Abstract:
:The clinico-pathological features of 12 cases of malignant histiocytosis (histiocytic medullary reticulosis), aged 12-60 years, were studied. Special emphasis was put on the analysis of the haematological, cytological and histopathological findings of the disease. Bone marrow aspirates and biopsies from involved tissues (liver, lymph node, spleen and skin) were found best suited for establishing the diagnosis. Cytochemical and ultrastructural data support the concept that the neoplastic cells belong to the mononuclear-phagocyte system. Three untreated patients died in less than 6 months. Median survival of the nine treated patients was 1 year. Seven patients were treated with the combination chemotherapy CHOP and four achieved a complete remission. Three of them are alive and continue in unmaintained remission for more than 1 year, surviving so far for 18+ to 31+ months. Two cases showed evidence of CNS involvement, one during life and the other as a post-mortem finding. The differential diagnosis from other related or unrelated disorders and the possible aetiological factors responsible for the disease are discussed in detail.
journal_name
Br J Haematoljournal_title
British journal of haematologyauthors
Lampert IA,Catovsky D,Bergier Ndoi
10.1111/j.1365-2141.1978.tb03640.xsubject
Has Abstractpub_date
1978-09-01 00:00:00pages
65-77issue
1eissn
0007-1048issn
1365-2141journal_volume
40pub_type
杂志文章abstract::It has been suggested that in blast crisis (BC) of chronic myeloid leukaemia (CML) the clinical and laboratory features of patients with 'lymphoid' phenotype differ from those of patients with non-lymphoid BC. In order to assess any differences, 97 patients consecutively diagnosed with BC that followed a known chronic...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1046/j.1365-2141.1998.00542.x
更新日期:1998-01-01 00:00:00
abstract::Mutations of the p53 tumour suppressor gene occur in 20% of chronic myeloid leukaemia (CML) patients in blastic crisis, but it is still uncertain whether this inactivation plays a role in the pathogenesis of blastic transformation or in maintaining the leukaemic proliferation in CML, as it does in several solid tumour...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1365-2141.1995.tb03374.x
更新日期:1995-05-01 00:00:00
abstract::The iron chelator deferasirox (DFX) prevents complications related to transfusional iron overload in several haematological disorders characterized by marrow failure. It is also able to induce haematological responses in a percentage of treated patients, particularly in those affected by myelodysplastic syndromes. The...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1111/bjh.13381
更新日期:2015-07-01 00:00:00
abstract::Diamond-Blackfan anaemia (DBA) is a constitutional pure red cell aplasia presenting in early childhood. In some patients, neutropenia and/or thrombocytopenia have also been observed during the course of the disease. We have followed 28 patients with steroid-refractory DBA for up to 13 years with serial peripheral bloo...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1046/j.1365-2141.2000.01796.x
更新日期:2000-01-01 00:00:00
abstract::A reduction of donor effects during centrifugal plateletpheresis with the Haemonetics Blood Processor was achieved by reducing the concentration of the citrate anticoagulant. Serum citrate and ionized calcium levels, immediately and 1 h post-pheresis, were affected to a lesser extent by using 5.0 g total ionized citra...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1365-2141.1976.tb03585.x
更新日期:1976-11-01 00:00:00
abstract::Hydroxyurea (HU), an inhibitor of DNA synthesis, can also induce haemoglobinization in certain erythroid cell lines. In this study, we report that intracellular peroxides levels were increased in HU-treated murine erythroleukaemia (MEL) cells and that l-acetyl-N-cysteine (LNAC), a potent reducing reagent, had a signif...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1046/j.1365-2141.2003.04309.x
更新日期:2003-05-01 00:00:00
abstract::Novel therapies have transformed the treatment paradigm for multiple myeloma with significant improvements in survival now seen in both younger and older patients. Nonetheless, the disease is heterogeneous and high-risk patients in particular continue to have poor outcome. Moreover, the disease remains incurable. Effo...
