Abstract:
:Platelet function disorders (PFD) and Von Willebrand disease (VWD) are among the uncommon causes of bleeding in haematological practice. The inherited variety of PFD includes defects in platelet adhesion, aggregation, secretion and platelet procoagulant activities. VWD is classified into three major categories-type 1 and 3 (quantitative deficiency) and type 2 VWD (qualitative defect). In the present study, the profile and prevalence of inherited PFD and VWD in Indians are described. Two thousand eight hundred patients with history of muco-cutaneous bleeding and other bleeding disorders were investigated. The tests performed included platelet count, bleeding time, PT, APTT, F VIII assay, platelet factor 3 (PF3) availability, platelet aggregation studies, VWF:Ag, VWF:RCo and multimeric analysis. Out of 2,800 patients investigated, a total of 872 (31.1%) were characterized to have either inherited coagulation defects (64.2%) or inherited platelet function disorders (35.8%). Of these 872 patients, 312 (35.8%) cases were characterized to have inherited PFD and 94 (16.8%) patients as VWD. Among 312 inherited PFD patients, isolated PF3 availability defect (48.1%) was most common, followed by unclassified PFD (37.2%). Among 94 VWD patients, type 2 VWD was most common (44.7%), followed by type 3 VWD (34.5%) and type 1 VWD (21.3%), respectively. Bleeding manifestations included easy bruising (46%), undue prolonged bleeding from trivial injuries (50% in PFD and type 1 and type 2 VWD and 100% in type 3 VWD), menorrhagia (31%), gum bleeds (22%), epistaxis (55%), haematuria (6%), GI bleeds (11%) and rarely, haematomas and haemarthoses (4%). In conclusion, VWD and inherited platelet function disorders are not uncommon among Indian population presenting with bleeding disorders.
journal_name
Ann Hematoljournal_title
Annals of hematologyauthors
Gupta PK,Charan VD,Saxena Rdoi
10.1007/s00277-006-0244-8subject
Has Abstractpub_date
2007-06-01 00:00:00pages
403-7issue
6eissn
0939-5555issn
1432-0584journal_volume
86pub_type
杂志文章abstract::Autoantibody against erythrocytes has occasionally been observed in patients with de novo acute myelocytic leukemia (AML). However, it is not clear whether this autoantibody in AML patients induces frank hemolysis (autoimmune hemolytic anemia, AIHA), as seen in lymphoid neoplasms. We present two de novo AML patients w...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/BF00663016
更新日期:1996-01-01 00:00:00
abstract::Extramedullary (e) relapse in multiple myeloma(MM) has an adverse prognosis, but knowledge concerning biological features and preferred treatment is scarce. We screened the myeloma registry of our institution for eMM relapses and identified 24 cases among 357 patients (pts).Only 8% of eMM relapses occurred after initi...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-012-1414-5
更新日期:2012-07-01 00:00:00
abstract::Pre-engraftment syndrome (PES) is a condition occurring after umbilical cord blood transplantation (UCBT) characterized by fever and erythematous skin rash prior to neutrophil engraftment. We sought to determine the incidence and characterize the pulmonary manifestations of PES. A retrospective review of patients who ...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-013-1981-0
更新日期:2014-05-01 00:00:00
abstract::Antithymocyte globulin (ATG) is the drug of choice for immunosuppressive therapy (IST) in patients with severe aplastic anemia (SAA) ineligible for allogeneic stem cell transplantation. Recently, rabbit ATG with cyclosporine A has been used as a first-line IST regimen in patients with SAA because of unavailability of ...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-013-1674-8
更新日期:2013-06-01 00:00:00
abstract::Hodgkin lymphoma (HL) in resource-poor population shows an early childhood peak with predominance of mixed cellularity (MCHL) and high Epstein-Barr virus (EBV) association, whereas HL in resource-rich population has a peak in ages 15-29 years with the predominance of nodular sclerosis (NSHL) and low EBV association. W...
journal_title:Annals of hematology
pub_type: 杂志文章,多中心研究,评审
doi:10.1007/s00277-013-1837-7
更新日期:2013-12-01 00:00:00
abstract::Several guidelines and recommendations on the management of chronic myeloid leukemia (CML) have been prepared by several scientific societies. The European LeukemiaNet (ELN) appointed a panel of experts who submitted their recommendations to peer-reviewed scientific journals in 2006, 2009, and 2013. Here, we make a cr...
