Abstract:
:Two patients from two separate families were diagnosed as having type IIB von Willebrand disease, because they had lifelong bleeding tendencies, prolonged bleeding times, no large von Willebrand factor multimers, and low levels of ristocetin cofactor in plasma with heightened ristocetin-induced platelet aggregation. There was no history of bleeding, and no laboratory abnormalities were found in the parents and sibship of either propositi, in contrast with the autosomal dominant pattern of inheritance usually observed in type IIB von Willebrand disease. Abnormalities of ristocetin-induced von Willebrand factor-platelet interactions were less severe than in a patient from a previously reported family with type IIB von Willebrand disease studied in parallel. The peculiar features of these cases provide additional evidence of the existence of heterogeneity within this variant.
journal_name
Am J Hematoljournal_title
American journal of hematologyauthors
Federici AB,Mannucci PM,Bader R,Lombardi R,Lattuada Adoi
10.1002/ajh.2830230410subject
Has Abstractpub_date
1986-12-01 00:00:00pages
381-90issue
4eissn
0361-8609issn
1096-8652journal_volume
23pub_type
杂志文章abstract::Human theta (theta 1)-globin gene represents a member of the alpha-like globin gene family residing on chromosome 16. theta 1-Specific transcripts have been detected so far only in erythroid tissues and in erythroleukemia K562 cells. To investigate systematically its inducible expression and developmental specificity,...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830350406
更新日期:1990-12-01 00:00:00
abstract::We report the long-term follow-up results of a phase II trial of thalidomide for early-stage multiple myeloma (MM). Patients were eligible if they had smoldering multiple myeloma (SMM) or indolent MM without the need for immediate therapy. Thalidomide was initiated at a dose of 200 mg/day and adjusted as tolerated. Di...
journal_title:American journal of hematology
pub_type: 临床试验,杂志文章
doi:10.1002/ajh.21821
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abstract::Celecoxib, a specific cyclooxygenase-2 (Cox-2) inhibitor, has been shown to possess antitumor activity in a variety of cancer cells. However, the antitumor activity of celecoxib in hematopoietic tumors, especially in chronic myeloid leukemia (CML), has not been well established. This study was designed to investigate ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20542
更新日期:2006-04-01 00:00:00
abstract::Acetylsalicylic acid (ASA) is currently recommended as an antithrombotic for patients with essential thrombocythemia (ET) who are at an increased risk of thrombotic events. However, ASA is also associated with an increased risk of bleeding in these patients as compared to the risk of bleeding in other patients treated...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(sici)1096-8652(199804)57:4<277::aid-ajh2>
更新日期:1998-04-01 00:00:00
abstract::Flow cytometry (FCM) is an adjunct study to routine analysis of cerebrospinal fluid (CSF) to investigate for involvement by a hematologic malignancy. However, in our experience, FCM only infrequently detects abnormalities in CSF. To help optimize resources without forfeiting clinically important data, we sought to det...
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.23806
更新日期:2014-10-01 00:00:00
abstract::Positron emission tomography-computed tomography (PET-CT) can identify bony lesions, assess disease burden, and detect extramedullary disease (EMD) in patients with multiple myeloma. We retrospectively reviewed records of patients who underwent PET-CT within 60 days of a new diagnosis (before therapy commenced) to ide...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.25279
更新日期:2018-12-01 00:00:00
abstract::Studies of in vitro chemotaxis and spontaneous migration of human leukocytes using the accepted method with the Boyden-chamber-filter are troublesome, because of the need for specially constructed vessels as well as the difficulties caused by the use of membrane filters. We describe a new and simplified method for mea...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830140308
更新日期:1983-05-01 00:00:00
abstract::The levels of adenosine deaminase (ADA), purine nucleoside phosphorylase (PNP), lactic dehydrogenase (LDH), and LDH isoenzyme patterns (LD1 to LD5) have been measured in lymphocyte extract from 28 patients with B-chronic lymphocytic leukemia (B-CLL). The activities of ADA, PNP, and LDH have been correlated with two mo...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830270302
更新日期:1988-03-01 00:00:00
abstract::Extramedullary meningeal hematopoiesis (EMH) represents an uncommon finding after stem-cell transplantation. We describe the case of an allogeneic bone marrow transplantation (BMT) recipient who developed EMH 1 month after radiation myelitis had been diagnosed. A 39-year-old man with multiple myeloma underwent matched...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20341