journal_title:British journal of haematology
pub_type: 杂志文章,评审
doi:10.1111/j.1365-2141.2011.08791.x
更新日期:2011-09-01 00:00:00
abstract::A patient with apparent anaemia and thrombocytopenia caused by a monoclonal paraprotein is described. The patient's serum contained a monoclonal IgM kappa, a cryoglobulin and a cold agglutinin. The cryoglobulin, similar to the serum paraprotein, was a monoclonal IgM kappa. Serum was studied to determine the relationsh...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1046/j.1365-2141.1998.00586.x
更新日期:1998-03-01 00:00:00
abstract::Hypercalcaemia complicating acute myelogenous leukaemia is a rare but well-recognized phenomenon. In most cases the pathogenetic mechanism causing the hypercalcaemia remains poorly understood. We recently studied in detail two patients with acute myelogenous leukaemia who developed hypercalcaemia during the course of ...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1365-2141.1983.tb02075.x
更新日期:1983-05-01 00:00:00
abstract::Interleukin 17A (IL17A), a cytokine involved in allergy, inflammation and osteoclastogenesis, was investigated in multiple myeloma (MM) to assess its role in the osteoclast (OC)-like activity of marrow immature dendritic cells (iDCs). Comparing nine MM patients with control subjects affected by monoclonal gammopathy o...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1111/bjh.12333
更新日期:2013-06-01 00:00:00
abstract::We report on a case of pyridoxine refractory hereditary sideroblastic anaemia (HSA) in a 19-year-old man who underwent peripheral blood stem cell transplantation (PBSCT) from his HLA-identical brother. By using short tandem repeat polymorphism, 100% donor cells were observed in peripheral blood on day +21; bone marrow...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1046/j.1365-2141.2000.02050.x
更新日期:2000-06-01 00:00:00
abstract::Mantle cell lymphoma is a distinct clinicopathological entity associated with t(11;14) and cyclin D1 overexpression. The majority of cases show uniform morphological and phenotypic features characterized by a monotonous proliferation of small-to-medium-sized irregular B cells that express CD5 and bright surface immuno...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1046/j.1365-2141.1998.00907.x
更新日期:1998-09-01 00:00:00
abstract::Patients with intermittent claudication have been reported to have disturbances in blood rheology and haemostasis. Whether these disturbances are a result of, or largely independent of, smoking history and arterial narrowing has not yet been established. The levels of whole blood and plasma viscosity, haematocrit, von...
journal_title:British journal of haematology
pub_type: 临床试验,杂志文章
doi:10.1046/j.1365-2141.1996.00283.x
更新日期:1996-01-01 00:00:00
abstract::The Xba I polymorphic site in the factor VIII gene is present in the int22h-1 region which is found in two other copies (int22h-2 and int22h-3) distal to the gene. Previously the polymorphic status of the Xba I locus was studied by either Southern blot or PCR that amplified all three copies. Here we report the use of ...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1046/j.1365-2141.1999.01430.x
更新日期:1999-06-01 00:00:00
abstract::The variable clinical outcomes of Multiple Myeloma (MM) patients are incompletely defined by current prognostication tools. We examined the clinical utility of high-resolution telomere length analysis as a prognostic marker in MM. Cohort stratification, using a previously determined length threshold for telomere dysfu...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1111/bjh.14643
更新日期:2017-07-01 00:00:00
abstract::Relapse remains the leading cause of treatment failure in children with acute lymphoblastic leukaemia (ALL) undergoing allogeneic haematopoietic stem cell transplantation (HSCT). We retrospectively investigated the prognostic role of minimal residual disease (MRD) before and after HSCT in 119 children transplanted in ...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1111/bjh.15086
更新日期:2018-03-01 00:00:00
abstract::Mantle cell lymphoma (MCL) is a heterogenic non-Hodgkin lymphoma entity, with a median survival of about 5 years. In 2008 we reported the early - based on the median observation time of 4 years - results of the Nordic Lymphoma Group MCL2 study of frontline intensive induction immunochemotherapy and autologous stem cel...
journal_title:British journal of haematology
pub_type: 临床试验,杂志文章
doi:10.1111/j.1365-2141.2012.09174.x
更新日期:2012-08-01 00:00:00
abstract::The available data on rituximab in combination with chemotherapy confirm that the addition of an independently active biological agent to full-dose standard chemotherapy results in higher rates of complete response, lower rates of relapse, prolonged survival, little additional toxicity and no compromise of the dose in...
