Abstract:
:To compare the features of sickle-cell anemia in Brazil with those in other locales, we studied the effects of the beta-globin-like gene cluster haplotype and alpha-thalassemia upon the clinical and hematological features in 85 patients. The distribution of haplotypes differed from that in the United States and Jamaica. The Central African Republic (CAR) haplotype predominated; 34% of patients were CAR haplotype homozygotes, 45% CAR/Benin homozygotes, and 11% Benin homozygotes. No Senegal haplotype chromosomes were observed. Alpha-thalassemia was present in 17.5% of patients. HbF levels were higher in Benin homozygotes, compared with the other two groups (P < 0.05). Nearly half the patients with a CAR haplotype had leg ulcers, compared to 12.5% of the Benin homozygote group; stroke did not occur in alpha-thalassemia carriers, but neither result was statistically significant. As in other studies, our results indicate that the CAR haplotype may be associated with more severe disease.
journal_name
Am J Hematoljournal_title
American journal of hematologyauthors
Figueiredo MS,Kerbauy J,Gonçalves MS,Arruda VR,Saad ST,Sonati MF,Stoming T,Costa FFdoi
10.1002/(SICI)1096-8652(199610)53:2<72::AID-AJH3>3subject
Has Abstractpub_date
1996-10-01 00:00:00pages
72-6issue
2eissn
0361-8609issn
1096-8652pii
10.1002/(SICI)1096-8652(199610)53:2<72::AID-AJH3>3journal_volume
53pub_type
杂志文章abstract::Fibrillar granules (FGs) represent neutrophilic primary granules containing clustered filaments. We investigated neutrophils in the bone marrow obtained from 17 patients with chronic myeloproliferative disorders (CMPD) by electron microscopy. FG-positive neutrophils were seen in 15 of the 17 CMPDs with varying frequen...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830470316
更新日期:1994-11-01 00:00:00
abstract::A 37-year-old untransfused, non-drinking man with Hemoglobin H-CS disease presented with insulin-dependent diabetes mellitus, markedly elevated serum ferritin level, and marked iron deposition in hepatocytes. He did not carry either of the two common mutations of the HLA-H gene for hereditary hemochromatosis, namely, ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(sici)1096-8652(199802)57:2<160::aid-ajh12
更新日期:1998-02-01 00:00:00
abstract::The relationship between thrombocytopenia and the level of anticardiolipin antibodies (aCL) and/or the existence of lupus anticoagulant (LA) ware studied in 146 patients with systemic lupus erythematosus (SLE). These patients were divided into six groups: A, those LA positive with a high level of aCL (>10 U/ml) (10 ca...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(sici)1096-8652(199805)58:1<55::aid-ajh10>
更新日期:1998-05-01 00:00:00
abstract:BACKGROUND:The histopathologic features characterizing the involvement of the bone marrow (BM) in systemic lupus erythematosus (SLE) have not been systematically analyzed to date. OBJECTIVES:The aim of this study was to assess morphologic and immunohistochemical characteristics of BM involvement in SLE. PATIENTS AND ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20593
更新日期:2006-08-01 00:00:00
abstract::The objective of this study is to establish a method to identify patients with primary immune thrombocytopenia (ITP) utilizing administrative data from diverse data sources that would be appropriate for epidemiologic studies of ITP, regardless of patients' age and source of health care. Medical records of the Oklahoma...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23226
更新日期:2012-09-01 00:00:00
abstract::BCR-ABL1-negative myeloproliferative neoplasms (MPNs) are clonal stem cell disorders defined by proliferation of one or more myeloid lineages, and carry an increased risk of vascular events and progression to myelofibrosis and leukemia. Portal hypertension (pHTN) occurs in 7-18% of MPN patients via both thrombotic and...
