Abstract:
:The development of an autoantibody to human Factor VIII is rare and presents many problems for diagnosis and treatment. We have seen several cases at our institution recently with widely heterogenous clinical and laboratory presentations. A wide range of treatment modalities were used in these cases with no gold standard of treatment or widely accepted guidelines existing. This has prompted us to examine all cases of this condition presenting at Fremantle Hospital over the last decade. We describe four cases which demonstrate the heterogeneity of this condition and its treatment and review the recent literature on the subject.
journal_name
Eur J Haematoljournal_title
European journal of haematologyauthors
Hall RL,Leahy MFdoi
10.1034/j.1600-0609.2001.00383.xsubject
Has Abstractpub_date
2001-03-01 00:00:00pages
206-9issue
3eissn
0902-4441issn
1600-0609pii
ejh383journal_volume
66pub_type
杂志文章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
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1111/ejh.13318
更新日期:2019-12-01 00:00:00
abstract:OBJECTIVES:To describe the clinical and biological features of a series of immunophenotypic variant of B-CLL (v-CLL) characterised by intermediate RMH score, in the absence of t(11;14)(q13;q32) in FISH analysis in comparison with a series of typical CLL. METHODS:We studied the clinical and biological features of 63 ca...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2010.01454.x
更新日期:2010-08-01 00:00:00
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
doi:10.1111/j.0902-4441.2006.t01-1-EJH2588.x
更新日期:2006-06-01 00:00:00
abstract::Treatment with recombinant erythropoietin (EPO) can alleviate anaemia in patients with myelodysplastic syndromes (MDS). The present study, based on a long-term follow-up of 68 MDS patients (26RA, 16 RAS, 26 RAEB) treated with EPO alone, pinpoints pre-treatment variables associated with response induction, response dur...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1034/j.1600-0609.2002.01530.x
更新日期:2002-03-01 00:00:00
abstract::Ibrutinib is an oral inhibitor of Bruton tyrosine kinase approved for the treatment of chronic lymphocytic leukaemia, mantle cell lymphoma and refractory Waldenstrom's disease. It increases progression-free survival, overall survival, response rate. The most frequent adverse reactions, are increased risk in of bleedin...
journal_title:European journal of haematology
pub_type:
doi:10.1111/ejh.13290
更新日期:2019-10-01 00:00:00
abstract:OBJECTIVES:Combined high-dose Interferon-alpha and psoralen plus ultraviolet A irradiation (PUVA) have been reported to be effective in the treatment of early mycosis fungoides (MF); however, our study is the first controlled prospective study in the literature exploring the activity and tolerability of the combination...
journal_title:European journal of haematology
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1111/j.1600-0609.2005.00497.x
更新日期:2005-08-01 00:00:00
abstract::Osteolytic lesions with activated osteoclast (OC) and suppressed osteoblast (OB) activity are characteristics of myeloma bone lesion. Recently, it has been shown that bortezomib treatment enhances OB function. To evaluate the effect of bortezomib on myeloma bone lesions, we performed bone scans, where increased uptake...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2010.01523.x
更新日期:2011-01-01 00:00:00
abstract::Chronic myelomonocytic leukemia (CMML) is a myelodysplastic/myeloproliferative neoplasm, characterized by persistent monocytosis. Due to the lack of unique surface markers expressed by neoplastic monocytes and the frequent CD34-negative blast immunophenotype, the diagnostic value of flow cytometric immunophenotyping (...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.12477
更新日期:2015-08-01 00:00:00
abstract:OBJECTIVE:The primary objective was to assess the effect of central nervous system involvement in acute myeloid leukemia (CNS-AML) on outcomes after allogeneic hematopoietic stem cell transplant (allo-HCT). The secondary objective was to assess the utility of pretransplant cerebrospinal fluid (CSF) assessment in AML. ...
journal_title:European journal of haematology
pub_type: 临床试验,杂志文章
doi:10.1111/ejh.13314
更新日期:2019-11-01 00:00:00
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
doi:10.1111/ejh.12129
更新日期:2013-08-01 00:00:00
abstract::Fas (APO-1/CD95) is a 45-kDa membrane protein which regulates apoptosis in many lymphoid cell types. In the present study, FAS expression was examined in primary leukemic cells from infants with acute lymphoblastic leukemia (ALL). The cells were resistant to apoptosis induction by an anti-FAS antibody and expressed ne...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1034/j.1600-0609.2003.00024.x
更新日期:2003-03-01 00:00:00
abstract::A panel of 14 monoclonal antibodies (McAb) against hematopoietic cell surface antigens was applied on mononuclear blood or bone marrow cells from 40 cases of acute leukemia in order to compare immunoenzymatic staining (IE) (alkaline phosphatase) of fixed cells with immunofluorescence staining (IF) of unfixed suspended...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1988.tb00884.x
更新日期:1988-08-01 00:00:00
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
doi:10.1111/ejh.12145
更新日期:2013-09-01 00:00:00
abstract:OBJECTIVES:Northern Norway consists of a multiethnic population of Sámi and non-Sámi. We evaluated iron status in these two groups with respect to gender, age and residence. METHODS:In 2002-2004, a cross-sectional study of health and living conditions in areas with both Sámi and Norwegian populations, SAMINOR, was per...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2007.00929.x
更新日期:2007-11-01 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
pub_type: 临床试验,杂志文章
doi:10.1111/j.1600-0609.1998.tb01058.x
更新日期:1998-07-01 00:00:00
abstract::Monoclonal antibodies 8A and 62B1, recognizing plasma cell-associated antigens, were covalently linked to saporin 6, a ribosome-inactivating protein similar to the A-chain of ricin. Both immunotoxins were tested on target human cell lines U266 and Raji, on non-target K562 cell line and on myeloid CFU-GM progenitors. T...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1989.tb00105.x
更新日期:1989-03-01 00:00:00
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
doi:10.1034/j.1600-0609.2000.9c119.x
更新日期:2000-05-01 00:00:00
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
doi:10.1111/j.1600-0609.1997.tb00966.x
更新日期:1997-09-01 00:00:00
abstract:BACKGROUND:Paroxysmal nocturnal hemoglobinuria (PNH) diagnostic guidelines recommend single-tube five- to six-color or two-tube four-color assays. PNH clones are detectable in only a fraction of patients at risk, and screening for new PNH cases can be complex and expensive. In this multicenter study, we have validated ...
