摘要:

AbstractThe syndrome thalassemia intermedia can be the clinical expression of heterozygosity for different types of thalassemia, β‐thalassemia and hereditary persistence of fetal hemoglobin, β‐thalassemia and Hb‐Lepore, and in blacks it may even represent a true β‐thalassemia homozygote. This report describes thalassemia intermedia in a white male due to β‐thalassemia and an unstable hemoglobin. Chain‐synthesis studies showed an excess of β‐chain production over β‐chain production in the propositus and his mother but balanced chain synthesis in the clinically normal father, who is heterozygous for the unstable hemoglobin. The unstable hemoglobin was found to be β14(A11) Leu→Pro, which has previously been described in a clinically normal African woman, and named Hb‐Saki. This hemoglobin is not distinguishable from Hb‐A on routine electrophoresis at alkaline or acid pH and tests for unstable hemoglobins are necessary for its detection. The increasing list of such hemoglobin variants and previous cases of heterozygosity for β‐thalassemia and un

ISSN:0361-8609    

DOI:10.1002/ajh.2830010302

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

年代:1976

数据来源: WILEY

摘要:

AbstractIn order to study characteristics of leukocytes which would be important determinants of their flow in the microcirculation, a model system was tested which utilizes in vitro filtration of leukocytes. Normal human peripheral blood leukocytes (85–90% granulocytes) were studied with filters with uniform 8 μm pore size.Studies were performed to determine the effects of EDTA, temperature, hydrostatic pressure, pH, and osmolarity on filtration. Filterability was optimal at 0.2% EDTA, 10 cm hydrostatic pressure, neutral pH, isotonicity, and at room temperature.Filtration was slowed greatly at leukocyte concentrations exceeding 25 × 199/liter. When leukocyte membranes were altered by formalin fixation, filtration slowed greatly, indicating that deformability is an important determinant of flow through small orifices. When mixtures of erythrocytes and leukocytes were filtered, there was a paradoxically enhanced transit of leukocytes compared to filtration of leukocytes alone, indicating interactions between these cells which alter flow.These studies serve to characterize this model system which can be used to study the contribution to flow in the microcirculation of both normal and pathological leukocy

ISSN:0361-8609    

DOI:10.1002/ajh.2830010303

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

年代:1976

数据来源: WILEY

摘要:

AbstractNeutrophilic leukocytes of patients with familial Mediterranean fever and of normal control subjects were studied in vitro. FMF neutrophils were found to be morphologically normal by light and electron microscopy and to have normal quantities of the lysosomal enzyme lysozyme. FMF cells demonstrated a slight decrease in their ability to migrate randomly in capillary tubes, this was primarily seen in Armenian patients and in those experiencing an acute attack. The leukocytes of these patients functioned normally in regard to their chemotactic and Candida‐killing activit

ISSN:0361-8609    

DOI:10.1002/ajh.2830010304

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

年代:1976

数据来源: WILEY

摘要:

AbstractThe absence of ristocetin‐induced platelet aggregation appears to correlate with the platelet defect in von Willebrand's disease, suggesting that this reaction mimics a physiological process. The effect of ristocetin on plasma and on the residual levels of the von Willebrand factor (vWF), Factor VIII procoagulant activity, and Factor VIII‐related protein in plasma after aggregation of platelet rich plasma by this agent has been studied in order to further elucidate the mechanism and requirements of this reaction. Ristocetin‐induced platelet aggregation causes a consumption of vWF, Factor VIII procoagulant activity, and Factor VIII antigen from the supernatant plasma which is proportional to the number of platelets aggregated. Such a consumption of these factors does not appear to occur after aggregation by other agents. Factor VIII procoagulant activity does not appear necessary for ristocetin‐induced platelet aggregation, yet is utilized in this process. These findings support the hypothesis that the molecule associated with Factor VIII procoagulant activity is carried by the molecule necessary for ristocetin‐induced platelet ag

ISSN:0361-8609    

DOI:10.1002/ajh.2830010305

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

年代:1976

数据来源: WILEY

摘要:

