摘要:

AbstractThe effects of conformational changes in purified canine von Willebrand's factor (VWF) were investigated to explore the relationship between its factor VIII‐related antigen (VIIIR:AG) and ristocetin cofactor (RCoF) properties and the factor VIII‐coagulant (VIII:C) binding site(s). Binding of VIII:C from canine von Willebrand's disease (VWD) plasma by VWF was used to measure the combining reaction of these proteins. The VWF was denatured to varying degrees by exposure to temperature and pH extremes, low ionic strength, and 6 M urea. Various treatments resulted in three types of change: elimination of RCoF, VIIIR:Ag, and VIII:C binding, removal of RCoF activity alone; or elimination of RCoF and retarded elution of VIIIR:Ag and VIII:C. As long as VIIIR:Ag reactivity was maintained, binding of VIII:C could be demonstrated; but in the absence of VIIIR:Ag, neither RCoF activity nor VIII:C binding remained. These results suggest that VIII:C binding and RCoF sites are separate on VWF and that interaction between VIII:C and VWF is possible even after significant structural changes occur in VWF. Furthermore, RCoF is more vulnerable to denaturation than the antigenic site. The spectrum of conformational changes that affect the properties of VWF may parallel the various recognized subtypes of

ISSN:0361-8609    

DOI:10.1002/ajh.2830220202

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

年代:1986

数据来源: WILEY

摘要:

AbstractTwenty‐two normal volunteers had approximately eight, 2‐hr‐long leukapheresis procedures over a 2‐year period and their natural killer (NK) cell function was prospectively measured. The NK activity of the preprocedure peripheral blood (pre‐PB) was found to correlate well with the NK activity of the inital leukocytes removed by leukapheresis (I‐Leuk). When the I‐Leuk specimens were compared with the leukapheresis specimens removed at the termination of leukapheresis (T‐Leuk), T‐Leuk showed a consistent 10% increase in NK activity. When the pre‐PB and the I‐Leuk values were analyzed for each donor over the 2 years of the study, 18 donors revealed no significant change from their baseline NK activity, two donors showed a minimal increase in NK cell activity, and two donors displayed a minimal decrease in NK cell activity. We conclude that although leukapheresis appears acutely to boost NK cell activity, this increase is transient and small in magnitude. Most importantly, repeated leukapheresis does not appear adversely to effect this important effector func

ISSN:0361-8609    

DOI:10.1002/ajh.2830220203

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

年代:1986

数据来源: WILEY

摘要:

AbstractSurgery with extracorporeal circulation (ECC) is associated with transient alterations of platelet function whose pathogenic mechanisms are not completely understood. To define further the platelet abnormalities, we determined the changes in platelet content of several granule‐associated substances during and after ECC in patients subjected to aortocoronary bypass surgery.Platelet β‐thromboglobulin (β‐TG) decreased to 79.8% of the preoperative level at the end of ECC (p<0.01) and, as expected, did not recover 1 hr after the end of surgery. Platelet fibrinogen and albumin decreased to 67.9% (p<0.01) and to 29.8% (p<0.01) of baseline, respectively. However, 1 hr after surgery, platelet fibrinogen rose to 92% and albumin to 55.5% of baseline, denoting that during the recovery from ECC, platelets incorporate some plasma proteins.During ECC, platelet 5‐hydroxytryptamine (5‐HT) and total ATP and ADP decreased to 50.8% (p<0.01), 63.2% (p<0.01), and 69.9% (p<0.01) of their respective preoperative values, indicating dense body release. One hour after surgery, ATP recovered to 83.8%, suggesting that previous depletion compromised also the metabolic pool of adenine nucleotides.In summary, 1) Our results confirm and extend previous observations demonstrating α‐granule release during ECC. 2) Platelets undergoing ECC can incorporate plasma proteins as evidenced by the rapid increase of platelet fibrinogen and albumin after bypass. Although the mechanisms of this increase and site of storage of the exogenous substances are unknown, this observation justifies further studies to determine if internalization of plasma proteins, especially fibrinogen, may take place in physiological conditions. 3) Dense body depletion with transient storage pool deficiency appears to be a component of the reduced platelet function during ECC. 4) Consumption of metabolic ATP with alteration of platelet energy metabolism may further impair platelet function, contributing to the bleeding episodes observed during su