journal_title:European journal of haematology
pub_type: 杂志文章,多中心研究
doi:10.1111/ejh.12885
更新日期:2017-07-01 00:00:00
abstract::Oxidative damage to erythrocytes in thalassaemia has been related to generation of free radicals by an excess of denaturated alpha- or beta-globin chains, intracellular iron overload and low concentration of normal haemoglobin (HGB). Two good indicators of such oxidative damage are the high red blood cell (RBC) malony...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1995.tb00705.x
更新日期:1995-11-01 00:00:00
abstract::It is well-known that uremic patients present prolonged bleeding times as a common complication. Factors responsible for this disorder have been extensively investigated. In order to elucidate the possible role of uremic middle molecules as responsible for the bleeding tendency observed in uremia, we have studied the ...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1987.tb00757.x
更新日期:1987-09-01 00:00:00
abstract::An unusual case having IgM monoclonal gammopathy with clinical and pathologic features of multiple myeloma (MM) in association with neutrophilia and nephrotic syndrome is reported. The patient showed lytic bone lesions, decreased IgG and IgA levels, Bence-Jones proteinuria, nephrotic proteinuria with edema, and histol...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2007.00869.x
更新日期:2007-07-01 00:00:00
abstract::We have previously shown that basic fibroblast growth factor (bFGF) stimulates megakaryocytopoiesis and granulopoiesis in vitro and that normal haematopoietic cells and several leukaemic cell lines express FGF receptors. In this paper, we demonstrate by reverse transcriptase-mediated polymerase chain reaction (RT-PCR)...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1995.tb00249.x
更新日期:1995-09-01 00:00:00
abstract::In secondary erythrocytosis, the elevated red cell count is powered by factors outside the erythroid compartment, for instance by raised erythropoietin (EPO) synthesis based on congenital defects of the oxygen-sensing pathway. The principal transcriptional regulator of EPO synthesis is endothelial PAS domain-containin...
journal_title:European journal of haematology
pub_type:
doi:10.1111/ejh.13241
更新日期:2019-07-01 00:00:00
abstract::During the period 1978-1982 in the three northernmost countries of Sweden all 143 patients with a registered diagnosis of chronic lymphocytic leukemia (CLL) were retrospectively analyzed. After re-evaluation, 122 cases remained in the study. The mean age was 71 years and the male/female ratio was 2.2:1. Sixty-one pati...
journal_title:European journal of haematology
pub_type: 杂志文章,多中心研究
doi:10.1111/j.1600-0609.1994.tb01295.x
更新日期:1994-02-01 00:00:00
abstract:OBJECTIVES:The benefits of combined chelation therapy with daily deferiprone (DFP) and subcutaneous desferrioxamine (DFO) have been widely reported in literature. We retrospectively evaluated the efficacy of different schedules of combined chelation therapy and the incidence of adverse events. METHODS:We evaluated 36 ...
journal_title:European journal of haematology
pub_type: 临床试验,杂志文章
doi:10.1111/j.1600-0609.2010.01447.x
更新日期:2010-07-01 00:00:00
abstract::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 ...
journal_title:European journal of haematology
pub_type: 杂志文章,评审
doi:10.1111/ejh.12543
更新日期:2015-09-01 00:00:00
abstract:OBJECTIVE:Interferon-alpha (IFNalpha) was the first effective pharmacologic treatment of hairy cell leukemia (HCL). Since 1990 purine analogs replaced IFNalpha because of higher rates of complete remission and an invariable disease recurrence after cessation of IFNalpha. However, there are only limited data about long-...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2008.01190.x
更新日期:2009-03-01 00:00:00
abstract::In the study of patients with Hodgkin's lymphoma (HL) the evaluation of bone marrow biopsy (BMB) can be difficult. In this review we analyze the main diagnostic features and the clinical risk factors of BM involvement. Although the role of BMB is criticized by some authors, its value is irreplaceable in the staging of...
journal_title:European journal of haematology
pub_type: 杂志文章,评审
doi:10.1111/j.1600-0609.2004.00292.x
更新日期:2004-09-01 00:00:00
abstract::Myeloperoxidase (MPO), a heme protein, is a major component of azurophilic granules of neutrophils. Optimal oxygen-dependent microbicidal activity depends on MPO as the critical enzyme for the generation of hypochlorous acid and other toxic oxygen products. MPO is synthesized during the promyelocytic stage of myeloid ...
journal_title:European journal of haematology
pub_type: 杂志文章,评审
doi:10.1111/j.1600-0609.1988.tb00805.x
更新日期:1988-02-01 00:00:00