journal_title:Annals of hematology
pub_type: 杂志文章,评审
doi:10.1007/s00277-015-2322-2
更新日期:2015-04-01 00:00:00
abstract::Recently, the combination of rituximab and bendamustine (R-Benda) has been defined as highly active in patients with follicular lymphomas, but little is known about the efficacy of R-Benda in mucosa-associated lymphoid tissue (MALT) lymphoma. In a retrospective analysis, we have defined 14 patients with MALT lymphoma ...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-013-1865-3
更新日期:2014-02-01 00:00:00
abstract::The aim of this review is to summarize the current knowledge on the clinical results of biotherapy of chronic myelogenous leukemia (CML) and potential mechanisms of the antitumor action of interferon alpha. IFN alpha treatment induces hematologic and cytogenetic remissions in patients with chronic phase CML. In additi...
journal_title:Annals of hematology
pub_type: 杂志文章,评审
doi:10.1007/BF01682030
更新日期:1995-03-01 00:00:00
abstract::A retrospective study of 120 patients with the clinically and histologically established diagnosis of idiopathic (primary) myelofibrosis (IMF) was performed to determine prognostic factors of predictive value, including parameters characterizing the dynamics of hematopoietic cell kinetics. In contrast to previous stud...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s002770050474
更新日期:1999-02-01 00:00:00
abstract::Less toxic and more active treatments are needed for indolent lymphomas as there is no curative treatment, and patients eventually die due to complications related to their disease. The purpose of the present study was to assess the antitumour activity of the combination of low doses of Enzastaurin and Lenalidomide (R...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-012-1490-6
更新日期:2012-10-01 00:00:00
abstract::Haploidentical hematopoietic stem cell transplantation (haplo-HSCT) has been accepted as a treatment option for aggressive (acute or lymphoma type) adult T cell leukemia/lymphoma (ATLL) patients with a poor prognosis, when a suitable HLA-matched donor is not available. However, haplo-HSCT carries a potential risk of t...
journal_title:Annals of hematology
pub_type: 临床试验,杂志文章
doi:10.1007/s00277-020-03934-6
更新日期:2020-03-01 00:00:00
abstract::Transplantation of autologous hematopoietic stem cells is a well established therapeutic procedure. Despite advances in efficacy of the stem cell mobilization and apheresis process until now a predictive factor for the expected stem cell yield before initiation of mobilization therapy could not be identified. The main...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-005-0074-0
更新日期:2006-06-01 00:00:00
abstract::The clinical targets for which new generations of antithrombotics have been or are currently under clinical development are those associated with a high risk for thromboembolism, (a) patients undergoing general, orthopedic, major abdominal, and cancer surgery, to prevent venous thromboembolism; (b) patients with deep-...
journal_title:Annals of hematology
pub_type: 杂志文章,评审
doi:10.1007/s002770050158
更新日期:1996-04-01 00:00:00
abstract::Isocitrate dehydrogenase IDH 1 and IDH 2 mutations were reported in several cancer forms, especially in hematological malignancies, but were never been investigated in familial aggregation. The aim of this study is to determine whether germline isocitrate dehydrogenase genes mutations are involved.We targeted IDH1 and...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-016-2813-9
更新日期:2016-12-01 00:00:00
abstract::The aim of the study was to investigate whether the well-known high anemia prevalence in pregnant women from the eastern Mediterranean and Asian regions decreased when the women immigrated to a low-frequency region (Denmark). During 70 months, 1,741 pregnant immigrant women referred from primary care to an obligatory ...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-007-0305-7
更新日期:2007-09-01 00:00:00
abstract::Autosomal recessive "malignant" osteopetrosis is a rare congenital disorder relating to bone resorption abnormalities. It is believed to arise due to the failure of osteoclasts to resorb immature bone. This leads to abnormal bone marrow cavity formation and, clinically, to the signs and symptoms of bone marrow failure...
journal_title:Annals of hematology
pub_type: 杂志文章,评审
doi:10.1007/s00277-002-0578-9
更新日期:2003-01-01 00:00:00
abstract::In the present study, we questioned whether the cholesterol synthesis inhibitor lovastatin potentiates the cytotoxicity of chemotherapeutic agents in the primitive CD34(+) subpopulation of acute myeloid leukemia (AML) cells. AML mononuclear cells (n = 17) were sorted in CD34(+) and CD34(-) fractions and compared to no...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-008-0633-2
更新日期:2009-06-01 00:00:00
abstract:PURPOSE:To determine whether, after very intensive induction and consolidation therapy in childhood AML, further maintenance therapy (MT) confers any advantage. PATIENTS AND METHODS:Three hundred-nine children with previously untreated AML were registered in the LAME 89/91 protocol. This three-cycle intensive regimen ...