AbstractCombined deficiencies of Factor VIII and Factor XI associated with moderate degree of bleeding symptoms were found in 3 brothers. Examination of Factor VIII activity and Factor VIII‐related antigen revealed that the Factor VIII activity/ Factor VIII‐related antigen ratio was significantly decreased in their mother and maternal grandmother consistent with the carrier state of hemophilia. Factor XI deficiency was found in 2 siblings, the father, and 2 of his sisters. The paternal grandmother was thought to carry the abnormal Factor XI gene, although her Factor XI level was normal, because of a significant bleeding history. It was concluded that the combined Factor VIII and XI deficiencies in the 3 brothers represent the coincidental inheritance of 2 separate and independent abnormal ge

ISSN:0361-8609    

DOI:10.1002/ajh.2830010306

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

年代:1976

数据来源: WILEY

摘要:

AbstractPurified plasma cell suspensions were produced from 3 subjects with malignant plasma cell disease. Various physical, biological, and immunological properties of these cells were studied. Neoplastic human plasmablasts were found to be denser than their mature forms, in contrast to the usual relationship of the lymphoid, myeloid, and erythroid series. The human plasma cells did not respond to phytohemagglutinin stimulation and were found to be weakly reactive in mixed lymphocyteplasma cell cultures. Potent antisera was produced against such cells, and the antisera demonstrated a broad cross reactivity with various human lymphocyte populations. The data suggest a lymphoid origin for human plasma cells and possibly a specific relationship to B‐type lymphocyte

ISSN:0361-8609    

DOI:10.1002/ajh.2830010307

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

年代:1976

数据来源: WILEY

摘要:

AbstractThe improved microchromatographic procedure for the detection of Hb‐S and/or Hb‐C in cord blood at birth (Schroeder et al.: J. Lab Clin Med 86:528‐532, 1975) as well as a modification thereof may also be used for the quantitative determination of Hb‐F in the presence of Hb‐S and/or Hb‐C. However, Hb‐A interferes and must be absent. The methods use 0.5 × 6 cm columns of CM‐cellulose with Tris or Bis‐tris developers and require 2‐4 hr to complete. At low percentages of Hb‐F, the sharper zone of the Tris method is more easily visible than that of the Bis‐tris method, but the latter is a somewhat more rapid procedure.About 300 cases with Hb‐S and/or Hb‐C have been examined by the micro‐ chromatographic procedure. Most of these results (Fmicro) have been compared with data from the determination of Hb‐F by one or more of the following methods: alkali denaturation (FAD), conventional DEAE‐Sephadex chromatography (FDES), or isoleucine analyses of zones from DEAE‐Sephadex chromatography (FIle). The accuracy and precision of the microchromatographic method is estimated to be 5‐10%.The microchromatographic methods require much less time than conventional chromatography but more time than alkali denaturation procedures. Compared to the latter, the new methods use whole blood and less blood and

ISSN:0361-8609    

DOI:10.1002/ajh.2830010308

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

年代:1976

数据来源: WILEY

摘要:

AbstractPost‐transfusion thrombocytopenic purpura (PTTP) is a rare syndrome which was first brought to widespread attention by Shulman et al. Eighteen cases have been reported. The present report describes an additional patient, a multiparous female who developed petechiae 7 days after blood transfusion. The patient's serum contained anti‐PIA1antibodies and her own platelets were PIA1‐negative. She was treated with prednisone and recovered spontaneously without exchange transfusion of whole blood or p

ISSN:0361-8609    

DOI:10.1002/ajh.2830010309

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

年代:1976

数据来源: WILEY

摘要:

AbstractTwo teenage brothers with recurrent thromboembolic disease were found to have antithrombin III deficiency. A family study spanning four generations revealed a total of 10 members with antithrombin III deficiency. Five of the 10 affected family members have had thrombotic problems. Antithrombin III deficiency was documented by coagulation assays measuring heparin cofactor, anti‐Factor Xa, and progressive antithrombin activity; the level of antithrombin III antigenic material measured by immunoelectrophoresis was low in subjects with abnormal coagulation assays. The clinical features which may lead one to suspect the hereditary hypercoagulable condition of antithrombin III deficiency are reviewe

ISSN:0361-8609    

DOI:10.1002/ajh.2830010310

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

年代:1976

数据来源: WILEY

摘要:

AbstractData presently available suggest that polycythemia vera is an acquired clonal disorder of the pluripotent stem cell resulting in expansion of committed stem cell pools, most prominently in the erythroid line. In vitro and in vivo studies suggest that erythropoiesis in polycythemia vera is at least partially modulated by erythropoietin.

ISSN:0361-8609    

DOI:10.1002/ajh.2830010311

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

年代:1976

数据来源: WILEY