journal_title:British journal of haematology
pub_type: 指南,杂志文章,实务指引
doi:10.1046/j.1365-2141.2003.04274.x
更新日期:2003-04-01 00:00:00
abstract::The pattern of distribution of von Willebrand factor (VWF) in relatively large sheets of rat aortic endothelial cells (EC) obtained by the Häutchen technique were analysed by immunocytochemistry and light microscopy. EC were examined pre and post administration of a procoagulant mixture of factor Xa (F.Xa) and phospho...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1046/j.1365-2141.1996.4661005.x
更新日期:1996-04-01 00:00:00
abstract::Pentose phosphate shunt activity was studied by the release of 14CO2 from 14C-1-glucose and 14C-2-glucose in the red cells of five patients with pyruvate kinase deficiency and found to be significantly decreased after new methylene blue stimulation when compared to high reticulocyte controls. Incubated Heinz body form...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1365-2141.1983.tb02122.x
更新日期:1983-07-01 00:00:00
abstract::Graft rejection and graft-versus-host disease are major problems in mismatched marrow transplants along with toxicity from standard myeloablative host treatments. We have established a tolerization model, using 1 Gy irradiation, which reduces stem cell capacity to < 10% of control while causing minimal myelosuppressio...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1046/j.1365-2141.2002.03801.x
更新日期:2002-10-01 00:00:00
abstract::The existence of functional gap junctions between haematopoietic progenitor cells (HPCs) and stromal cells of the haematopoietic microenvironment in the human system is a controversial issue. Primary CD34+ HPCs isolated from leukapheresis products were co-incubated with the human fibroblastoid bone marrow stromal cell...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1046/j.1365-2141.2000.02385.x
更新日期:2000-11-01 00:00:00
abstract::SAn ageing population and the continuing expansion of clinical indications for coumadin therapy have increased pressure on hospital anticoagulant clinics. One solution is patient self-testing (PST) of the international normalized ratio (INR) using capillary blood samples on point-of-care coagulation monitors at home. ...
journal_title:British journal of haematology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1111/j.1365-2141.2004.05300.x
更新日期:2005-01-01 00:00:00
abstract::Reversible aggregation of red blood cells (RBC) plays an important role in determining blood flow properties, and it is this aggregation which increases blood viscosity at low shear rates. The structure and sites of venous thrombi, as well as the fact that stasis is a major predisposing factor in venous thrombosis, su...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1365-2141.1994.tb04993.x
更新日期:1994-09-01 00:00:00
abstract::Antibodies grouped together by the Third Workshop on Leucocyte Differentiation Antigens on the basis of pan-macrophage reactivity on tissue sections were analysed in immunoprecipitation experiments. Antibodies Y2/131, EBM11, Ki-M6 and Ki-M7 all precipitated antigens of Mr 110,000 which were shown to be identical by pr...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1365-2141.1989.tb00210.x
更新日期:1989-09-01 00:00:00
abstract::Over the last few years there has been rapid and radical change in the way clinical research in the UK is funded and supported within the NHS. This has resulted from restructuring and major new investment in research infrastructure, co-ordinated through Clinical Local Research Networks (CLRNs) and equivalent organisat...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1365-2141.2010.08384.x
更新日期:2010-09-01 00:00:00
abstract::Graft failure is a life-threatening complication after allogeneic haematopoietic stem cell transplantation (HSCT). We report a cohort of 19 consecutive patients (median age: 8·5 years) with acute leukaemias (n = 14) and non-malignant diseases (n = 5) who experienced graft failure after previous HSCT from matched (n = ...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1111/bjh.14190
更新日期:2016-10-01 00:00:00
abstract::This study was designed to determine the incidence of relapse and factors predictive for relapse in 719 patients with severe aplastic anaemia (SAA) after immunosuppressive treatment (IS). Patients developing myelodysplasia or acute leukaemia after IS, and patients receiving a transplant, were excluded from this analys...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1365-2141.1993.tb03181.x
更新日期:1993-10-01 00:00:00
abstract::The use of L-asparaginase (L-ASP) in paediatric patients with acute lymphoblastic leukaemia (ALL) is associated with thrombotic complications. We evaluated the activities of tissue factor (TFa), thrombomodulin (TMa) and procoagulant phospholipids (PPL) in 26 consecutive children with ALL (25 B-ALL and one T-ALL) treat...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1365-2141.2009.07958.x
更新日期:2010-02-01 00:00:00
abstract::Genetic risk factors play an important role in the aetiology of vascular diseases. The insertion/deletion polymorphism (4G/5G) in the promotor region of the plasminogen activator inhibitor 1 (PAI-1) gene has been associated with an increased risk of myocardial infarction. We investigated 136 patients with minor stroke...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1046/j.1365-2141.2000.02164.x
更新日期:2000-08-01 00:00:00