Abstract:
:The investigation of kindreds with recurrent thrombotic disease has advanced the understanding of the mechanisms of coagulation and fibrinolysis. In those cases where an etiology has been established, congenital thrombotic disorders are associated either with deficiencies or qualitative abnormalities in inhibitors of activated coagulation factors, qualitative abnormalities of fibrinogen, fibrinolytic defects that impair clot lysis, or an inborn error of metabolism, homocystinuria. The etiologies of congenital thrombotic disorders, their clinical features, and an approach to their laboratory diagnosis are summarized in this review.
journal_name
Am J Hematoljournal_title
American journal of hematologyauthors
Rodgers GM,Shuman MAdoi
10.1002/ajh.2830210411subject
Has Abstractpub_date
1986-04-01 00:00:00pages
419-30issue
4eissn
0361-8609issn
1096-8652journal_volume
21pub_type
杂志文章,评审abstract::Hb Saint Nazaire [beta 103 (G5) Phe-->Ile] was found in four apparently unrelated French families. The five patients carrying this hemoglobin have been detected because of a moderate erythrocytosis. The structural abnormality of Hb Saint Nazaire concerns the same residue as in Hb Heathrow [beta 103 (G5) Phe-->Leu). A ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830440105
更新日期:1993-09-01 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::Zoledronic acid (ZOL), an intravenous bisphosphonate, has been shown to reduce and delay the incidence of skeletal-related events (SREs) in multiple myeloma (MM) patients with bone disease. A retrospective claims-based analysis was conducted that used two distinct US managed care databases to examine the relationship ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23164
更新日期:2012-05-01 00:00:00
abstract::The serum free light chain (FLC) assay quantitates free immunoglobulin kappa and lambda light chains, which has prognostic value in plasma cell dyscrasias. However, there is limited data on serum FLC in lymphoid malignancies. We analyzed the association of pretreatment FLC with event-free survival (EFS) and overall su...
journal_title:American journal of hematology
pub_type: 杂志文章,多中心研究
doi:10.1002/ajh.22168
更新日期:2011-12-01 00:00:00
abstract::To better assess the efficacy and safety of rituximab in adults' warm antibody autoimmune hemolytic anemia (wAIHA), we conducted a retrospective study including 27 adults (mean age 49.7 +/- 21 years) with either primary (n = 17) or secondary (n = 10) wAIHA. On average, the patients received 2.1 +/- 1.4 treatment lines...
journal_title:American journal of hematology
pub_type: 临床试验,杂志文章
doi:10.1002/ajh.21341
更新日期:2009-03-01 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::We describe 3 episodes of microangiopathic hemolytic anemia (MAHA) in 2 solid organ recipients under FK506 (tacrolimus) therapy. In both cases, discontinuation of FK506 and treatment with plasma exchange, fresh frozen plasma replacement, corticosteroids, aspirin, and dipyridamole led to resolution of MAHA. In one pati...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(SICI)1096-8652(199608)52:4<310::AID-AJH11
更新日期:1996-08-01 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::Iron studies were compared in 434 patients from 80 hemochromatosis families classified as putative homozygotes, heterozygotes, and normal by HLA typing. There were 28 of 255 (11%) heterozygotes with an elevated serum ferritin and 22 of 255 (8.6%) with an elevated transferrin saturation. Serum ferritin (140 +/- 10.2 mi...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830450210
更新日期:1994-02-01 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::Acquired α-thalassemia myelodysplastic syndrome (MDS) (ATMDS) is an acquired syndrome characterized by a somatic point mutation or splicing defect in the ATRX gene in patients with myeloid disorders, primarily MDS. In a large MDS patient series, the incidence of ATMDS was below 0.5%. But no large series has yet assess...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.24073
更新日期:2015-08-01 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::The standard dose of clofarabine is 52 mg/m2 for pediatrics and 40 mg/m2 in adults. Clofarabine dosed at 52 mg/m2 was used in adult patients with refractory ALL to maximize response before allo-HSCT. All patients had a significant response to therapy. Published pharmacokinetic analysis revealed no difference in peak p...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.21365
更新日期:2009-04-01 00:00:00
abstract::This study concerned the gamma chain composition of Hb F and the haplotypes of 44 patients with beta-thalassemia major or intermedia and many of their relatives. Seventeen patients came from Northern (Turkish) Cyprus, 12 from the Istanbul area, and 15 from Macedonia and Bulgaria. Analysis of the A gamma T-G gamma-A ga...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830200103
