Leukaemia Group (SVALL).

更新日期：2019-08-01 00:00:00

abstract：:Very high-dose chemotherapy with autologous blood stem cell (BSC) rescue becomes more and more widely performed. In order to simplify the technique, a large volume apheresis programme combined with an uncontrolled rate cryopreservation at -80 degrees C was developed. Twenty-six patients suffering from multiple myeloma...

journal_title：European journal of haematology

authors： Feremans WW,Bastin G,Moine FL,Ravoet C,Delville JP,Pradier O,Wallef G,Dupont E,Capel P,Lambermont M

更新日期：1996-05-01 00:00:00

abstract：OBJECTIVE:To describe a case of venlafaxine-induced ecchymoses. METHODS:A patient with a history of ecchymoses coincident with venlafaxine therapy was rechallenged with the drug. Her platelet function was assessed with aggregation and ATP release studies before the rechallenge and after she developed ecchymoses. In ad...

journal_title：European journal of haematology

authors： Sarma A,Horne MK 3rd

更新日期：2006-12-01 00:00:00

abstract：OBJECTIVES:To compare the gene expression pattern in children and adults with acute lymphoblastic leukaemia (ALL) in order to improve our understanding of the difference in disease biology and prognosis. METHODS:The gene expression profiles in diagnostic samples from 29 children and 15 adults with ALL were analysed us...

journal_title：European journal of haematology

authors： Kuchinskaya E,Heyman M,Grandér D,Linderholm M,Söderhäll S,Zaritskey A,Nordgren A,Porwit-Macdonald A,Zueva E,Pawitan Y,Corcoran M,Nordenskjöld M,Blennow E

更新日期：2005-06-01 00:00:00

abstract：OBJECTIVES:In patients with essential thrombocythemia (ET), vascular complications contribute to both morbidity and mortality. To better predict the occurrence of thrombotic events, an International Prognostic Score of thrombosis for ET (IPSET-thrombosis) was recently developed. We hereby presented an external validati...

journal_title：European journal of haematology

authors： Fu R,Xuan M,Lv C,Zhang L,Li H,Zhang X,Zhang D,Sun T,Xue F,Liu X,Liang H,Zhang L,Yang R

更新日期：2014-06-01 00:00:00

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

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

更新日期：1999-10-01 00:00:00

abstract：:The therapeutic management of patients with idiopathic hemochromatosis (IH) requires an accurate estimate of hepatic iron overload in order to prevent tissue fibrosis and organ failure. Magnetic resonance imaging (MRI) was used to estimate liver iron overload in 5 patients with IH and in 8 normal controls. Signal inte...

journal_title：European journal of haematology

authors： Andersen PB,Birgegård G,Nyman R,Hemmingsson A

更新日期：1991-09-01 00:00:00

abstract：:Older patients with haemophilia (PWH) face many challenges related not only to haemophilia but also to general comorbidities associated with ageing. This article discusses the clinical experience published about the high prevalence of diseases in older PWH. These conditions are managed in the general population by hea...

journal_title：European journal of haematology

authors： Canaro M,Goranova-Marinova V,Berntorp E

更新日期：2015-02-01 00:00:00

abstract：OBJECTIVES:To assess the molecular basis of phenotypic heterogeneity in north Indian patients with thalassemia major (TM). METHODS:To determine the clinical severity, 130 patients of TM were studied for the age of first presentation and frequency of blood transfusion. The type of beta mutations, Xmn-1(G)gamma polymorp...

journal_title：European journal of haematology

authors： Sharma N,Das R,Kaur J,Ahluwalia J,Trehan A,Bansal D,Panigrahi I,Marwaha RK

更新日期：2010-06-01 00:00:00

abstract：BACKGROUND:Primary Hepatic (PHL) and Primary Splenic (PSL) non-Hodgkin's Lymphoma are rare entities. Small series of PHL and PSL have been reported, suggesting a non-fortuitous association with Hepatitis C Virus (HCV) infection. The prognosis is believed to be dismal, with early recurrence and short survival. PATIENTS...

journal_title：European journal of haematology

authors： De Renzo A,Perna F,Persico M,Notaro R,Mainolfi C,de Sio I,Ciancia G,Picardi M,Del Vecchio L,Pane F,Rotoli B

更新日期：2008-07-01 00:00:00

abstract：:The heat production rate in peripheral blood lymphocytes was evaluated by direct calorimetry in 76 untreated adults with non-Hodgkin's lymphoma (NHL). Elevated values were recorded for 20 out of 54 patients with lymphomas of high or intermediate malignancy grade (37%) and for 1 out of 22 patients (5%) with low grade l...

