Abstract:
:This report describes studies investigating the use of a collagen binding assay to improve the laboratory monitoring of desmopressin (DDAVP) therapy in patients with von Willebrand's disease (vWD). We evaluated the response of seven patients with vWD (four type I, three type IIA) to DDAVP, administered using a standard protocol, by assessing levels of von Willebrand factor (vWF) and factor VIII, as well as performing skin bleeding times (SBT) prior to, and at sequential time points following, DDAVP administration. The study employed the following assays: von Willebrand factor antigen assay (vWF:Ag; determined by ELISA); a novel functionally based collagen binding assay (CBA; determined by ELISA); ristocetin cofactor assay (RCof; determined by platelet aggregometry); von Willebrand factor multimer analysis (using SDS-agarose gels); factor VIII coagulant (FVIIIC; determined by clotting assay); and factor VIII antigen (FVIIICAG; determined by ELISA). All patients showed an initial incremental increase in vWF/FVIII levels using all assays above, and some showed some correction in SBT. Although the absolute levels of vWF/FVIII antigen or activity varied between patients, the CBA was found to provide consistently the greatest proportional incremental increases (i.e., -fold) compared to baseline (pre-DDAVP) levels. Accordingly, we consistently observed an increase in the CBA to vWF:Ag ratio for all patients evaluated. This supplements previous findings that have suggested a unique ability of our CBA procedure to bind preferentially to higher molecular weight (i.e., more functionally active) forms of vWF. We therefore propose that the use of the above test combination (e.g., vWF:Ag plus CBA) may provide the basis for more accurate estimation of a patient's functional responsiveness to DDAVP therapy in future studies.
journal_name
Am J Hematoljournal_title
American journal of hematologyauthors
Favaloro EJ,Dean M,Grispo L,Exner T,Koutts Jdoi
10.1002/ajh.2830450303subject
Has Abstractpub_date
1994-03-01 00:00:00pages
205-11issue
3eissn
0361-8609issn
1096-8652journal_volume
45pub_type
杂志文章abstract::We prospectively treated 46 patients with favorable myelodysplastic syndrome classified as refractory anemia (RA), refractory cytopenia (RC), or refractory anemia with ringed sideroblasts (RARS). These patients received one of two schedules of 13-Cis-Retinoic Acid (low dose 80 mg daily for 6 months vs. high dose 200 m...
journal_title:American journal of hematology
pub_type: 临床试验,杂志文章,多中心研究,随机对照试验
doi:10.1002/ajh.2830480405
更新日期:1995-04-01 00:00:00
abstract::Levels of many coagulation factors are low in the healthy infant and even lower in the asphyxiated premature infant. We investigated whether a brief exposure to asphyxia at the time of birth causes the activation and consumption of coagulation factors. Following delivery by caesarean section, premature lambs were asph...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830280317
更新日期:1988-07-01 00:00:00
abstract::The effect of short-chain aliphatic acids on [3H]deoxythymidine incorporation into DNA was studied in human mitogen-stimulated lymphocytes. Butyric acid at 1-2 mM level was strongly inhibitory; however, its hydroxy or amino derivatives were ineffective. Valeric and propionic acids were less inhibitory. Formic, acetic,...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830130203
更新日期:1982-09-01 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::Transplant-associated thrombotic microangiopathy (TA-TMA) has a wide range of presentations after hematopoietic stem-cell transplantation (HSCT). We retrospectively studied the risk factors and outcomes of patients with early (≤day 100) and late (>day 100) TA-TMA. Among the 1451 HSCT recipients, early TA-TMA occurred ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.25922
更新日期:2020-07-02 00:00:00
abstract::The biosynthesis of the minor hemoglobin FIc, which contains acetylated gamma chains, and the major hemoglobin Fo was studied during erythroid cell differentiation and maturation in cultures of erythroid precursors isolated from five human umbilical cord blood samples. A gradual decrease in the synthesis of Hb FIc was...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830120302
更新日期:1982-05-01 00:00:00
abstract::Pulmonary hypertension (PH) of sickle cell disease (SCD), as defined by a tricuspid regurgitant jet velocity by echocardiogram of >or=2.5 m/sec, occurs in approximately 1/3 of HbSS adults and is an independent risk factor for mortality. Although studies of the past few years have greatly expanded our knowledge of the ...
