摘要:

AbstractIdiopathic thrombocytopenic purpura (ITP) is a disorder characterized by increased platelet destruction in the setting of normal megakaryopoiesis. Approximately 20% of patients with ITP are refractory to corticosteroids and splenectomy. Recently, pulse high‐dose dexamethasone was reported to be effective in the treatment of chronic ITP in adult patients. We treated 9 patients with severe chronic ITP with monthly high‐dose dexamethasone. None of the 9 patients responded with a sustained increase in platelet count. Five of these patients were unable to tolerate the regimen. The failure of high‐dose dexamethasone in our hands contrasts with the good results of an earlier publication and suggests that there could be a subset of responders who will require better identification. Am. J. Hematol. 54:267–270, 1997. © 1997 Wiley

ISSN:0361-8609    

DOI:10.1002/(SICI)1096-8652(199704)54:4<267::AID-AJH1>3.0.CO;2-T

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

年代:1997

数据来源: WILEY

摘要:

AbstractThe efficacy of the three common intra‐ and extragenic polymorphic sites of the factor VIII and IX genes has been examined in the Indian population, with an aim to develop a strategy that would be accurate and informative, yet economical. The approach for hemophilia A carrier detection includes tests for Bcll, Xbal, and Taql polymorphic sites for introns 18 and 22 and the extragenic locus St 14, respectively, whereas for hemophilia B, tests include detection of Taql, Ddel, and Hhal polymorphic sites for introns 4 and 1, and the 3′ flanking region of the factor IX gene, respectively. In hemophilia A, the cumulative efficiency of these three polymorphisms has been found to be 100%, since all 37 tested families were informative for at least one of these three polymorphisms. It is of interest to note that a case of recombination between St 14 and the factor VIII gene was also observed. Of the 47 unrelated X chromosomes examined (normal = 10, factor VIII:C deficiency = 37), heterozygosity for Bcll, Xbal, and St 14 was found to be 47%, 36%, and 86%, respectively, in the factor VIII gene. However, when 37 unrelated X chromosomes (normal = 10, factor IX:C = 27) were analyzed for polymorphism with Taql, Ddel, and Hhal, it was found that the polymorphism detection rate was only 18% for the Taql site but 45% each for the Ddel and Hhal sites, in the factor IX gene. This indicates a low effectiveness of the Taql restriction site in carrier analysis of hemophilia B families in our population. Am. J. Hematol. 54:271–275, 1997 © 1997 Wiley‐L

ISSN:0361-8609    

DOI:10.1002/(SICI)1096-8652(199704)54:4<271::AID-AJH2>3.0.CO;2-S

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

年代:1997

数据来源: WILEY

摘要:

AbstractIn order to elucidate the possibility of lymphoproliferation in cases of chronic active Epstein‐Barr virus infection (CAEBV), to clarify the clonality and genotype of proliferating lymphocytes, and to search for the factors that induce lymphoproliferation, we studied 11 cases of CAEBV, using genetical and immunological techniques.Epstein‐Barr virus (EBV) DNA in peripheral mononuclear cells was detected in eight cases by Southern blotting. Among those eight cases, monoclonal proliferation of EBV DNA‐positive cells was observed in three cases and oligoclonal proliferation in three cases. In the cases of monoclonal proliferation, one case manifested T‐cell lymphoproliferation and the rest natural killer (NK) cell lymphoproliferation. The anti‐EBV antibody titers in the study did not have any relativity to lymphoproliferation. On the other hand, three of the four cases of NK cell lymphoproliferation and one of the two cases of T‐cell lymphoproliferation exhibited hypersensitivity to mosquito bites (HMB) in their clinical histories, while none of the three nonlymphoproliferation cases did.These facts indicate that T‐cell and NK cell lymphoproliferative diseases (LPDs) could be more closely associated with EBV infection than we had previously expected. Also, the anti‐EBV antibody titers may not be the indicator of EBV‐associated LPD, and HMB may be one of the factors that induce EBV‐associated LPD. Am. J. Hematol. 54:276–281, 1997.

ISSN:0361-8609    

DOI:10.1002/(SICI)1096-8652(199704)54:4<276::AID-AJH3>3.0.CO;2-S

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

年代:1997

数据来源: WILEY

摘要:

AbstractThe role of microtubules in the brefeldin A (BFA)‐associated relocation of major histocompatibility complex (MHC) class II αβ chains (αβ) and the invariant chain (Ii) was characterized in Raji cells by the use of nocodazole (ND). BFA blocked the transport of αβIi proteins through the Golgi and redistributed them to the endoplasmic reticulum (ER) along with Golgi‐resident enzymes. The result of the colocalization of processing enzymes and newly synthesized proteins was a downshift of αβIi molecular weight (MW) of 2 kDa, and their resistance to endoglycosidase H (endo H) after 6 hr of chase. ND by itself had no effect on the processing and transport of αβ to the cell surface. The addition of ND to BFA‐treated cells downshifted αβIi by 4 kDa. Additionally, αβIi proteins remained sensitive to neuraminidase after 16 hr of chase. In vitro α‐mannosidase treatment of immunoprecipitated αβIi generated a similar 4‐kDa downshift of MW. Either 1‐deoxymannojirimycin (DJN) or swainsonine (SWN) blocked the MW downshift caused by BFA + ND treatment. These observations indicated that in Raji cells, most of the BFA‐associated relocations of cis‐, medial Golgi proteins, and the addition of sialic acid from the trans‐Golgi were microtubule‐independent. The retrograde transport of the medial Golgi enzyme N‐acetylglucosamine transferase, however, required microtubular function. Microtubule disrupters could affect BFA treatment of viral infections by further disrupting viral protein processing. Am. J. Hematol. 54:

ISSN:0361-8609    

DOI:10.1002/(SICI)1096-8652(199704)54:4<282::AID-AJH4>3.0.CO;2-R

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

年代:1997

数据来源: WILEY

摘要:

AbstractThe formation of inositol phosphates was compared in aspirin‐treated, washed human platelets suspended in Tyrode's‐albumin solution containing 2 mM calcium and stimulated with SFLLRN (thrombin receptor‐activating peptide) or thrombin. SFLLRN (20 μM) and thrombin (1 U/ml) resulted in maximal irreversible aggregation and 80–90% secretion of dense granule contents. SFLLRN (50–100 μM) caused larger increases at 10 sec than 20 μM SFLLRN in the formation of inositol trisphosphate (IP3, measured as [3H]inositol label). These increases were not significantly less than those caused by thrombin (1 unit/ml). However, whereas the labeling of IP3increased from 10–60 sec with thrombin, with SFLLRN it was much less at 60 sec than that at 10 sec. The decrease was not due to degradation of SFLLRN by ectopeptidases, since it was not prevented by amastatin, an inhibitor of ectopeptidases. Degradation of glycoprotein Ib (GPIb) with an O‐sialoglycoprotein endopeptidase did not affect the thrombin‐stimulated labeling of inositol phosphates, indicating that binding to GPIb is not involved in the sustained thrombin‐induced formation of inositol phosphates. The finding that the thrombin‐stimulated formation of IP3was not dependent on Ca2+in the medium (EGTA added) indicates that the transient SFLLRN‐induced formation of IP3is not due to failure to cause Ca2+influx. The finding that formation of IP3was transient in SFLLRN‐stimulated platelets, whereas platelet aggregation and secretion were maximal, indicates that the sustained activation of phospholipase C caused by thrombin may have roles related to later processes in which platelets participate. Am. J. Hematol. 54:288–295,

ISSN:0361-8609    

DOI:10.1002/(SICI)1096-8652(199704)54:4<288::AID-AJH5>3.0.CO;2-R

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

年代:1997

数据来源: WILEY

摘要:

AbstractTo determine the incidence and prognostic significance of thrombocytopenia among hemophiliacs, we analyzed clinical and hematologic data from the Multicenter Hemophilia Cohort study. Nineteen percent of HIV‐infected subjects had thrombocytopenia (platelet count of35 years compared to younger subjects. The risk increased after an AIDS‐defining illness, particularly among older subjects, nearly one‐half of whom had thrombocytopenia within 1 year after AIDS. When adjusted for age and CD4‐positive lymphocyte counts, thrombocytopenia was associated with an increased risk of death [relative risk (RR) 1.7, 95%Cl = 1.2–2.3] but with little change in the risk of progression to AIDS (RR = 1.2, 95%Cl = 0.8–1.7). Treatment with zidovudine was associated with a decreased risk of thrombocytopenia (RR = 0.5, 95%Cl = 0.3–0.7). Although 59 HIV‐infected subjects died of hemorrhage, only 11 (19%) of the 59 had a reported platelet count of<50,000/mm3, and only 2 (3%) of the deaths were temporally associated with thrombocytopenia. Thus, the risk of death was increased for thrombocytopenia HIV‐infected hemophiliacs but this was not explained by an increased risk of developing AIDS and was rarely associated with death from bleeding. Am. J. Hematol. 54:296–300, 1997.

