Abstract:
:We report a family with a dominantly transmitted syndrome resembling Fanconi's anemia and spanning two generations. This syndrome was characterized by an ill-defined hematologic stem cell disorder, immune dysfunction, poor dentition, hyperpigmented skin, warts, and multiple second trimester spontaneous abortions and included one case of acute myelomonocytic leukemia (acute non-lymphocytic leukemia, M4). This family lacks the characteristic chromosomal aberrations of Fanconi's anemia. We believe this constellation of findings represents an entity not previously described.
journal_name
Am J Hematoljournal_title
American journal of hematologyauthors
Alter CL,Levine PH,Bennett J,Kessler C,Rick M,Washburn RG,Gallin JI,Miller RW,Auerbach ADdoi
10.1002/ajh.2830320402subject
Has Abstractpub_date
1989-12-01 00:00:00pages
241-7issue
4eissn
0361-8609issn
1096-8652journal_volume
32pub_type
杂志文章abstract::Fifty-nine HIV-1 antibody positive and 58 antibody negative hemophiliacs were evaluated over a 2 year study period to gain insight into the natural history and prognosis of HIV-1 disease in members of this risk group. Mean CD4 (Leu 3+) cell counts calculated at 6 month intervals decreased gradually in seropositive pat...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830320406
更新日期:1989-12-01 00:00:00
abstract::We have characterized another subset of acute nonlymphocytic leukemia (ANLL) based on the cytogenetic and morphologic findings in a group of nine patients. Five patients had chromosomal analyses performed at the University of Chicago, two patients were studied at the All-Union Cancer Research Center in Moscow, and one...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830180409
更新日期:1985-04-01 00:00:00
abstract::We have previously shown that humic acid (well-water humic acid, HA, and synthetic humic acid, SHA) enhances cell surface expression of tissue factor (TF). Here we report that incubation of human umbilical vein endothelial cells (HUVEC) for 2 hr with HA or SHA cause a rapid rise in TF mRNA levels, as shown by Northern...
journal_title:American journal of hematology
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更新日期:1996-03-01 00:00:00
abstract::Argentine hemorrhagic fever (AHF) is a viral disease caused by Junin virus and characterized by hematologic and neurological involvement. The main hematologic features are leukopenia, thrombocytopenia, and bone marrow hypoplasia. Hematopoietic growth factors serum levels were measured by ELISA technique in forty-eight...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(sici)1096-8652(200005)64:1<1::aid-ajh1>3.
更新日期:2000-05-01 00:00:00
abstract::As the number of anticoagulant drugs increases and new ones are brought to market, the utility of the routine screening coagulation tests of today--namely the prothrombin time and activated partial thromboplastin time--will be significantly reduced in many clinical situations. Although the new anticoagulants are desig...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.22222
更新日期:2012-02-01 00:00:00
abstract::We have developed a murine monoclonal antibody (mAb) specific for the delta chain of hemoglobin (Hb) A2 that does not cross-react with alpha, beta, or gamma chains. The mAb reacted with Hb P-Nilotic (beta delta hybrid), but not with Hb Lepore-Boston (delta beta hybrid), indicating an epitope consisting of positions 11...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830380311
更新日期:1991-11-01 00:00:00
abstract::Eight patients with severe chronic autoimmune thrombocytopenic purpura (AITP) refractory to high-dose intravenous immunoglobulin (IVIgG) and/or oral prednisone were treated with one to three infusions of high-dose methylprednisolone (HDMP) (15 mg/kg/day). The mean platelet count before treatment was 12 +/- 10 x 10(9)/...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830480416
更新日期:1995-04-01 00:00:00
abstract::The effect of the synthetic vasopressin derivative 1-desamino-8D-arginine vasopressin (DDAVP = desmopressin) on bleeding time was studied in three patients with Hermansky Pudlak syndrome. A good response was observed in this type of storage pool disease. DDAVP might be useful in managing the bleeding disorder found in...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830300307
更新日期:1989-03-01 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::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::Ticlopidine-induced aplastic anemia (TIAA) is considered very uncommon. We present two new cases, and we review 55 additional cases from the literature. The first case concerns a 70-year-old man who developed severe aplastic anemia 7 weeks after treatment with 500 mg of ticlopidine daily. The patient sustained a sever...
journal_title:American journal of hematology
pub_type: 杂志文章,meta分析,评审
doi:10.1002/ajh.10150
更新日期:2002-09-01 00:00:00
abstract::Polycythemia vera (PV) is associated with a high incidence of thrombosis. The association of apparent and secondary polycythemia with thrombosis is not clear. It was suggested that activation of the coagulation system contributes to thrombus formation in PV. However, the mechanism of activation is unknown. Monocytes g...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(sici)1096-8652(199709)56:1<5::aid-ajh2>3.