journal_title：European journal of haematology

authors： Monti M,Brandt L,Ikomi-Kumm J,Olsson H

更新日期：1990-11-01 00:00:00

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

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

更新日期：2012-12-01 00:00:00

abstract：:Cardiac disease is the primary cause of mortality in beta-thalassemia patients. Except for ventricular dysfunction and pulmonary hypertension that represent the main forms of heart disease in these patients, valvular abnormalities including valvular regurgitation, endocardial thickening and calcification and mitral va...

journal_title：European journal of haematology

authors： Farmakis D,Deftereos S,Giakoumis A,Polymeropoulos E,Aessopos A

更新日期：2004-04-01 00:00:00

abstract：:In groups of 26 patients with myeloproliferative disorders (MPD), 8 with chronic myelogenous leukaemia (CML); 8 with polycythaemia vera (PV); 10 with essential thrombocythaemia (ET); and 6 patients with reactive thrombocytosis (RT), we studied the growth characteristics of bone marrow CFU-M in agar culture. The bone m...

journal_title：European journal of haematology

authors： Hamaguchi H,Takano N,Sakamaki H,Enokihara H,Saito K,Furusawa S,Shishido H

更新日期：1988-08-01 00:00:00

abstract：INTRODUCTION:Increased levels of Wilms' tumor (WT1) mRNA have been used to establish risk categories in patients with acute myeloid leukemia (AML). Raised values of WT1 have been associated with progression in myelodysplastic syndrome (MDS). METHODS:We retrospectively analyzed the available bone marrow (BM) samples fr...

journal_title：European journal of haematology

authors： Santaliestra M,Garrido A,Carricondo M,Bussaglia E,Pratcorona M,Blanco ML,Gich I,Hoyos M,Esquirol A,García-Cadenas I,Brunet S,Martino R,Sierra J,Nomdedéu JF

更新日期：2019-09-01 00:00:00

abstract：OBJECTIVES:The objective of this study was to compare the major transplant outcomes between patients receiving hematopoietic stem cell transplantation (HSCT) from bone marrow (BM) or peripheral blood stem cells (PBSC). METHODS:All consecutive HSCT patients using BM or PBSC from an HLA-matched related donors for haemat...

journal_title：European journal of haematology

authors： Campregher PV,Hamerschlak N,Colturato VA,Mauad MA,de Souza MP,Bouzas LF,Tavares Rde C,Barros JC,Chiattone R,Paz A,Silla L,Vigorito AC,Miranda E,Funke VA,Flowers ME

更新日期：2015-11-01 00:00:00

abstract：OBJECTIVES:This study aims to characterize the epidemiology of immunocompetent Primary central nervous system lymphoma (PCNSL) diagnosed 2000-2013 in Sweden. METHODS:Cases were identified in the population-based Swedish Lymphoma Register. Incidence per 100 000 person-years and 95% confidence intervals (CI) were calcul...

journal_title：European journal of haematology

authors： Eloranta S,Brånvall E,Celsing F,Papworth K,Ljungqvist M,Enblad G,Ekström-Smedby K

更新日期：2018-01-01 00:00:00

abstract：:In acute myeloid leukemia (AML) mouse models, the RUNX1-RUNX1T1 fusion protein has failed to produce leukemia by itself, but alternative splicing of exon 9a of the RUNX1-RUNX1T1 fusion transcript (FT) has recently been shown to enhance the leukemogenic potential. We have analyzed 138 diagnosis and follow-up samples fr...

journal_title：European journal of haematology

authors： Ommen HB,Ostergaard M,Yan M,Braendstrup K,Zhang DE,Hokland P

更新日期：2010-02-01 00:00:00

abstract：:This report describes a family with TUBB1-associated macrothrombocytopenia diagnosed based on abnormal platelet β1-tubulin distribution. A circumferential marginal microtubule band was undetectable, whereas microtubules were frayed and disorganized in every platelet from the affected individuals. Patients were heteroz...

journal_title：European journal of haematology

authors： Kunishima S,Nishimura S,Suzuki H,Imaizumi M,Saito H

更新日期：2014-04-01 00:00:00

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

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

abstract：:We have studied the kinetics of plasma levels of circulating (c)selectins in 8 patients undergoing bone marrow or stem cell transplantation to gain estimates for the distribution and half-life of (c)selectins and to potentially identify an endothelial source of cP-selectin in patients who are deprived of platelets and...

journal_title：European journal of haematology

authors： Jilma B,Eichler HG,Becherer A,Brugger S,Kalhs P,Stohlawetz P,Kapiotis S,Kapiotis S

