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, Cys282Tyr and His68Asp, nor did he have the associated HLA marker (HLA-A3, B7 nor B-14) for the disease. Patient with HbH disease should be monitored for iron overload.
journal_name
Am J Hematoljournal_title
American journal of hematologyauthors
Chim CS,Chan V,Todd Ddoi
10.1002/(sici)1096-8652(199802)57:2<160::aid-ajh12subject
Has Abstractpub_date
1998-02-01 00:00:00pages
160-3issue
2eissn
0361-8609issn
1096-8652pii
10.1002/(SICI)1096-8652(199802)57:2<160::AID-AJH12journal_volume
57pub_type
杂志文章abstract::Epstein-Barr virus (EBV) occasionally infects T and NK cells and causes EBV-infected T/NK-cell lymphoproliferative disease (LPD), which comprises chronic active EBV infection, EBV-associated hemophagocytic syndrome, mosquito allergy, hydroa vacciniforme, aggressive NK-cell leukemia, and NK/T-cell lymphoma. The diagnos...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20659
更新日期:2006-08-01 00:00:00
abstract::We performed a laparoscopic splenectomy (LS) in 60 patients (age 9-83, 45 females) with idiopathic thrombocytopenic purpura (ITP) who did not achieve sustained remission on steroid therapy. Using a modified procedure, the mean duration of LS was 78 min (range 25-240 min) and surgery was associated with only 5% major a...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(sici)1096-8652(200001)63:1<7::aid-ajh2>3.
更新日期:2000-01-01 00:00:00
abstract::The pharmacokinetics of factor VIII were studied in a series of 20 hemophilia-A patients undergoing surgery. Regardless of the type of operation, elimination of factor VIII was shown to be increased only in ten cases (50%) during the post-operative period. In this subgroup of patients, factor VIII half-life, measured ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830300305
更新日期:1989-03-01 00:00:00
abstract::Intensive induction chemotherapy using anthracycline and cytarabine backbone is considered the most effective upfront therapy in physically fit older patients with acute myeloid leukemia (AML). However, outcomes of the standard induction in elderly AML are inferior to those observed in younger patients, and they are s...
journal_title:American journal of hematology
pub_type: 杂志文章,随机对照试验
doi:10.1002/ajh.24654
更新日期:2017-04-01 00:00:00
abstract::Immune dysregulation and altered T-cell hemostasis play important roles in the pathogenesis of myelodysplastic syndromes (MDS). Recent studies suggest an increased risk of MDS among patients with autoimmune diseases. Here, we investigated the prevalence of autoimmune diseases among MDS patients, comparing characterist...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.24333
更新日期:2016-05-01 00:00:00
abstract::A case of idiopathic myelofibrosis (IMF) presenting with hypercalcemia and hypercalcitriolemia is reported. It is proposed that ectopic production of the active vitamin D metabolite related to ongoing clonal expansion in the bone marrow accounts for the hypercalcemic state. Consistently low levels of circulating type ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830390316
更新日期:1992-03-01 00:00:00
abstract::Long-term survival following chemotherapy or autologous bone marrow transplantation in adults with relapsed/refractory non-Hodgkin's lymphoma was evaluated. English language articles published from January 1, 1988 to September 1, 1993 were obtained from a broad-based MEDLINE search retrieving 3,854 citations regarding...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.2830500208
更新日期:1995-10-01 00:00:00
abstract::Forty-two patients with chronic lymphocytic leukemia (CLL) were studied for morphology of lymphocytes by light and electron microscopy (EM), in vitro responses of lymphocytes to a battery of physical and chemical agents, overall clinical status, immunologic status, course, and response to therapy. CLL lymphocytes coul...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830020110
更新日期:1977-01-01 00:00:00
abstract::The objective of the study was to explore the risks and benefits of splenectomy in advanced agnogenic myeloid metaplasia (AMM). We searched the literature (Medline, 1970-1987) for studies of postoperative survival, operative mortality and effects of splenectomy on painful splenomegaly, and portal hypertension or trans...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.2830330210