journal_title:American journal of hematology
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1002/ajh.24798
更新日期:2017-09-01 00:00:00
abstract::An adolescent male patient developed functional asplenia in the course of graft versus host disease (GVHD) after successful allogeneic bone marrow transplantation (BMT) for aplastic anemia. Coincident with the onset of the asplenia, amelioration of the ongoing GVHD was observed. Unexpectedly, after 34 months of functi...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830120112
更新日期:1982-02-01 00:00:00
abstract::Cephalosporins are commonly used drugs that interact with red blood cell membranes. They frequently cause a positive direct antiglobulin test but rarely cause hemolysis. A case of cefotetan-induced hemolytic anemia is described in which two types of antibodies were detected. One reacted with red blood cells by a hapte...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.2830400209
更新日期:1992-06-01 00:00:00
abstract::During 1971-1988, 194 adults with de novo acute myeloid leukemia (AML) received initial therapy at the University of Minnesota with an anthracycline-based regimen. Seventy-two of the 194 required further chemotherapy and received a second cycle of the same or similar therapy; 63 of these 72 were evaluable. For each ma...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830470305
更新日期:1994-11-01 00:00:00
abstract::Quantitative changes of hemostatic molecular markers were studied in patients with nephrotic syndrome. The plasma levels of fibrinopeptide A (FPA), thrombin-antithrombin III complex (TAT), products of thrombin activation, and fragment F1 + 2 (F1 + 2), a product of prothrombin activation, were measured by enzyme immuno...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830440411
更新日期:1993-12-01 00:00:00
abstract::Plasma cell dyscrasias are a group of clinically and biochemically diverse disorders of unknown etiology, characterized by the disproportionate proliferation of one or more clones of B cells, and the presence of a structurally and electrophoretically homogeneous (monoclonal) immunoglobulin or polypeptide subunit in se...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.20736
更新日期:2006-12-01 00:00:00
abstract::Follicular lymphoma (FL) frequently transforms into the more aggressive diffuse large B cell lymphoma (DLBCL-tr), but no protein biomarkers have been identified for predictive or early diagnosis. Gene expression analyses have identified genes changing on transformation but have failed to be reproducible in different s...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.21549
更新日期:2009-12-01 00:00:00
abstract::The FAB classification of myelodysplastic syndromes (MDS) has been useful in predicting prognosis; however, additional methods are required to detect patients at high risk for early conversion to acute nonlymphoblastic leukemia (ANLL). Using a panel of monoclonal antibodies to myelomonocytic surface antigens (MMSA) an...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.2830430302
更新日期:1993-07-01 00:00:00
abstract::A growing body of evidence suggests that iron overload is associated with inferior outcomes after myeloablative allogeneic hematopoietic stem cell transplantation (HSCT). However, all of those studies used surrogate markers of iron overload, especially serum ferritin, and most had a retrospective design. We conducted ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23188
更新日期:2012-06-01 00:00:00
abstract:DISEASE OVERVIEW:Polycythemia vera (PV) and essential thrombocythemia (ET) are myeloproliferative neoplasms primarily characterized by erythrocytosis and thrombocytosis, respectively. Other disease features include leukocytosis, splenomegaly, thrombohemorrhagic complications, vasomotor disturbances, pruritus, and a sma...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.21946
更新日期:2011-03-01 00:00:00
abstract::A case of red cell adenosine deaminase (ADA) overproduction associated with hereditary hemolytic anemia is reported here. This appears to be the second report. Proband is a 38-year-old Japanese male who had hemoglobin, 15.8 g/100 ml; reticulocyte count, 4.5%; serum indirect bilirubin, 4.9 mg/100 ml; 51Cr-labeled red c...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830050205
更新日期:1978-01-01 00:00:00
abstract::Lenalidomide and azacitidine are active in MDS patients, and may complement each other by targeting the bone marrow microenvironment and the malignant clone. A recent Phase I trial testing the lenalidomide and azacitidine combination yielded encouraging results; however, lenalidomide’s contribution was unclear. In thi...