更新日期:1985-09-01 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::In addition to transferrin, ferritin can also function as a source of iron for heme synthesis (Gelvin D, et al. Blood 1996;88:3200-3207; Meyron-Holtz EG, et al. Blood 1999;94:3205-3211). In the present study we investigate the effect of external ferritin on the proliferation and hemoglobinization of human erythroid pr...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.10355
更新日期:2003-07-01 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::Among a cohort of 142 patients with Hodgkin disease (HD), pathologic stages (PS) IA through IVB, 84 remained in complete remission (CR) at least 3 years after the completion of initial protocol therapy. Eight of these patients subsequently developed a recurrence of HD and were defined as having a late relapse. The dis...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830100106
更新日期:1981-01-01 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
abstract::A 10-month-old infant with chronic myelomonocytic leukemia (CMML) of 5 months' duration, who had been treated only with transfusion, displayed leukemic transformation characterized by lymphoid morphology, PAS positivity, and myeloperoxidase negativity. Surface marker analysis of blast cells revealed expression of lymp...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(SICI)1096-8652(199607)52:3<212::AID-AJH14
更新日期:1996-07-01 00:00:00
abstract::Platelet-rich plasma were treated with increasing concentrations of vitamin E (alpha-tocopherol). Washed platelets were exposed to oxidized low density lipoprotein (LDL) and examined by aggregometry and electron microscopy. The treatment of washed platelets by oxidized LDL induced morphological signs of activation lik...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(sici)1096-8652(199903)60:3<242::aid-ajh15
更新日期:1999-03-01 00:00:00
abstract::The value of menorrhagia as a predictor for mild bleeding disorders has been very little studied and the results are divergent. In the present study on 30 women with objectively verified menorrhagia, we found a significantly increased prevalence of von Willebrand's disease (20%). By keeping a strict sampling and labor...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(SICI)1096-8652(199612)53:4<234::AID-AJH4>
更新日期:1996-12-01 00:00:00
abstract::The role of peripheral platelet destruction as a reversible etiology of thrombocytopenia in chronic lymphocytic leukemia (CLL) was evaluated in nine patients with CLL and refractory thrombocytopenia who underwent splenectomy. The patients' ages ranged from 54 to 74 years. Progressive thrombocytopenia refractory to ant...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830150306
更新日期:1983-11-01 00:00:00
abstract::Twenty-seven lymphomas of mucosa-associated lymphoid tissue (MALT) derived from distinct anatomical sites were tested for the presence of genetic lesions commonly involved in B-cell lymphomagenesis, including activation of proto-oncogenes (BCL-1, BCL-2, BCL-6, and c-MYC), disruption of tumor suppressor loci (p53, 6q),...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(sici)1096-8652(199712)56:4<206::aid-ajh2>
更新日期:1997-12-01 00:00:00
abstract::Primary systemic light-chain (AL) amyloidosis a disorder characterized by accumulation of monoclonal light chains as aggregated amyloid fibrils in tissues of multiple organs to cause organ dysfunction and death (Kyle and Gertz, Semin Hematol 1995;32:45-59; Merlini and Bellotti, N Engl J Med 2003;349:583-596). Although...
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.23333
更新日期:2013-01-01 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::Older patients with acute myeloid leukemia (AML) have poor outcomes, with median durations of complete remission lasting less than 1 year. Increased toxicity in older patients limits the delivery of standard consolidation therapies, such as allogeneic stem cell transplant or high-dose cytarabine. Azacitidine, a nucleo...
journal_title:American journal of hematology
pub_type: 杂志文章,多中心研究
doi:10.1002/ajh.24087
更新日期:2015-09-01 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::Comparison of the chromosome findings obtained on routine examination (10-50 cells) of the marrows from patients with Ph1-positive CML with those based on a large number (110-500 cells) of metaphases in six of these patients, in whom appropriate material was available, revealed the presence of small percentages of ane...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830030202
更新日期:1977-01-01 00:00:00
abstract::A 6-year-old child of northern European ancestry was found to have microcytic, hypochromic anemia with an elevated level of hemoglobin A2 and an unbalanced pattern of globin chain synthesis characteristic of beta-thalassemia trait. Hematologic and globin synthesis studies of both parents yielded entirely normal result...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830040211
更新日期:1978-01-01 00:00:00