journal_title:American journal of hematology
pub_type: 社论
doi:10.1002/ajh.21083
更新日期:2008-01-01 00:00:00
abstract::Serum lactic dehydrogenase (LDH) levels are mildly elevated in beta-thalassemia major due to ineffective erythropoiesis. We reviewed the charts of 15 consecutive thalassemic children who underwent allogeneic, T-cell-depleted bone marrow transplantation (BMT) in our department during the last 3 years. Eleven patients h...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(SICI)1096-8652(199602)51:2<166::AID-AJH13
更新日期:1996-02-01 00:00:00
abstract::The expression of mature B-cell markers and T markers was determined in lymphocytes isolated from the peripheral blood (PBL) of 20 healthy and 51 patients with non-Hodgkin malignant lymphoma (NHL). The disease was classified as newly diagnosed, in remission, or being treated with chemotherapy and of low-, intermediate...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830260203
更新日期:1987-10-01 00:00:00
abstract::Pure red cell aplasia (PRCA) sometimes accompanies thymoma. Herein, we report a PRCA patient with thymoma with a clonal disorder of T cells. A 55-year-old man presented with anemia and anterior mediastinum tumor. The laboratory study revealed hemoglobin 8.2 g/dl; leukocytes 15.8 x 10(9)/L with 76.5% neutrophils, 20.0%...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(sici)1096-8652(199704)54:4<324::aid-ajh12
更新日期:1997-04-01 00:00:00
abstract::We recently defined event-free survival at 24 months (EFS24) as a clinically relevant outcome for patients with DLBCL. Patients who fail EFS24 have very poor overall survival, while those who achieve EFS24 have a subsequent overall survival equivalent to that of the age- and sex-matched general population. Here, we de...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.24223
更新日期:2016-02-01 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::We studied the growth of erythroid burst-forming units (BFU-E) and erythroid colony forming units (CFU-E) from bone marrow and blood in six patients with erythroleukemia. Five patients grew CFU-E, while BFU-E were found in the marrow of two and in the peripheral blood of only one patient. In all cases with colony grow...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830050403
更新日期:1978-01-01 00:00:00
abstract::An elderly patient with evidence of atherosclerosis and uremic bleeding diathesis developed two foci of cerebral thrombosis immediately after an infusion of desmopressin (DDAVP). Because large molecular weight multimers of von Willebrand factor (vWF) have been demonstrated to cause platelet aggregation under condition...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830280115
更新日期:1988-05-01 00:00:00
abstract::Karyotypic analysis at time of diagnosis has an important value in determining initial response to treatment, remission duration and overall survival (OS) in acute myeloid leukemia (AML). Less is known about its value before allogeneic hematopoietic cell transplantation (allo-HCT) in patients transplanted with active ...
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.26000
更新日期:2021-01-01 00:00:00
abstract::Despite advances in chemotherapeutic agents, the prognosis for some cancers remains extremely poor, suggesting the need for other treatment modalities. Immunotherapy appears an ideal approach because the mechanisms of tumor cell killing induced by tumor vaccines are different from those from chemotherapy. Various inve...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.20415
更新日期:2005-09-01 00:00:00
abstract::Most reports of prognosis and therapy in diffuse small non-cleaved cell lymphoma (DSNCL), an aggressive high-grade non-Hodgkin's lymphoma (NHL) which appears to be of two histopathologic subtypes, have included predominantly a pediatric population and very few adults. We studied 20 newly diagnosed, previously untreate...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830460408
更新日期:1994-08-01 00:00:00
abstract::We report a case of T-cell chronic lymphoproliferative disorder (CLPD) that shows neither features of T-cell prolymphocytic leukemia nor disease progression for more than 34 months. Flow cytometric analyses of the lymphocytes revealed high expression of CD4 and CD25. Up-regulation of Foxp3, a master regulatory gene fo...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20688
更新日期:2006-09-01 00:00:00
abstract::Since trisomy 12 is the most common chromosome abnormality found in CLL and MDM2 has been mapped to this chromosome, we examined the possible association of MDM2 in the pathogenesis of CLL. A rearrangement of the MDM2 gene was observed in 4 of 11 peripheral blood mononuclear cells (PBMC) from patients with CLL by Sout...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830470215