更新日期:2005-08-01 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::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::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::The -117(G-->A)A gamma hereditary persistence of fetal hemoglobin (Greek HPFH) and beta zero 39-thal mutations are rather frequent in Sardinia so that their interaction is to be expected. Characterization of eight compound heterozygotes for these defects indicated that HPFH was linked to haplotype VII and beta zero 39...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830490402
更新日期:1995-08-01 00:00:00
abstract::Reverse transcription quantitative polymerase chain reaction (RTqPCR)is currently the most sensitive tool available for the routine monitoring of disease level in patients undergoing treatment for BCRABL1 associated malignancies. Considerable effort has been invested at both the local and international levels to stand...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23212
更新日期:2012-07-01 00:00:00
abstract::We report a case of a patient who developed a fatal adult respiratory distress syndrome (ARDS) during treatment with rh granulocyte-macrophage colony stimulating factor (rhGM-CSF) (250 mcg/m2/day s.c.) and low-dose cytosine-arabinoside (Ara-C) (20 mg/m2/day s.c.). Several mechanisms which might explain the lung tissue...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830360413
更新日期:1991-04-01 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::A 50-year-old woman with muscular fatigue and monoclonal gammopathy of unknown significance was shown to have high levels of antibodies against the acetylcholine receptor of the skeletal muscle endplate. The specific antibody activity was exclusively associated with the monoclonal component. There were no objective si...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830290112
更新日期:1988-09-01 00:00:00
abstract::Ten patients developing lymphomas after disease modifying anti-rheumatic drugs (DMARD) (methotrexate, n = 3, mean cumulative dose = 3.4 g; cyclophosphamide, n = 2, mean dose = 70 g; azathioprine, n = 6, mean dose = 243 g) were investigated. Methotrexate-related lymphomas were Epstein-Barr virus (EBV)-positive, had inf...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20508
更新日期:2006-01-01 00:00:00
abstract::Serologic diagnosis for Epstein-Barr virus (EBV) infection is problematic when patients receive exogenous immunoglobulin. We recently diagnosed primary EBV infection by detecting EBV-determined nuclear antigen (EBNA) and EBV-DNA in peripheral blood mononuclear cells (PBMC) using immunofluorescence, in situ hybridizati...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830360416
更新日期:1991-04-01 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::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::Precursor B-ALL blasts may be positive for CD20 in up to 50% of cases. There are few reports on the use of the anti CD20 monoclonal antibody, rituximab, in children with B-ALL. We report on two adult patients with precursor B-ALL who developed significant hepatic toxicity during induction chemotherapy. Single agent ri...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20675
更新日期:2006-12-01 00:00:00
abstract::Pentosan polysulfate is a low-molecular-weight sulfated polysaccharide used as an antithrombotic drug. We present two patients who developed thrombocytopenia and venous thrombosis during treatment with pentosan polysulfate. The relationship between pentosan polysulfate and thrombocytopenia is supported by platelet agg...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.2830450312
更新日期:1994-03-01 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::Plerixafor, a recently approved peripheral blood progenitor cell mobilizing agent, is often added to granulocyte-colony stimulating factor (G-CSF) to mobilize peripheral blood progenitor cells in patients with lymphoma or myeloma who cannot mobilize enough CD34+ cells with G-CSF alone to undergo autologous stem cell t...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23499
更新日期:2013-09-01 00:00:00
abstract::Pulmonary alveolar proteinosis (PAP) is a heterogeneous disease that occasionally develops with hematological malignancy. However, PAP in association with hematopoietic stem cell transplantation is quite rare. Here we present the first report of a patient who developed PAP after cord blood transplantation (CBT). A 45-...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.10107
更新日期:2002-06-01 00:00:00
abstract::Matching for HLA-A, -B, -C, and -DRB1 loci (8/8 match) is currently the gold standard for unrelated donor hematopoietic cell transplantation (HCT). In Europe, patients are also matched at the HLA-DQB1 loci (10/10 match). However, there is increasing evidence that matching at HLA-DRB3/4/5 loci may help to lower transpl...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.25133
更新日期:2018-05-04 00:00:00
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journal_title:American journal of hematology
pub_type: 信件,随机对照试验
doi:10.1002/ajh.21827
更新日期:2010-10-01 00:00:00
abstract::The presence of teardrop-shaped red cells in peripheral blood has traditionally been felt to reflect altered marrow architecture, namely myelofibrosis. We evaluated two patients with splenomegaly, moderately severe hemolytic anemia due to warm-reactive IgG anti-red cell autoantibody, and bone marrow erythroid hyperpla...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830210410
更新日期:1986-04-01 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