ISSN:0361-8609    

DOI:10.1002/ajh.2830220204

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

年代:1986

数据来源: WILEY

摘要:

AbstractPeripheral blood samples collected from normal individuals and patients with benign monoclonal gammopathy and multiple myeloma were separated and assayed for their T‐ and B‐cell subpopulations as well as immunoglobulin (IgG, IgM) synthesis in vitro. To rule out any functional or quantitative alterations related to therapy, only newly diagnosed multiple myeloma patients and subjects with benign monoclonal gammopathy were included in our study. Multiple myeloma patients were further subdivided into two groups. Group A consisted of patients with low and intermediate tumor burdens, while group B included patients with high tumor mass. B‐ and T‐cell subset analysis revealed an abnormal ratio between B/T and OKT4+/OKT8+lymphocytes compared to healthy controls. These alterations were especially prominent in group B multiple myeloma. Immunoglobulin synthesis was studied in pokeweed‐mitogen‐stimulated lymphocyte cultures. The results indicate that the failure to synthesize and secrete immunoglobulin resides within the B‐cell lineage and is probably due to a functional B‐cell defect. T‐cell immunoregulatory functions seem to be unaffected in both multiple myleoma and benign monoclonal ga

ISSN:0361-8609    

DOI:10.1002/ajh.2830220205

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

年代:1986

数据来源: WILEY

摘要:

AbstractClinical and cytogenetic details of 12 patients with polycythemia vera and complete or partial trisomy of the long arm of chromosome 1 are reported. All patients had trisomy for at least the segments 1q22 to 1qter. The 1q or material from 1q was translocated to another chromosome in eight patients. This was chromosome 9 in four patients, and those cases all had trisomy also for 9p. The trisomy 1q was found at the time of diagnosis in three patients, later during the polycythemic phase in five, and in four patients when they were first examined during a late stage of the disease. Acute leukemia or a myelodysplastic syndrome developed in eight of the 12 patients. Signs of advanced disease, eg, myeloid metaplasia or myelofibrosis, preceded the leukemia in four cases and was noted in one more patient.Trisomy 1q was the most frequent structural chromosome abnormality in patients with polycythemia vera. It is thus one of several nonrandom abnormalities that can appear at any stage of the disease. It seems to occur with higher frequency in patients with myelofibrosis and/or leukemia, but it is not a specific characteristic of these complications.

ISSN:0361-8609    

DOI:10.1002/ajh.2830220206

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

年代:1986

数据来源: WILEY

摘要:

AbstractThe secretion of interferon (IFN)‐γ by T lymphocytes is mediated by the synthesis of interleukin 2 (IL‐2) and the availability of IL‐2 receptors. Since some Burkitt's lymphoma lines express Tac antigen and can be triggered to secrete IL‐2 following activation with the new tumor promoter teleocidin, we addressed the question of whether the induction of IL‐2 by B lymphocytes is accompanied by the induction of IFN‐γ. IFN‐γ has not been detected in any of the 25 cell lines studied, and following stimulation with teleocidin, we triggered the synthesis of IFN‐γ in JLP(C), a pre‐Burkitt's cell line.The mechanism of IFN‐γ secretion by B lymphocytes is not clear. Our findings demonstrate that the synthesis of IL‐2 by B cells is not accompanied by IFN‐γ and suggest that the synthesis of IFN‐γ is not mediated by IL‐2 or IL‐1 or B‐cell growth factor. Neutralization studies have shown that IFN‐γ secretion is not accompanied by the induction of IFN‐α or IFN‐β.Our data imply that B cells can be triggered to secrete IFN‐γ under certain circumstances. Wh