journal_title:American journal of hematology
pub_type: 杂志文章,多中心研究
doi:10.1002/ajh.21891
更新日期:2011-01-01 00:00:00
abstract::We report the case of an immunocompetent woman who developed disseminated intravascular coagulation (DIC) due to acute cytomegalovirus (CMV) infection and was successfully treated with plasma exchange. Extensive investigations revealed no other cause for her DIC, and she did not have any recurrence after more than 1 y...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20602
更新日期:2006-06-01 00:00:00
abstract::Several histone deacetylase inhibitors (HDACi), including vorinostat, have been approved for the therapy of cutaneous T-cell lymphoma (CTCL). Emerging data suggest that HDACi may exert immune suppressive effects which would be disadvantageous for therapy of CTCL. We describe a patient with Sezary syndrome who was moni...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.22231
更新日期:2012-02-01 00:00:00
abstract:DISEASE OVERVIEW:Cutaneous T-cell lymphomas are a heterogenous group of T-cell lymphoproliferative disorders involving the skin, the majority of which may be classified as Mycosis Fungoides (MF) or Sézary Syndrome (SS). DIAGNOSIS:The diagnosis of MF or SS requires the integration of clinical and histopathologic data. ...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.23756
更新日期:2014-08-01 00:00:00
abstract::Newly identified iron binding proteins isolated from rat duodenal homogenates permit better understanding of iron absorption. Mucins bind iron at acid pH to keep iron soluble and available for absorption at the more alkaline pH of the duodenum; this explains iron deficiency following prolonged achlorhydria. Integrin (...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.2830420114
更新日期:1993-01-01 00:00:00
abstract::The occurrence of factor VIII inhibitors in non-hemophilic patients is a rare event with a potentially lethal outcome. Despite its infrequent occurrence, the association of this inhibitor with multiple autoimmune diseases is well recognized. We report the case of a patient with the recently described autoimmune lympho...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/1096-8652(200007)64:3<214::aid-ajh14>3.0.c
更新日期:2000-07-01 00:00:00
abstract::Indolent non-hodgkin lymphomas (iNHL) are a rare cause of monoclonal immunoglobulin deposits-related glomerulopathy (mIgGN). In patients with iNHL-related mIgGN, whether treatment should include either single or a combination of drug(s) to target the malignant clone and renal inflammation remains elusive. In this retr...
journal_title:American journal of hematology
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1002/ajh.23798
更新日期:2014-10-01 00:00:00
abstract::Polycythemia vera (PV) is a myeloproliferative disorder characterized by thrombotic and, less often, bleeding complications. Many mechanisms have been advanced to explain the occurrence of these complications, none of them satisfactory. We examined a cohort of 27 patients with PV, secondary erythrocytosis, and essenti...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(sici)1096-8652(199903)60:3<196::aid-ajh5>
更新日期:1999-03-01 00:00:00
abstract::Oral chronic graft versus host disease (cGVHD) is common and a major cause of morbidity and loss of quality of life in long term survivors. Cyclosporine with prednisone remains the first line therapy for oral manifestations of cGVHD. However, even with routine administration of systemic agents, many patients with oral...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20814
更新日期:2007-05-01 00:00:00
abstract::Lysosomes (granules) of rabbit PMN leukocytes were extracted with either HCl or H2SO4, and the extracts were chromatographed over Sephadex to separate protein constituents. Some of the low molecular weight cationic proteins homogeneous on SDS PAGE (8% and 12.5% gels) were characterized by electrophoretic mobility in a...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830070309
更新日期:1979-01-01 00:00:00
abstract::Cytotoxic agents like Hydroxyurea, Busulfan and Interferon-alpha are to date the most commonly used therapeutic approaches in Essential Thrombocythemia (ET). However, few data on the efficacy and safety of these agents in the long-term are currently available. We report a retrospective analysis of the long-term outcom...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.21360
更新日期:2009-04-01 00:00:00
abstract::Idiopathic thrombocytopenic purpura is an autoimmune disease which involves opsonization of platelets by autoantibodies directed against different surface glycoproteins, leading to their premature destruction by the reticuloendothelial system. Management of patients with refractory ITP is difficult. Recent studies hav...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20276
更新日期:2005-04-01 00:00:00
abstract::Coagulation factor VIII and von Willebrand factor (VWF) are key proteins in procoagulant activation. Higher FVIII coagulant activity (FVIII :C) and VWF antigen (VWF :Ag) are risk factors for cardiovascular disease and venous thromboembolism. Beyond associations with ABO blood group, genetic determinants of FVIII and V...
journal_title:American journal of hematology
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1002/ajh.24005
更新日期:2015-06-01 00:00:00
abstract::We evaluated possible prognostic factors just before salvage therapy with vincristine, doxorubicin, and dexamethasone (VAD) for 36 patients with refractory multiple myeloma. The median duration from diagnosis to the first VAD salvage was 14 months (range 2-76 months). Among parameters that have been shown to be associ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/1096-8652(200010)65:2<132::aid-ajh7>3.0.co
更新日期:2000-10-01 00:00:00