摘要:

AbstractWe examined the changes in plasma levels of endothelin‐1 (ET‐1), a potent vasoconstrictor peptide, in 47 cases of disseminated intravascular coagulation (DIC) to investigate the role of ET‐1 in DIC and its relation to multiple organ failure (MOF). A significant elevation of plasma levels of ET‐1 was observed in some cases of DIC, especially in patients with sepsis, blastic crisis of chronic myelogenous leukemia, and cancer. However, no such significant elevation was observed in patients with acute promyelocytic leukemia (APL), acute leukemias except for APL, or non‐Hodgkin lymphoma. Plasma levels of ET‐1 were higher in patients with DIC with MOF than in those without MOF. Although the levels of ET‐1 were decreased or remained low with clinical improvement in most DIC patients, the levels were further increased or remained high in patients who showed no improvement in DIC. It is suggested that ET‐1 must play an important role in further progression of MOF with the vasoconstriction and microcirculatory disorders. © 1992

ISSN:0361-8609    

DOI:10.1002/ajh.2830410202

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

年代:1992

数据来源: WILEY

摘要:

AbstractTo examine the role of 5‐aminolevulinate synthase (ALAS) in the pathogenesis of sideroblastic anemias, levels of mRNAs for erythroid and housekeeping ALAS isozymes were examined, and total ALAS activity was assessed in bone marrow cells. In two patients with X‐linked sideroblastic anemia the levels of mRNA for erythroid ALAS as well as for α and β globin appear to be decreased while levels of mRNA for glycophorin A in both patients were the same as in normal individuals. However, amounts of housekeeping ALAS mRNA were increased two‐ to threefold in these patients. Total ALAS activity was also increased two‐ or threefold, perhaps reflecting increased transcription of the housekeeping gene in response to diminished cellular heme in erythroid cells and/or enhanced translation of the erythroid isoform in response to iron accumulation. In a third patient with X‐linked sideroblastic anemia ALAS activity was low but increased to twice the normal value after pyridoxine administration, suggesting a structural defect of the enzyme. In a fourth patient, with isolated congenital, pyridoxine‐responsive sideroblastic anemia, the erythroid ALAS mRNA was normal and a low enzyme activity was strikingly enhanced by pyridoxal‐phosphate albeit to subnormal levels. In idiopathic acquired sideroblastic anemia, ALAS mRNA for both isozymes was normal and enzyme activity was slightly elevated. These observations thus reflect heterogeneous aberrations of erythroid heme synthesis in the various types of sideroblastic anemia and suggest that defects involving erythroid ALAS underlie at least some of them. © 1992

ISSN:0361-8609    

DOI:10.1002/ajh.2830410203

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

年代:1992

数据来源: WILEY

摘要:

AbstractWe have investigated the biological activities of recombinant human interleukin‐9 (IL‐9) on enriched hematopoietic progenitors, alone or in combination with other cytokines, including Epo, G‐CSF, IL‐3, and GM‐CSF, under serum‐containing and serum‐free cultures. IL‐9 alone did not support colony formation. However, IL‐9 plus Epo induced erythroid burst (BFU‐E) formation derived from peripheral blood (PB) progenitors. Delayed addition experiments demonstrated that a part of bone marrow (BM) derived BFU‐E, which seems to be immature, only responded to IL‐9 and formed erythroid bursts. The burst‐promoting activity (BPA) of IL‐9 was confirmed using neutralizing aIL‐3, aGM‐CSF, and aIL‐9 antisera and serum‐free culture. IL‐9 supported a part of BFU‐E population that respond to IL‐3, which was almost identical to the number of BFU‐E supported by GM‐CSF. IL‐9 had no additive effect on erythroid and mixed colony formation supported by IL‐3. In contrast, IL‐9 showed an additive effect on erythroid burst formation supported by GM‐CSF in serum‐free culture. These data suggest that IL‐9 and GM‐CSF act on distinct IL‐3‐responsive BFU‐E population. In addition, delayed addition experiment clearly demonstrated that IL‐9 supports survival and the early stage of proliferation of BFU‐E. These results led us to propose that IL‐9 possibly acts as a BPA and selectively supports a subpopulati

ISSN:0361-8609    

DOI:10.1002/ajh.2830410204

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

年代:1992

数据来源: WILEY

摘要:

AbstractThe effect of lupus anticoagulant (LA) positive plasma on the expression of human monocyte procoagulant activity (PCA) was studied. LA positive plasma were able to enhance the endotoxin or TNF alpha induced monocyte associated PCA. The monocyte PCA had the characteristic of tissue factor activity (factor VII, factor × dependence). The enhancement of monocyte PCA could be confirmed using purified LA positive IgG. The stimulating effect was supported by the F(ab')2 fragments. © 1992 Wiley‐Liss,

ISSN:0361-8609    

DOI:10.1002/ajh.2830410205

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

年代:1992

数据来源: WILEY

摘要:

AbstractWe have sequenced the 5′ hypersensitive‐2 (5′HS‐2) site of the locus control region (LCR) and the promoters of the two γ‐globin genes located on chromosome 11 of a black patient with mild β‐thalassemia (β‐thal) major due to a homozygosity for the C → T mutation at position – 88 of the β promoter and with a high Hb F level. Sequence variations in the 5′HS‐2 were the same as observed for the βschromosome with haplotype number 3, while most of theGγ promoter and theAγ promoter had sequences similar to that of the βschromosome with haplotype number 19. This atypical haplotype (number 19A) is apparently associated with an increased γ chain production which is particularly evident during periods of severe hematopoietic stress. Additional studies on relatives of the proband and on 10 unrelated black β‐thal homozygotes with either the C → T mutation at – 88 or the A → G mutation at –29, confirm the possible importance of the sequence differences in the 5′HS‐2, and also suggest that at least two additional factors, namely a C → T mutation at position –158 of theGγ promoter and a relative deficiency in α chain synthesis play a (perhaps less important) role in the increased Hb F