ISSN:0361-8609    

DOI:10.1002/ajh.2830220207

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

年代:1986

数据来源: WILEY

摘要:

AbstractA comparison between the immunofluorescent (IF) method for terminal deoxynucleotidyl transferase (TdT) activity and the immunoperoxidase (IP) method by peroxidase‐anti‐peroxidase (PAP) technique was done for 100 cases of acute leukemia. For the acute lymphoblastic leukemias (ALL) there was agreement in 93% of the cases. However, the IP method detected 51/55 (93%) TdT+cases versus 47/55 (85%) by the IF method. For the acute nonlymphocytic leukemias (ANLL), there was an agreement in 89% of the cases. The IP method detected 8/36 (22%) TdT‐positive cases while IF detected 4/36 (11%) positive cases. If a figure of 10% TdT+cells is considered significant in the marrow of the ANLLs, then the IP method would detect eight additional cases for a total of 16/36 (44%) TdT+cases. This latter figure questions the ability of the IP TdT assay as a single test adequately to determine the lineage of a cell line. It may be rather that TdT is a marker that is expressed in a stem

ISSN:0361-8609    

DOI:10.1002/ajh.2830220208

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

年代:1986

数据来源: WILEY

摘要:

AbstractTwo glucose‐6‐phosphate dehydrogenase (G6PD) variants were investigated. G6PD Amman‐1 was partially purified from the red cells of a patient suffering from recurrent jaundice and spontaneous episodic attacks of severe hemolysis in the absence of oxidant drugs, infection, or fava beans. The enzymatic characteristics of G6PD Amman‐I were markedly reduced activity, fast eletrophoretic mobility, slightly increased km for NADP, normal km for G‐6‐P, normal heat stability, normal utilization of substrate analogues 2‐deoxy G‐6‐P and deamino‐NADP, and a monophasic pH curve with a peak at 8.5 to 9.3. The second variant, G6PD Amman‐2, was partially purified from the red cells of a patient suffering from recurrent jaundice with episodic mild hemolysis caused by infection or unknown factors. G6PD Amman‐2 characteristics were severely reduced activity, slow electrophoretic mobility, normal km for NADP, decreased km for G‐6‐P, decreased heat stability, increased utilization of substrate analogues, and a monophasic pH curve with a narrow peak at pH 9.5. The red cell level of reduced glutathione was markedly decreased with twofold increase in the activity of glutathione reductase in th

ISSN:0361-8609    

DOI:10.1002/ajh.2830220209

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

年代:1986

数据来源: WILEY

摘要:

AbstractWe report the occurrence of immunoglobulin‐related amyloidosis (AL) in three separate families, each family having two members affected. None of the six patients had evidence to suggest the presence of familial amyloidosis (AF). Previously, immunoglobulin‐related amyloidosis (AL) was considered to be a sporadic disease process. Because of the occasional familial occurrence of other monoclonal gammopathies such as monoclonal gammopathy of undetermined significance, multiple myeloma, and macroglobulinemia of Waldenström, amyloidosis (AL) should be added to the list of immunopathies with a familial predisposi

ISSN:0361-8609    

DOI:10.1002/ajh.2830220210

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

年代:1986

数据来源: WILEY

摘要:

AbstractA 28‐year‐old man with Ph‐positive chronic granulocytic leukemia (CGL) was treated by high‐dose chemoradiotherapy and transplantation of marrow cells harvested from his HLA‐identical brother. One year after bone marrow transplantation (BMT) examination of his marrow showed a minority population of Ph‐positive cells; their proportion subsequently fell such that 2 years after transplant analysis of marrow cells showed only cytogenetically normal cells. The patient remains clinically normal with a persisting mild lymphocytosis but without hematological evidence of leukemia. We cannot in this patient distinguish between persisting leukemia that later could no longer be recognized and relapse of leukemia that is now suppressed, perhaps only temporarily. This case emphasizes the need for caution in interpreting chromosomal finding after

ISSN:0361-8609    

DOI:10.1002/ajh.2830220211

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

年代:1986

数据来源: WILEY