journal_title:Annals of hematology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1007/s00277-004-0850-2
更新日期:2004-01-01 00:00:00
abstract::To gain insight into the natural history of cytomegalovirus (CMV) infection following unrelated cord blood transplantation (UCBT) in seropositive patients, we analyzed the data of 349 seropositive patients who received UCBT in Korea between 2000 and 2011. CMV reactivation occurred in 49 % (171/349) of the CMV-seroposi...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-014-2222-x
更新日期:2015-03-01 00:00:00
abstract::We investigated a new protein-C (PC) concentrate in a child with a type-II homozygous deficiency, concerning tolerance and safety. By means of various functional and antigen assays the in vivo recovery and the half-life were determined. In order to compare the results we reduced the measured values to the average half...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/BF01697402
更新日期:1992-03-01 00:00:00
abstract::Children with sickle cell disease (SCD) benefit from newborn screening, because life-threatening complications can be prevented by pre-symptomatic diagnosis. In Germany, the immigration of people from endemic countries is steadily growing. Comprehensive data about the epidemiology and prevalence of SCD in Germany are ...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-015-2573-y
更新日期:2016-02-01 00:00:00
abstract::Since December 2019, a novel coronavirus has spread throughout China and across the world, causing a continuous increase in confirmed cases within a short period of time. Some studies reported cases of thrombocytopenia, but hardly any studies mentioned how the virus causes thrombocytopenia. We propose several mechanis...
journal_title:Annals of hematology
pub_type: 杂志文章,评审
doi:10.1007/s00277-020-04019-0
更新日期:2020-06-01 00:00:00
abstract::We have studied some of the factors involved in the cytotoxic actions of the anticancer anthracycline antibiotics doxorubicin (DOX) and idarubicin (IDA) towards human B-cell lymphoma cells in vitro. IDA was found to accumulate within cells to a greater degree than the related drug DOX for both short (1 h) and long-ter...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/BF01757348
更新日期:1994-01-01 00:00:00
abstract::We have studied the effects of recombinant human thrombopoietin (TPO, mpl ligand) on the megakaryocyte colony formation from control human bone marrow cells, human leukemia cells at diagnosis, and human bone marrow cells after induction chemotherapy for acute leukemias. In the control human bone marrow cells from four...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s002770050408
更新日期:1998-07-01 00:00:00
abstract::Amifostine increases in vitro burst-forming unit-erythroid and colony-forming unit-granulocyte/granulcoyte-macrophage cultured from bone-marrow cells from patients with myelodysplastic syndrome (MDS). Several small clinical studies give divergent informations about the potential of amifostine as single agent to improv...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s002770050589
更新日期:2000-05-01 00:00:00
abstract::Azacitidine (AZA) is a DNA hypomethylation agent administered in myeloid neoplasms; however, there is still a lack of established predictors of response. We studied 113 patients with myelodysplastic syndromes (n = 85) or acute myeloid leukemia (n = 28) who received AZA to assess the predictive value on response of cli...
journal_title:Annals of hematology
pub_type: 临床试验,杂志文章
doi:10.1007/s00277-020-03932-8
更新日期:2020-03-01 00:00:00
abstract::Iron status, i.e. serum ferritin and haemoglobin (Hb) levels, was assessed in a population survey in 1994 (Dan-Monica 10) comprising 1319 Caucasian Danish women in age cohorts of 40, 50, 60 and 70 years. In the entire series, ferritin levels increased significantly from 40 years to 60 years of age. The prevalence of s...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s002770000209
更新日期:2000-11-01 00:00:00
abstract::The aim of the study was to investigate the association of leptin with hematological parameters in beta-thalassemia patients in Greece. We measured plasma levels of soluble transferrin receptor (sTfR) and leptin by enzyme-linked immunosorbent assay (ELISA) in 40 beta-thalassemia patients (21 transfusion dependent and ...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-002-0499-7
更新日期:2002-09-01 00:00:00
abstract::Cancer incidence in patients with recurrent unprovoked venous thromboembolism (VTE) is much higher than after a first event, but the incidence of myeloproliferative neoplasms (MPN) in this situation is still unknown. We tested for JAK2V617F and calreticulin mutants, 372 DNA samples of patients treated for (VTR). Among...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-016-2853-1
更新日期:2017-03-01 00:00:00
abstract::Since July 2017, different generic imatinib formulations have been introduced in Italy for the treatment of patients with chronic myeloid leukemia (CML). We analyzed 168 chronic phase CML patients treated with branded imatinib for a median of 12 years (range 1-16) at a single institution who switched to a single gener...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-020-04096-1
更新日期:2020-12-01 00:00:00