ISSN:0361-8609    

DOI:10.1002/(SICI)1096-8652(199704)54:4<296::AID-AJH6>3.0.CO;2-Q

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

年代:1997

数据来源: WILEY

摘要:

AbstractPrevious reports have established the synthesis of interleukin‐6 (IL‐6) and IL‐6 receptors (IL‐6R) in several human leukemia cells and found that IL‐6 and the IL‐6R could be expressed in cell lines with erythroid/megakaryocytic features. IL‐6 is a pleiotropic cytokine involved in megakaryocytic differentiation. The finding that endogenous IL‐6 levels in serum increased after 5‐fluorouracil (5‐FU) treatment suggests that IL‐6 may play some role in the recovery of hematopoietic systems. This observation may assist the understanding of erythroid regeneration caused by antineoplastic agents such as tiazofurin. Tiazofurin inhibits the activity of IMP dehydrogenase. Its exposure to K562 cells at 10 μM tiazofurin stimulates erythroid differentiation. Stimulation of cells with tiazofurin gave a significant increase in IL‐6 production. Its levels were quadrupled after 2 days of culture. Tiazofurin also caused a trivial reduction in the percentage of cells with the IL‐6R. This evidence implies that tiazofurin produced no significant effect on the IL‐6R. Tiazofurin also increased the percentage of benzidine‐positive cells representing hemoglobin production, confirmed by GpA expression. We concluded that IL‐6 is rate limiting in regard to hemoglobin production and that IL‐3 could be used for clinical benefit to stimulate erythropoiesis and synergize with tiazofurin. Am. J. Hematol. 54:301

ISSN:0361-8609    

DOI:10.1002/(SICI)1096-8652(199704)54:4<301::AID-AJH7>3.0.CO;2-Z

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

年代:1997

数据来源: WILEY

摘要:

AbstractAdult T‐cell leukemia (ATL) is a neoplasm of mature helper (CD4) T lymphocytes, and human T‐cell lymphotropic virus type‐I (HTLV‐I) has been suggested to be the causative virus of ATL. HTLV‐I integrates its proviruses into random sites in host chromosomal DNA. Clonal integration has been observed in patients with ATL, including smoldering, chronic, and acute states. However, random and/or polyclonal integration has only been reported in a few asymptomatic HTLV‐I carriers. To clarify the clonality of HTLV‐I‐infected cells in carriers, we used an inverse polymerase chain reaction (IPCR), which is more sensitive than Southern blot analysis. We used the peripheral blood momonuclear cells (PBMC) from 16 asymptomatic carriers and the separated CD4‐positive cells. No cases showed either a monoclonal or polyclonal integration of the HTLV‐I provirus by Southern blot. But, using IPCR, 7 of 16 cases showed either mono‐ or oligoclonal integration. In addition, the populations of clonal provirus in the total PBMC were frequently different from those in the CD4‐positive cells. Three cases showed expression of HTLV‐I tax/rex mRNA in the total PBMC, but no such expression was found in CD4‐positive cells. In this study, an unexpected frequency of clonal HTLV‐I provirus DNA was observed in HTLV‐I carriers. These findings indicate that the clonal but nonmalignant proliferation of HTLV‐I‐infected cells already occurs even in HTLV‐I carriers, and therefore that some other step is necessary to induce malignant proliferation. Am. J. Hemato. 54:

ISSN:0361-8609    

DOI:10.1002/(SICI)1096-8652(199704)54:4<306::AID-AJH8>3.0.CO;2-Z

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

年代:1997

数据来源: WILEY

ISSN:0361-8609    

DOI:10.1002/(SICI)1096-8652(199704)54:4<313::AID-AJH9>3.0.CO;2-Y

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

年代:1997

数据来源: WILEY

摘要:

AbstractLymphoproliferative disorder of natural killer cells is a heterogeneous disorder, and an association with Epstein‐Barr virus (EBV) is suggested in some cases. A Japanese male presenting with recurrent nasopharyngeal problems developed fever, generalized lymphadenopathy, and hepatosplenomegaly. Separated cells from lymph nodes were shown to have a natural killer (NK) cell, CD2(+), CD3(−), CD16(+), CD56(+), HLA‐DR(+) phenotype. A progressive abnormality of hepatic function was associated with hepatorenal failure and death. A serologic study suggested reactivated EBV infection. In situ hybridization (ISH) studies showed Epstein‐Barr virus‐encoded RNA (EBER)‐1 in lymph nodes, with lymphocytes infiltrating the liver and tissue from ethmoid sinus surgery 3 years prior to development of obvious lymphoproliferative disease. Polymerase chain reaction performed on lymph node DNA, using oligonucleotide primers specific for the EBV lymphocyte‐determined membrane antigen (LYDMA) gene, revealed a single band, suggesting monoclonal proliferation of the tumor. NK activities of the lymphocytes from the lymph node and peripheral blood were markedly decreased. These findings suggest a close relationship between EBV infection and development of NK cell lymphoproliferative disorder. Am. J. Hematol. 54:314–320, 1997. © 1997

ISSN:0361-8609    

DOI:10.1002/(SICI)1096-8652(199704)54:4<314::AID-AJH10>3.0.CO;2-B

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

年代:1997

数据来源: WILEY