更新日期:1990-02-01 00:00:00
abstract::Clinical management of lymphoplasmacytic lymphoma (LPL)/Waldenström macroglobulinemia (WM) has changed considerably over recent years, reflected in the use of new therapeutic agents (purine analogs, monoclonal antibodies, thalidomide- and bortezomib-based therapies). No population-based studies and few randomized tria...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23351
更新日期:2013-01-01 00:00:00
abstract::Polycythemia vera (PCV) and multiple myeloma are both clonal disorders of hematopoietic stem cells. The simultaneous occurrence of these diseases in an individual patient is rare. A case of synchronous PCV and smoldering myeloma is presented and the literature is reviewed. The issues of clinical importance in this unu...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.2830440311
更新日期:1993-11-01 00:00:00
abstract::The increased adhesiveness of sickle erythrocytes (SS RBC) to endothelial cells has been confirmed in a static system utilizing fresh umbilical vein endothelium. Adherence of SS RBC to the endothelium was as great in the presence of calcium-containing buffer as when incubated in plasma. SS RBC suspended in autologous ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830340110
更新日期:1990-05-01 00:00:00
abstract::By using Dexter-type long-term marrow cultures (D-LTMC), it has been shown previously that hematopoietic progenitor cells (HPC) from patients with aplastic anemia (AA) have a deficient proliferation in vitro. The studies reported to date, however, have focused exclusively on granulomonocytic progenitors and no informa...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(sici)1096-8652(199810)59:2<149::aid-ajh8>
更新日期:1998-10-01 00:00:00
abstract::Little is known about the frequency and significance of clinically unapparent or occult hemorrhage in ITP. Therefore, we prospectively explored the sites and frequency of occult bleeding in children with severe ITP at diagnosis or upon symptomatic relapse in a prospective, single-institution cohort study of patients ≤...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.24264
更新日期:2016-03-01 00:00:00
abstract::Immunoglobulin light-chain amyloidosis (AL) is a rare disorder characterized by production of a monoclonal light chain. This insoluble light chain, or a fragment thereof, deposits in tissues as amyloid and results in disruption of organ function and, ultimately, in death. Although melphalan and prednisone are benefici...
journal_title:American journal of hematology
pub_type: 临床试验,杂志文章
doi:10.1002/(sici)1096-8652(199906)61:2<115::aid-ajh7>
更新日期:1999-06-01 00:00:00
abstract::Hemoglobin Lyon-Bron was found in two members of a family of German ascent presenting with a moderate normocytic anemia. In this alpha 2 globin variant, the N-terminal valine of the chain was replaced by an alanine. Electrospray mass spectrometry of the alpha chain showed that, as normally, the initiator methionine wa...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.10051
更新日期:2002-03-01 00:00:00
abstract::FIP1L1-PDGFRA-positive myeloid neoplasm with eosinophilia (F/P+ MN-eo) is a rare disease: robust epidemiological data are lacking and reported issues are scarce, of low sample-size and limited follow-up. Imatinib mesylate (IM) is highly efficient but no predictive factor of relapse after discontinuation has yet been i...
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.25945
更新日期:2020-11-01 00:00:00
abstract::Recombinant DNA technology and protein engineering are creating hope that we can address ongoing challenges in hemophilia care such as reducing the costs of therapy, increasing the availability to the developing world, and improving the functional properties of these proteins. Technological advances to improve the hal...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.23146
更新日期:2012-05-01 00:00:00
abstract::Anthracyclines have contributed to a marked increase in survival in different types of cancer [1,2]. Unfortunately, they are associated with dose-dependent cardiotoxicity and heart failure (HF) [3–8]. Change to a weekly dosage schedule with slow infusions has been tested, a strategy that requires more frequent hospita...
