ISSN:0361-8609    

DOI:10.1002/ajh.2830240314

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1987

数据来源: WILEY

摘要:

AbstractA functionally normal but structurally abnormal prothrombin variant was found in a Mexican family. Immunoisoelectricfocusing studies revealed that this variant has a more acidic isoelectric point (4.01) than normal prothrombin (4.29), but it proved to have a normal molecular weight as assessed by sodium‐dodecyl‐sulphate polyacrylamide gel electrophoresis. Two‐dimensional immunoelectrophoresis studies showed an abnormal cleavage of the prothrombin molecule by factor Xa andEchis carinatusvenom as well, despite the fact that both yield functionally normal thrombin molecules. Finally, the ability of the molecule to bind calcium ions as well as its overall antigenic structure were investigated and found to be normal. These results taken together suggest a simple (substitution or translocation) mutation at the fragment 2 level. Since this prothrombin variant is different from others described previously, the name “Mexico City” is proposed to id

ISSN:0361-8609    

DOI:10.1002/ajh.2830240302

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1987

数据来源: WILEY

摘要:

AbstractRecent data published on the prevalence of inhibitors to factor VIII in hemophiliacs on treatment show great variations, with prevalence rates ranging from 3.6 to 14.2%. We have studied the cumulative risk of inhibitor development in a cohort of 62 patients with hemophilia A. All patients were born after 1960, were natives of the Vienna area, had a factor VIII activity of less than 5%, and were treated at least once. Using the method of Cutler and Ederer, the cumulative risk of inhibitor development was found to be 24% at the age of 25 years. Most inhibitors developed between the ages of 3 and 7 years. The current prevalence of F VIII inhibitors in the group of patients studied is 17.5%. It is concluded that prevalence data underestimate the true risk of inhibitor development.

ISSN:0361-8609    

DOI:10.1002/ajh.2830240303

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1987

数据来源: WILEY

摘要:

AbstractAcquired von Willebrand disease (AVWD) has been described in two cases of nephroblastoma. We studied a patient with nephroblastoma who presented with a coagulopathy suggestive of AVWD. The subject had undetectable levels of F. VIIIR: Ag, diminished F. VIIIR: WF (16.3%), F. VIII: C activity (37%), and lack of platelet aggregation to ADP, epinephrine, collagen, and arachidonic acid. These results were associated with abnormally high serum levels (850 mg/dl) of hyaluronic acid (HA), which made the patient's serum hyperviscous. Examination of the neoplasm revealed HA in the tumor matrix. All abnormalities of coagulation resolved after chemotherapy and extirpation of the neoplasm, which produced normal serum HA levels.To study the effects of HA on platelet function, we added HA to normal platelet‐poor plasma (NPP), which rendered F. VIIIR: Ag undetectable; treatment of HA with hyaluronidase eliminated F. VIIIR: Ag assay interference caused by HA. F. VIII: C activity decreased in vitro when HA was mixed with normal platelet‐poor plasma (NPP). HA reduced the initial slope of normal platelet aggregation. Partial correction of platelet aggregation occurred after hyaluronidase treatment of HA‐spiked PRP. Experiments in rabbits exposed to HA (serum level 400 mg/dl) demonstrated abnormalities similar to those noted in the patient. Shear rate studies of whole blood containing HA (500 mg/dl) yielded high shear stress, 27‐136 dynes/cm2over shear rates of 10‐216 sec‐1. We conclude that the coagulopathy demonstrated in this case is secondary to hyperviscosity produced by elevated levels of HA, which interferes with the assay for F. VIIIR: Ag. Thus the acquired coagulopathy associated with other cases of nephroblastoma may present as spurious von Willebr

ISSN:0361-8609    

DOI:10.1002/ajh.2830240304

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1987

数据来源: WILEY

摘要:

AbstractWe have characterized the plasma and platelet von Willebrand factor (vWf) multimeric structure of a patient with von Willebrand disease (vWd) as having a long bleeding time, no aggregation of her platelet‐rich plasma (PRP) to ristocetin, and very low plasma and platelet von Willebrand antigen (vWf Ag) and vWf activity. The abnormalities of the plasma and platelet vWf have not been previously described. In particular, the patient's plasma and platelet vWf lacked the intermediate and largest vWf multimers and the slowest migrating minor band (band 1) of the triplet was markedly diminished compared to the major band (band 2) and the fastest migrating band of the triplet (band 3). A similar multimeric structure was seen in the platelet vWf. Collection of the patient's blood in protease inhibitors prior to the analysis of the platelet and plasma vWf structure revealed that the plasma vWf multimeric structure did not change, while the patient's platelet vWf showed a marked change with the appearance of the intermediate and large multimers and almost total disappearance of the abnormal multimeric structure. Direct comparison of this patient's plasma with previously reported cases of type IIA, IIC, and IID revealed marked differences in their multimeric organization compared to our patient. Employing the presently accepted convention, we have designated this variant type of vWd type II

ISSN:0361-8609    

DOI:10.1002/ajh.2830240305

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1987

数据来源: WILEY

摘要:

AbstractThe hematological aspects of the original case of Rhmodare reported. The subject, as in other reported cases, had a chronic hemolytic anemia characterized by stomatocytosis, reduced osmotic fragility, and abnormal autohemolysis correctable with the addition of glucose. The51Cr red cell survival studies showed the spleen to be the preferential site of red cell destruction and splenectomy produced a dramatic improvement in red cell survival. The topic of Rh deficiency syndrome (Rhnulland Rhmod) is briefly reviewed with regard to the number of cases reported, to genetic aspects, to the hematological findings, and to the results of splenectomy.

ISSN:0361-8609    

DOI:10.1002/ajh.2830240306

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1987

数据来源: WILEY

摘要:

AbstractA 4‐hour in vivo test for iron chelator activity in the rat is described. The amount of radioiron in the gut and urine that results from chelator‐induced excretion of previously injected radioiron labelled ferritin is measured. Hepatocyte localization and desferrioxamine‐induced radioiron mobilization from in vitro tagged homologous ferritin is shown to be similar to that from in vivo tagged ferritin. Non‐homologous ferritin preparations labelled in vitro proved unsatisfactory. Radioiron mobilization by chelator occurred regardless of the iron status of the animal. Employing this measurement, the effectiveness of three iron chelators, pyridoxal isonicotinoyl hydrazone, the dimethyl ester of ethylene diamine‐N, N′‐dis (3‐hydroxyphenyl acetic acid), and the dimethyl ester N, N′‐di (3‐hydroxybenzyl) ethylene‐1, ethylene‐1, 2‐diamine‐N, N′‐diacetic acid, given orally was determined. All three chelators, when given in comparable dosage, induced iron execretion similar in amount to that observed with parenteral desferrioxamine. In addition, pyridoxal isonicotinoyl hydrazone administered in the diet over a period of 4 weeks was shown to reduce hepatic and splenic iron of normal animals by about one third, providing further validation of this method of

ISSN:0361-8609    

DOI:10.1002/ajh.2830240307

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1987

数据来源: WILEY

摘要:

AbstractPure red cell aplasia (PRCA) is an unusual complication of chronic lymphoproliferative disorders. A patient with T‐cell chronic lymphocytic leukemia (T‐CLL) had severe anemia and neutropenia. Initial in vitro studies demonstrated no evidence of T‐cell suppression of erythropoiesis. Sequential bone marrow examinations demonstrated progressive red cell aplasia. In vitro studies showed that the T‐cells from the patient suppressed allogeneic but not autologous BFU‐E. Treatment with antithymocyte globulin (ATG) reduced circulating leukemic cells and produced a definite but transient improvement in eryth

ISSN:0361-8609    

DOI:10.1002/ajh.2830240308

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1987

数据来源: WILEY

摘要:

AbstractThe emergence of a near‐haploid clone of cells in blast phase chronic myelogenous leukemia is an unusual event. We report such a case and review eight other cases described in the English literature. The significance of the substantial loss of genetic material is discussed as is the phenotypic and genotypic heterogeneity observed in this group of patient

ISSN:0361-8609    

DOI:10.1002/ajh.2830240309

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1987

数据来源: WILEY

摘要:

AbstractA Chinese patient with hemoglobin (Hb) H and Hb New York is described. Unlike classical Hb H disease, the additional βNew Yorkchain defect resulted in a more marked α β 3‐globin chain imbalance in this patient with only one α‐gene, accounting for her severe anemia (3.4‐6.8 g/dl) during the ste

ISSN:0361-8609    

DOI:10.1002/ajh.2830240310

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1987

数据来源: WILEY