Abstract:
:Sickle cell (SS) disease is a complex of various genetic conditions. In some, homozygosity for the beta S gene may be present alone or in combination with the heterozygous or homozygous alpha-thalassemia-2 condition. Such combinations might ameliorate the clinical and hematological condition of the patient. The same may be true for the high levels of Hb F and F-cells observed in many Hb S homozygotes. Howeever, the chemical heterogeneity of Hb F appears not to be related to the clinical status of the Hb S homozygote. Combinations of a Hb S heterozygosity with a heterozygosity for a Hb D-type of variant, for either one of two types of beta-thalassemia, two types of alpha beta- thalassemia, and five types of HPFH are discussed, and data are compared with those obtained for Hb S homozygotes. The use of advanced laboratory procedures and family studies is often necessary for an accurate diagnosis.
journal_name
Am J Hematoljournal_title
American journal of hematologyauthors
Huisman THdoi
10.1002/ajh.2830060210subject
Has Abstractpub_date
1979-01-01 00:00:00pages
173-84issue
2eissn
0361-8609issn
1096-8652journal_volume
6pub_type
杂志文章,评审abstract::"PVP storage disease" is a disorder occurring in patients who have received high molecular weight polyvinylpyrrolidone (PVP), which cannot be excreted from the body. These large polymers deposit in the histiocytes and cause proliferation and infiltration of histiocytes in the reticuloendothelial system. There was usua...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(sici)1096-8652(199801)57:1<68::aid-ajh12>
更新日期:1998-01-01 00:00:00
abstract::The clinical development of lenalidomide (Revlimid™), then pomalidomide (Actimid™) as members of immunomodulatory drugs (IMiDs) for the treatment of multiple myeloma (MM), exemplifies how insight into disease biology can lead to design of effective therapeutic agents. Increased experience and understanding of IMiD's d...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.23234
更新日期:2012-12-01 00:00:00
abstract::Because of the increase in the use of warfarin in the population in recent years, reversal of warfarin-related coagulopathy has become common in daily hospital practice. Transfusion of fresh frozen plasma (FFP) is the preferred treatment method for urgent warfarin reversal in the USA. We have undertaken a 1-month audi...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20902
更新日期:2007-12-01 00:00:00
abstract::Splenic lymphoma with villous lymphocytes (SLVL) is a relatively new entity with only a few reports published. We report seven cases of SLVL with detailed clinicopathologic and comprehensive immunophenotypic studies to further characterize this lymphoma, which is frequently confused with hairy cell leukemia and other ...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.2830450107
更新日期:1994-01-01 00:00:00
abstract::Chronic myeloid leukemia (CML) is considered to be a pleuripotential stem cell disorder with the capacity to differentiate into myeloid, erythroid, megakaryocytic, and lymphoid cell lines. Consequently, blast crisis (BC) involving each of the above lineages has been well described. Among lymphoblastic crises, differen...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830360204
更新日期:1991-02-01 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::The effect of heparin on platelet aggregation was systematically examined on platelets in plasma (PRP), as well as on gel-filtered, washed, and formaldehyde-fixed platelets. Results indicate that, although heparin causes a mild potentiation of platelet aggregation in the PRP systems, a significant inhibitory activity ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830170310
更新日期:1984-10-01 00:00:00
abstract::Autologous stem cell transplantation (ASCT) is feasible and effective in selected older patients with Multiple Myeloma, but specific criteria for evaluating ASCT eligibility in elderly patients are lacking. We evaluated 131 patients aged 65-75 considered for ASCT at our center: The Charlson Comorbidity Index (CCI), He...
journal_title:American journal of hematology
pub_type: 临床试验,杂志文章
doi:10.1002/ajh.25797
更新日期:2020-07-01 00:00:00
abstract::Leukemic cells of 43 patients with acute promyelocytic leukemia (M3) were investigated morphologically and cytochemically to determine the percentage of aberrant enzymes and whether or not the presence impacts on the clinical outcome. Twelve patients (27.9%) showed alpha-naphthyl acetate esterase (ANAE) activity in th...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830290306
更新日期:1988-11-01 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::Cyclooxygenases (COX) are key enzymes in the conversion of arachidonic acid to prostaglandins. Several studies have shown a relation between angiogenesis and COX-2 expression. Elevated expression of cyclooxygenase-2 (COX-2), however, has not been reported in multiple myeloma (MM) in the literature. The aim of this stu...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20460
更新日期:2005-11-01 00:00:00
abstract::The authors report a 10-year-old boy with hepatosplenic gammadelta T-cell lymphoma, a rare form of lymphoma that is highly aggressive, exceedingly rare in children, and primarily seen in young men. Conventional multi-agent chemotherapy appears to be inadequate for cure. This is the first report with this type of lymph...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.10466
更新日期:2004-02-01 00:00:00
abstract::Several studies provided evidence of a consistent antileukemic effect induced by cytomegalovirus (CMV) replication in acute myeloid leukemia (AML) patients receiving allogeneic hematopoietic stem cell transplantation (HSCT), however the use of antithymocyte globulin (ATG) as graft-versus-host disease prophylaxis, may ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23998
更新日期:2015-06-01 00:00:00
abstract::Binding of chemoattractant to polymorphonuclear leukocytes (PMNL) triggers a series of events like polymerization of actin and tubulin, orientation of cells, chemotaxis, increase in fluid pinocytosis and phagocytosis, and stimulation of microbicidal pathways which includes lysosomal degranulation and generation of rea...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(SICI)1096-8652(199607)52:3<155::AID-AJH4>
更新日期:1996-07-01 00:00:00
abstract::In a phase II trial, we evaluated chlorambucil and rituximab (CLB-R) as first-line induction treatment with or without R as maintenance for elderly chronic lymphocytic leukemia (CLL) patients. Treatment consisted of eight 28-day cycles of CLB (8 mg/m(2) /day, days 1-7) and R (day 1 of cycle 3, 375 mg/m(2) ; cycles 4-8...