journal_title:American journal of hematology
pub_type: 杂志文章,随机对照试验
doi:10.1002/ajh.21840
更新日期:2010-11-01 00:00:00
abstract::High-density lipoprotein (HDL) of 100-400 micrograms/ml did not prevent morphological alterations of human blood platelets treated with serotonin (1-5 microM). Highly concentrated HDL (1,200 micrograms/ml) appeared to activate platelets in vitro. These findings indicate that whole HDL may not inhibit agonist-induced p...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830500417
更新日期:1995-12-01 00:00:00
abstract::Infantile malignant osteopetrosis (IMO) includes various genetic disorders that affect osteoclast development and/or function. Genotype-phenotype correlation studies in IMO have been hampered by the rarity and heterogeneity of the disease and by the severity of the clinical course, which often leads to death early in ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.21447
更新日期:2009-08-01 00:00:00
abstract::Defects in stomatin, alpha-adducin, and beta-adducin have been implicated in erythrocyte disorders of cation permeability. We performed linkage analysis of the genetic loci for these proteins in a large kindred with xerocytosis (dehydrated hereditary stomatocytosis). Using polymerase chain reaction-based genotyping te...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(sici)1096-8652(199901)60:1<72::aid-ajh13>
更新日期:1999-01-01 00:00:00
abstract::An association between thrombocytopenia and thyrotoxicosis in a single individual is well documented, and the theories for this event include a common immunologic cause or a thyrotoxic-induced decrease in platelet survival. We report the first description of the coexistence of autoimmune thrombocytopenic purpura (AITP...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830390411
更新日期:1992-04-01 00:00:00
abstract::In the current study, we investigated C/EBPA gene mutations and promoter hypermethylation in a series of 53 patients with CN-AML. In addition, we also analyzed two other frequent mutations (FLT3/ITD and NPM1) in these patients and correlated them with C/EBPA gene alterations. 13/53 patients were FLT3/ITD+/NPM1-, 11/53...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.21706
更新日期:2010-06-01 00:00:00
abstract::Mutation of the VHL tumor suppressor gene is a frequent genetic event in the carcinogenesis of renal-cell carcinoma (RCC). Circulating endothelial progenitor cells (EPCs) have important role in neoangiogenesis, and mobilization of these cells is induced by various growth factors including erythropoietin (EPO). With th...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.21019
更新日期:2008-02-01 00:00:00
abstract::Little is known about the mechanisms explaining the wide variation in platelet counts (PLT) and other hematologic parameters in humans. We previously showed that the sex-based difference in hematocrit was associated with nucleotide variation in the erythropoietin receptor gene (EPOR). We sought to identify new polymor...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20095
更新日期:2004-09-01 00:00:00
abstract::CD7 antigen, a T-cell lineage associated antigen, is expressed in a minority of patients with acute myeloid leukemia (AML). The biologic and clinical significance of this finding is not clearly established. In this retrospective study of patients with de novo acute myeloid leukemia, we have identified CD7 expression a...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(sici)1096-8652(199808)58:4<278::aid-ajh5>
更新日期:1998-08-01 00:00:00
abstract::Fifteen recently diagnosed patients with acute leukemias admitted for induction chemotherapy were selected for study. When thrombocytopenic (venous platelet count less than 20 X 10(9)/l) these patients received prophylactic platelet transfusions. A total of 67 platelet transfusion therapies were administered and evalu...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830110305
更新日期:1981-11-01 00:00:00
abstract::Several parameters may predict disease severity and overall survival in chronic lymphocytic leukemia (CLL). The purpose of our study of 190 CLL patients was to compare immunoglobulin heavy chain variable region (IgV(H)) mutation status, cytogenetic abnormalities, and leukemia cell CD38 and Zap-70 to older, traditional...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20987
更新日期:2007-12-01 00:00:00
abstract::The main treatment for many patients with Myelodysplastic Syndromes (MDS) remains red cell transfusion to attenuate the symptoms of chronic anemia. Fatigue can reduce a patient's health related quality of life (HRQoL), but there is little understanding of the optimal use of transfusions to improve this. A systematic r...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.21503
更新日期:2009-10-01 00:00:00