更新日期:1994-10-01 00:00:00
abstract::The Muir-Torre syndrome (MTS) is defined as the concurrent or sequential discovery of at least one sebaceous gland tumor and a minimum of one internal malignancy. A man with Hodgkin's lymphoma who subsequently developed an ocular sebaceous carcinoma in situ is described and the world literature of patients with the MT...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830400114
更新日期:1992-05-01 00:00:00
abstract::Acute lymphoblastic leukemia (ALL) with distinct fusion transcripts has unique clinical features. In this study, the incidence, clinical characteristics, early treatment response, and outcomes of 1,004 Chinese pediatric ALLs were analyzed. Patients with TEL-AML1 and E2A-PBX1 fusion genes or other B cell precursor ALLs...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23307
更新日期:2012-11-01 00:00:00
abstract::A single-tube osmotic fragility test has been proposed for thalassemia screening with a range of different concentrations of saline having been employed. We have compared the sensitivity and specificity of 0.32%, 0.34%, and 0.36% buffered saline, and on the basis of our findings, recommend the use of 0.36% saline. Thi...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20387
更新日期:2005-07-01 00:00:00
abstract::Thalassemia is one of the most prevalent monogenic diseases usually caused by quantitative defects in the production of β-globin leading to severe anemia. Technological advances in genome sequencing, stem cell selection, viral vector development, transduction and gene editing strategies now allow for efficient exvivo ...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.25909
更新日期:2020-09-01 00:00:00
abstract::Community practice experience allows a nonselective care of patient using information derived from a more controlled clinical trial environment. We present our community experience with multiple myeloma patients with advanced age, long disease duration since diagnosis, advanced stage, multiple prior therapies includin...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20883
更新日期:2007-07-01 00:00:00
abstract::Endothelin-1 (Et-1) is a vasoconstrictor produced by endothelial and vascular smooth muscle cells in response to hypoxia, which induces hypertrophy in cultured cardiac myocytes. We measured plasma Et-1 levels and left ventricular dimensions in 13 patients with sickle cell anemia (SCD) and in 12 African-American contro...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(sici)1096-8652(199807)58:3<195::aid-ajh6>
更新日期:1998-07-01 00:00:00
abstract::The ability of circulating progenitor cells to develop erythroid colonies was studied in vitro in the presence or absence of growth factors (5637-CM and erythropoietin) in 63 patients with sickle cell disease (SCD) (36 homozygotes for hemoglobin [Hb] S, 13 double heterozygotes for Hb S and beta thalassemia, and 14 SC ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(sici)1096-8652(199905)61:1<40::aid-ajh8>3
更新日期:1999-05-01 00:00:00
abstract::Congenital malformations of the inferior vena cava (IVC) are uncommon and may be associated with an increased risk of venous thrombosis. We report the case of a man with congenital absence of the IVC and remote history of deep venous thrombosis who now presents with severe abdominal wall superficial thrombophlebitis. ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.21089
更新日期:2008-03-01 00:00:00
abstract::Variable survival outcomes are seen following treatment for aggressive non-Hodgkin lymphoma (NHL). This study examined whether outcomes for aggressive B-cell NHL are associated with single nucleotide polymorphisms (SNPs) in oxidative stress-related genes, which can alter drug metabolism and immune responses. Genotypes...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23709
更新日期:2014-06-01 00:00:00
abstract::A case of leukemia cutis (LC) of monocytic lineage in a patient with myelodysplastic syndrome (MDS) is presented. Cutaneous infiltrates were recognized concurrent with diagnosis of refractory anemia (RA) with monocytosis. Skin infiltrates subsequently spontaneously regressed although MDS progressed with increasing mon...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/1096-8652(200102)66:2<120::AID-AJH1027>3.0
更新日期:2001-02-01 00:00:00
abstract::Leg ulcers are a well recognized complication of sickle cell disease that has been attributed to venous insufficiency. We studied 16 patients with sickle cell disease and active ulcers using venous pulse volume recordings and photoplethysmography (Doppler studies). Based on hemodynamic monitoring, all 16 patients exhi...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830370215
更新日期:1991-06-01 00:00:00