journal_title:American journal of hematology
pub_type: 杂志文章,随机对照试验
doi:10.1002/ajh.23668
更新日期:2014-05-01 00:00:00
abstract::Although large vessel thrombi are occasionally reported in patients with homozygous sickle cell disease, the role of intravascular coagulation in typical pain crises is controversial. Therefore, we studied 24 sickle cell patients during and between episodes of pain crisis, using several sensitive tests of hemostasis. ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830230403
更新日期:1986-12-01 00:00:00
abstract::Clopidogrel is a widely used antiplatelet agent that irreversibly inhibits platelet P2Y12 ADP receptors after conversion to an active metabolite. There are a number of laboratory tests capable of detecting clopidogrel-induced platelet inhibition and published literature correlates suboptimal clopidogrel response to ad...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.22112
更新日期:2011-12-01 00:00:00
abstract::Some properties of a monoclonal antibody generated against the fibrinogen component of a factor VIII preparation were investigated. The antibody bound with equal affinity in solid phase radioimmunoassays to fibrinogens isolated from both normal patients and patients with von Willebrand disease. It reacted in a sensiti...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830180202
更新日期:1985-02-01 00:00:00
abstract::Sixty-two previously untreated patients with B-cell chronic lymphocytic leukaemia were analysed to study the prognostic value of both the immunologic phenotype and the clinicobiologic characteristics. Univariate studies showed that none of the immunological markers analysed, sheep-rosette, mouse-rosette, slg, and HLA/...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830310105
更新日期:1989-05-01 00:00:00
abstract::In this study we describe the time-dependent effects of a high dose (750 micrograms/ml/24 hr) continuous infusion of recombinant human granulocyte-macrophage colony-stimulating factor (rhGM-CSF) on monocyte number, cytokine release, and superoxide anion production. Blood was taken from patients prior to rhGM-CSF infus...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830430410
更新日期:1993-08-01 00:00:00
abstract::Over a 36-month period, 46 consecutive Mexican mestizos with a clinical marker associated with a primary hypercoagulable state were prospectively assessed by searching for the sticky platelet syndrome (SPS), the activated protein C resistance (aPCR) phenotype, coagulation protein C activity and antigen, coagulation pr...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20233
更新日期:2005-01-01 00:00:00
abstract::We present a female patient with T-gamma LGL leukemia, who was followed for the last 20 years. Over these years she developed several autoimmune disorders, including Sjögren's syndrome, Hashimoto's thyroiditis, premature ovarian failure (compatible with type II autoimmune polyglandular syndrome), amegakaryocytic throm...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.10024
更新日期:2002-02-01 00:00:00
abstract::Granulysin is a newly identified CTL/NK cell-related cytotoxic protein, which is secreted in both constitutive and Ca-dependent manner. To evaluate its significance in stem-cell transplantation (SCT), serum granulysin was measured by newly established ELISA method in 26 patients undergoing SCT (21 allogeneic and 5 aut...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20570
更新日期:2006-05-01 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::This case report describes a patient with hepatitis C virus infection responding to pegylated INF/ribaviron therapy, who developed immune thrombocytopenia. The severe thrombocytopenia failed to resolve with cessation of the peg-IFN/ribaviron. Because of rising hepatitis C virus RNA levels and evidence of rising serum ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20270
更新日期:2005-02-01 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::A patient with acute nonlymphoblastic leukemia (FAB M4), showing a near tetraploid chromosome complement on his marrow cells, was treated with low dose cytosine arabinoside and achieved remission. During remission the near tetraploid marrow chromosome complement disappeared and reappeared upon relapse of leukemia. The...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830190412
更新日期:1985-08-01 00:00:00
abstract::We describe a 23-year-old retired military officer who was immunocompetent but diagnosed with hemophagocytic syndrome (HPS) by Plasmodium vivax infection. Initially, the patient was suspected to have toxic hepatitis related to heavy drinking. But abnormal hematologic findings required a further bone marrow examination...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.10390
更新日期:2003-10-01 00:00:00
abstract::Autoimmune hemolytic anemia is thought to be mediated via auto-antibodies produced by lymphoid B cells. This may be an idiopathic process or secondary to an underlying infection or lymphoproliferative disorder. Conventional treatment comprises immunosuppression with corticosteroids and, in some cases, splenectomy. A p...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.20220
更新日期:2005-02-01 00:00:00