ISSN:0361-8609    

DOI:10.1002/ajh.2830410206

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

年代:1992

数据来源: WILEY

摘要:

AbstractBecause B lymphocytes bearing the CD5 antigen have been involved in many B‐cell malignancies, we have investigated the presence of the CD5 B‐cell antigen on B and plasma cells in monoclonal gammopathy. Quantification of CD5 B cells was made in the peripheral blood of seven individuals with monoclonal gammopathy of undetermined significance (MGUS) and in that of 21 patients with multiple myeloma (MM). The bone marrow of ten patients with MM was also studied. Patients with progressive MM presented a significant reduction in both B and CD5 B lymphocytes (i.e., percentages and absolute numbers), when compared with individuals with MGUS and patients with stable MM. These latter individuals and patients did not differ from healthy donors. No CD5 B cells were found in the bone marrow of patients with MM. Moreover, no CD5 antigen could be detected on eight freshly established human myeloma cells lines including six totally dependent on Interleukin‐6. However, it was weakly expressed on two standard myeloma cell lines not requiring exogenous interleukin‐6 (i.e., RPMI 8226 and U 266). In conclusion, our data show mainly an overall reduction of the polyclonal CDS B lymphocytes similar to what is observed for the other polyclonal B lymphocytes in patients with active MM. Finally, the expression of the CDS antigen human myeloma cell lines is not constant. © 1992 Wiley

ISSN:0361-8609    

DOI:10.1002/ajh.2830410207

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

年代:1992

数据来源: WILEY

摘要:

AbstractFor sufficient collection of hemopoietic stem cells from peripheral blood for autologous peripheral blood stem cell transplantation (PBSCT), four patients with B‐cell‐type non‐Hodgkin lymphoma (B‐NHL) were examined for the appearance of circulating hemopoietic progenitors in blood (PSC) during the hemopoietic recovery phase following marrow ablative therapy in combination with or without administration of recombinant human granulocyte colony‐stimulating factor (rhG‐CSF). Each patient received only chemotherapy in the first course, and rhG‐CSF (1 βg/kg/day) was administered for 14 consecutive days from the last day of the second chemotherapy. In the second chemotherapy course with rhG‐CSF administration, white blood cell (WBC) counts demonstrated two peaks, and the appearance of granulocyte–macrophage precursor cells (CFU‐GM) in blood at the maximum level was coincident with the second peak of WBC elevation. Erythroid precursor cells (BFU‐E) were also detectable in blood after chemotherapy but the peak level was not enhanced by the use of rhG‐CSF. To determine whether the minimal residual disease (MRD) cells were contaminated in PSC corrected from blood, kappa–lambda imaging (KLI) analysis was performed to detect the malignant B‐cell population (mBp) before and after chemotherapy. No mBp was found in two of four patients in blood, although three of them were involved with mBp in bone marrow. The presence of mBp was detected in two patients both before and after chemotherapy, even though these cells were hardly detected morphologically, suggesting the necessity of judging for the incidence of contamination of MRD cells when collecting

ISSN:0361-8609    

DOI:10.1002/ajh.2830410208

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

年代:1992

数据来源: WILEY

摘要:

AbstractThe laboratory evaluation of patients with recurrent thrombosis is frequently frustrating, with a low diagnostic yield obtained despite extensive testing. The likelihood of reaching a diagnosis in these patients can be increased by considering diagnostic possibilities usually overlooked and by using assays optimal for their detection. This review summarizes clinical and laboratory issues important in inherited thrombotic disease and discusses practical aspects and a strategy for laboratory testing. New information is provided on the fibrinolytic disorders that may be a common cause of recurrent thrombosis. © 1992 Wiley‐Liss, I

ISSN:0361-8609    

DOI:10.1002/ajh.2830410209

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

年代:1992

数据来源: WILEY

摘要:

AbstractBecause extracorporeal photopheresis has been shown to be clinically effective in Sezary syndrome, a disease characterized by a circulating malignant clone, we initiated a pilot study of its use in B‐cell chronic lymphocytic leukemia (B‐CLL) to see if it could be similarly effective. We treated three patients with Rai stage III and IV B‐CLL with photopheresis (3 consecutive days a week every 3 weeks) and followed serial clinical and immunologic parameters. While we noted no major toxicity, there was neither clinical response to treatment nor marked improvement in white counts or mitogen assays. We conclude that photopheresis was not effective in inducing remission in this pilot study of advanced stage B‐CLL patients. © 1992 Wiley

ISSN:0361-8609    

DOI:10.1002/ajh.2830410210

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

年代:1992

数据来源: WILEY

摘要:

AbstractA 17‐year‐old girl presented with a lymphoproliferative disease involving the bone marrow, peripheral blood, and liver associated with reactive hyperplasia of the spleen. Neoplastic cells were atypical medium‐sized lymphoblasts with convoluted nuclei and nucleoli without features of large granular lymphocytes (LGL). The phenotype was CD3+CD4‐CD8‐, TCRα/β‐, TCRγ/δ+, δTCS1‐. and CD16+, and these cells exhibited spontaneous natural killer (NK) activity. DNA analysis showed rearrangement of the TCRγ gene but not of TCRβ or of lg μ genes. This unusual lymphoproliferative disease may represent the neoplastic expansion of a minor subset of normal Tγ/δ cells with NK activity.

ISSN:0361-8609    

DOI:10.1002/ajh.2830410211

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

年代:1992

数据来源: WILEY