更新日期:1997-09-01 00:00:00
abstract::The second generation BCR/ABL kinase inhibitor nilotinib is increasingly used for the treatment of imatinib-resistant chronic myeloid leukemia (CML). So far, nilotinib is considered a well-tolerated drug with little if any side effects, although an increase in the fasting glucose level has been reported. We examined a...
journal_title:American journal of hematology
pub_type: 临床试验,杂志文章
doi:10.1002/ajh.22037
更新日期:2011-07-01 00:00:00
abstract::Sitosterolemia (phytosterolemia) is a rare inherited sterol storage disorder, characterized by significantly elevated plasma levels of plant sterols. The clinical features of sitosterolemia are xanthomas, premature atherosclerosis, arthritis, and, occasionally, liver function impair and hematologic abnormalities. This...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23619
更新日期:2014-03-01 00:00:00
abstract::Botrocetin, a protein isolated from the venom of the snake Bothrops jararaca, induces platelet aggregation/agglutination by von Willebrand factor (vWF) binding to the membrane glycoprotein (GP) Ib, an action resembling that of ristocetin. However, some differences in the interaction between vWF and platelet GPIb induc...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830330409
更新日期:1990-04-01 00:00:00
abstract::Allogeneic hematopoietic stem cell transplantation (HSCT) represents the only curative option for primary hemophagocytic lymphohistiocytosis (HLH), a rare disease of infants and young children, characterized by recurrent fever, hepatosplenomegaly, and cytopenia. We report a case of successful engraftment and stable fu...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.10266
更新日期:2003-02-01 00:00:00
abstract::Hairy cell leukemia is a chronic lymphoproliferative disorder characterized clinically by splenomegaly and cytopenias. Spontaneous remissions are rare and splenectomy is often performed when the blood counts worsen and cause symptoms. Three of our patients with hairy cell leukemia developed recurrent pancytopenia and ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830250307
更新日期:1987-07-01 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
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abstract::At sites of vascular injury, the platelet collagen receptor Glycoprotein Ia/IIa (GPIa/IIa) acts as an important mediator of platelet adhesion to fibrillar collagens. Two silent polymorphisms (807C/T and 873G/A) within the glycoprotein Ia gene have been implicated in increased risk of developing thrombosis and myocardi...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.10029
更新日期:2002-01-01 00:00:00
abstract::A minority of super-utilizing adults with sickle cell disease (SCD) account for a disproportionate number of emergency department (ED) and hospital admissions. We performed a retrospective cohort study comparing the rate of admission before and after the opening of a clinic for adults with SCD. Unique to this clinic w...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23912
更新日期:2015-03-01 00:00:00
abstract::ASXL1 and SRSF2 mutations in AML are frequently found in patients with preexisting myeloid malignancies and are individually associated with poor outcomes. In this multi-institutional retrospective analysis, we assessed the genetic features and clinical outcomes of 43 patients with ASXL1mut SRSF2mut AML and compared o...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.26110
更新日期:2021-01-27 00:00:00
abstract::Recent observations that the sickle RBC are excessively susceptible to phagocytosis by macrophages in vitro prompted me to look for evidence of in vivo erythrophagocytosis (Ep) in patients with sickle cell anemia (SS). Freshly prepared smears of unmanipulated blood of 27 patients with SS in steady state were examined ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830200406
更新日期:1985-12-01 00:00:00
abstract::The Sickle Cell Disease Clinical Research Network (SCDCRN) designed the PROACTIVE Feasibility Study (ClinicalTrials.gov NCT00951808) to determine whether elevated serum levels of secretory phospholipase A2 (sPLA2) during hospitalization for pain would permit preemptive therapy of sickle cell acute chest syndrome (ACS)...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.22265
更新日期:2012-03-01 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::Three Indonesian patients with identical genotypes, each compound heterozygotes for Filipino beta(o)-thalassemia/HbE, expressed different clinical severities. One patient has mild disease and is transfusion independent, while the other two are severely affected and transfusion dependent. The size of the Filipino beta(...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(sici)1096-8652(199909)62:1<7::aid-ajh2>3.
更新日期:1999-09-01 00:00:00
abstract::Thrombospondin (TSP), a large protein found in platelet alpha-granules (as TSP-1), mediates adhesion of sickle reticulocytes to cultured vascular endothelium. To further explore the physiologic relevance of this observation, we have measured plasma TSP levels and platelet TSP-1 content in subjects with sickle cell dis...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(SICI)1096-8652(199604)51:4<296::AID-AJH8>
更新日期:1996-04-01 00:00:00
abstract::We studied 15 patients with essential thrombocythemia (ET) before treatment and after normalization of platelet count by anagrelide. Significantly increased plasma levels of PDGF, TGFbeta, and bFGF were found. Patients with mild reticulin fibrosis in bone marrow had higher PDGF levels. During treatment, plasma TGFbeta...
journal_title:American journal of hematology
pub_type: 临床试验,杂志文章
doi:10.1002/ajh.10091
更新日期:2002-06-01 00:00:00
abstract::We analyzed 12 MLL/ENL positive ALL patients consecutively diagnosed between 1999 and 2009. The MLL/ENL fusion was identified in 4/150 (2.6%), 8/993 (0.8%), and 0/70 of pediatric, adult, and elderly patients, respectively. Eight patients had a WBC count >50 × 10(9) /L. Ten cases had an evaluable immunophenotyping. A B...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.22161
更新日期:2011-12-01 00:00:00
abstract::Therapy-related acute myeloid leukemia (t-AML) arises as a late complication following antecedent solid tumors or hematologic diseases and their associated treatments. There are limited data regarding risk factors and outcomes following allogeneic hematopoietic cell transplantation (HCT) for t-AML following a prior so...
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.25395
更新日期:2019-04-01 00:00:00
abstract::A 63-year-old white man with myasthenia gravis and red cell aplasia was found to have hepatic iron overload. The differential diagnosis included hereditary hemochromatosis and secondary iron overload related to red cell aplasia. Family studies of siblings, including HLA typing, initially suggested a diagnosis of secon...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830450314
更新日期:1994-03-01 00:00:00