更新日期：1998-07-01 00:00:00

abstract：INTRODUCTION:Primary and post-ET/PV myelofibrosis are myeloproliferative neoplasms harboring in most cases driving mutations in JAK2, CALR or MPL, and a variable number of additional mutations in other genes. Molecular analysis represents a powerful tool to guide prognosis and clinical management. Only about 10% of pat...

journal_title：European journal of haematology

authors： Mannina D,Gagelmann N,Badbaran A,Ditschkowski M,Bogdanov R,Robin M,Cassinat B,Heuser M,Shahswar R,Thol F,Beelen D,Kröger N

更新日期：2019-12-01 00:00:00

abstract：UNLABELLED:Protein C (PC) deficiency is an autosomal dominant inherited disorder associated with spontaneous and recurrent thrombotic events. Factor V Leiden (FVL) increases the risk of thrombosis in PC-deficient type I families. We have investigated the relationship between PC deficiency genotype and clinical phenotyp...

journal_title：European journal of haematology

authors： Cafolla A,D'Andrea G,Baldacci E,Margaglione M,Mazzucconi MG,Foà R

更新日期：2012-04-01 00:00:00

abstract：OBJECTIVE:We report an extension study of patients with essential thrombocythaemia (ET) in the Hungarian Myeloproliferative Neoplasm (HUMYPRON) Registry, which demonstrated that over 6 years anagrelide significantly decreased the number of patients experiencing minor arterial and minor venous thrombotic events (TEs) vs...

journal_title：European journal of haematology

authors： Kellner A,Dombi P,Illes A,Demeter J,Homor L,Ercsei I,Simon Z,Karadi E,Herczeg J,Gy Korom V,Gasztonyi Z,Szerafin L,Udvardy M,Egyed M

更新日期：2020-10-01 00:00:00

abstract：:Development of resistance to cytotoxic agents is a common problem in the treatment of acute leukemia. In cell lines having multidrug resistance (MDR) phenotype, a decrease in the intracellular accumulation of drugs has been closely related to the overexpression of P-glycoprotein/mdr1 genes. We analyzed the relationshi...

journal_title：European journal of haematology

authors： Kato S,Ideguchi H,Muta K,Nishimura J,Nawata H

更新日期：1991-08-01 00:00:00

abstract：:15 cases of acute leukemia (AL) displaying a TdT+, HLA-DR+ phenotype were studied; surface immunoglobulins, T cell markers and the common acute lymphoblastic leukaemia (c-ALL) antigen were negative, as were peroxidase and non-specific esterase cytochemical reactions. All cases were extensively investigated by conventi...

journal_title：European journal of haematology

authors： Basso G,Putti MC,Catoretti G,Consolini R,Galdiolo D,Guglielmi C,Messina C,Milanesi C,Testi AM,Xillo MZ

更新日期：1987-02-01 00:00:00

abstract：:Hemoglobinopathies are the most common autosomal recessive disorders and are mostly inherited in a recessive manner. However, certain mutations can affect the globin chain stability, leading to dominant forms of thalassemia. The aim of this work was the molecular and structural characterization of two heterozygous in-...

journal_title：European journal of haematology

authors： Scheps KG,Hasenahuer MA,Parisi G,Targovnik HM,García E,Veber ES,Crisp R,Elena G,Varela V,Fornasari MS

更新日期：2018-06-01 00:00:00

abstract：:Upshaw-Schulman syndrome (USS) is due to severe congenital deficiency of von Willebrand factor (VWF)-cleaving protease ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 domains, nr 13) activity resulting in the presence of unusually large forms of VWF in the circulation, causing intravascular plat...

journal_title：European journal of haematology

authors： Rank CU,Kremer Hovinga J,Taleghani MM,Lämmle B,Gøtze JP,Nielsen OJ

更新日期：2014-02-01 00:00:00

abstract：:Cells from 3 patients with B-chronic lymphocytic leukaemia (B-CLL) and 1 with B-prolymphocytic leukaemia (B-PLL) were treated in vitro with the phorbol ester 12-0-tetradecanoylphorbol (TPA), the calcium ionophore A23187, or a combination of TPA and A23187. TPA induced the cells to adhere to the culture flask or to clu...

journal_title：European journal of haematology

authors： Drexler HG,Brenner MK,Gignac SM,Hoffbrand AV

更新日期：1988-09-01 00:00:00

abstract：:6 patients with massive splenomegaly but no peripheral adenopathy were diagnosed by splenectomy as having lymphoma; and were found to have widespread disease. Despite the seriousness of their illness at presentation, all have survived for a median time of 101.5 months and reached complete remission. ...

journal_title：European journal of haematology

authors： King DJ,Dawson AA,Thompson WD

更新日期：1987-02-01 00:00:00