摘要:

AbstractPlasma thrombin‐antithrombin 111 complex (TAT), FDP‐D‐dimer, activated protein C (APC)‐protein C inhibitor (PCI) complex, and tissue type plasminogen activator (t‐PA), PA inhibitor‐1 (PAI‐I) were significantly increased in patients with acute myocardial infarction (AMI) at onset. These patients exhibited a hypercoagulable state and protein C activation at onset. The plasma PCI level at onset of AMI was within the normal range, but was significantly decreased after percutaneous transluminal coronary angioplasty (PTCA). After PTCA, plasma t‐PA, FDP‐D‐dimer, and plasmin‐a,‐plasmin inhibitor were increased but APC‐PCI complex and TAT were not. The decrease in PCI after PTCA may have been caused by the activation of fibrinolysis. PCI may play an important role in the inhibition of fibrinolysis in stimulated or damaged endothelial cells. These findings suggest that the protein C pathway plays an important role in the onset of AMI and after PTC

ISSN:0361-8609    

DOI:10.1002/ajh.2830490102

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

年代:1995

数据来源: WILEY

摘要:

AbstractThe expression of CD45 RA/RO antigen was investigated in neoplasms including cases expressing CD7 antigen as the sole pan‐T antigen (n = 8), T‐lineage acute lymphoblastic leukemia (ALL)/lymphoblastic lymphoma (LBL) at various stages of differentiation (n = 32), peripheral stage T‐lineage leukemia (n = 10) and adult T‐cell leukemia (ATL) (n = 14). The p56Ickgene expression was also investigated in selected cases.The expression pattern of CD45 RA/RO antigen was defined as of RA, mixed, or RO type. All but one CD7+ CD5− CD2− case were of the RA type. The CD7+ CD5+ CD2− prothy‐mic stage included seven RA and one mixed type cases. One CD7+ CD5− CD2+ case was of the RA type, but the other was of the RO type. The CD7+ CD5+ CD2+ prothymic stage included three RA and four mixed type cases. All seven CD3− CD4+ CD8+ (double‐positive) thymic cases were of the RO type. The CD3+ CD4+ CD8+ (triple‐positive) stage included two RO and three mixed‐type cases. One CD3+ CD4+ CD8− late thymic case was of the mixed type. The peripheral stage cases included five RA, three RO, and two mixed type cases. All ATL cases were of the RO type. The expression of p56Ickgene in the prothymic stage was less marked than that in the thymic stage.On the basis of these results, the following sequence of pattern of the CD45 RA/RO antigen expression along with T‐lineage differentiation was reconstructed: prothymic stage [RA and mixed type] → double‐positive thymic stage [RO type]→ triple‐positive thymic stage [RO and mixed type] → peripheral stage [RA, mixed, and RO type]. While one RO‐type CD7+ CD5− CD2− and one RO‐type CD7+ CD5− CD2+ cases were not in accord with this sequence, the pattern of CD45 RA/RO antigen expression in most of T‐lineage neoplasms could be determined by the respective stage of differentiation. The poor expression of the p56Ickgene by the prothymic blasts compared with the thymic blasts may be related to the expression pattern of the CD45 RA/RO molecules, which exhibits phosphatase activity. The consistent RO‐type expression in the ATL cases may reflect the activated status of the neoplastic T cells due to the presence of the HTLV‐I gene. Alternatively, the target cells for HTLV‐I‐induced neoplastic transformat

ISSN:0361-8609    

DOI:10.1002/ajh.2830490103

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

年代:1995

数据来源: WILEY

摘要:

AbstractChromosomal in situ hybridization (ISH) has extended the scope of cytogenetic analysis to nondividing cells by the use of chromosome‐specific probes detected by nonisotopic techniques. This provides a rapid and sensitive method for identifying chromosomes in interphase cells, and is useful in gauging engraftment following bone marrow transplantation, particularly when the number of cells obtained is minimal. We have performed ISH using a Y‐heterochromatin‐specific probe to monitor patients with malignant hematological disease who have received a sex‐mismatched transplant. The results have been compared with those obtained from concurrently performed standard cytogenetic analysis. Host cells were detected by interphase cytogenetics in all patients posttransplant, at times varying from 28‐1,825 days, whereas routine analysis detected host cells in only 4 patients, 3 of whom were found to be in relapse. The significance of the persistence of host cells is unknown, but it does not appear to indicate impending relapse. © 1995 Wiley

ISSN:0361-8609    

DOI:10.1002/ajh.2830490104

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

年代:1995

数据来源: WILEY

摘要:

AbstractA new variant of von Willebrand's disease has been discovered in 2 members of a Macedonian family of 6. The proband, an 8‐year‐old boy, showed a prolonged bleeding episode on 1 occasion. Ristocetin‐induced platelet aggregation and bleeding time were normal. In plasma, ristocetin cofactor activity (RCo) and von Willebrand factor (vWf) antigen were reduced to the same clearly low level. The determination of vWf antigen of platelets resulted in borderline values, while RCo was clearly reduced. Low‐ and intermediate‐resolution agarose gel electrophoresis showed absence of the largest multimers in plasma vWf, and slight reduction in platelet vWf. High‐resolution gels revealed abnormal multimeric structure only in plasma vWf. The smaller multimers could be resolved in a broad central band flanked by 4 fainter satellite bands; however, satellite bands close to the central band were more intense, and more distant ones were fainter, compared to normal plasma. The central band of the fastest‐moving multimer was markedly intensified, and the mobility of the whole quintuplet was slightly reduced. Heredity seems to be autosomal‐dominant. No mutation was found in exon 28 of the vWf gene.Because there was only 1 mild bleeding episode in the family, this structural variation seems to have only little clinical consequence. We conclude that this vWf abnormality is different from those observed in other type II variants previously described. Based on the revised classification by the International Society on Thrombosis and Haemostasis, we proposed designation type 2A‐Bern for this new subtype. © 19

ISSN:0361-8609    

DOI:10.1002/ajh.2830490105

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

年代:1995

数据来源: WILEY

摘要:

AbstractWe have reviewed the clinical, morphologic, immunophenotypic, and cytogenetic features of 52 patients with erythroleukemia (FAB Cooperative Group; AML‐M6) studied by the Cancer and Leukemia Group B (CALGB). The purpose of this study was to correlate morphology with the clinical features, immunophenotypes, and karyotypes of neoplastic cells, and with the response to therapy of patients with AML‐M6. Thirty‐three patients (63%) were male, median age 59 (range 16‐81) years, 47 patients (90%) were white, and 42 patients (81%) had a performance status of<2. Myelodysplastic changes were observed in at least 1 cell lineage in all cases, and in 2 cell lineages in 45 of 52 (86%) cases. Fifty percent or more of cases studied were positive for CD11b, CD13, CD15, CD33, glycophorin‐A, and HLA‐DR markers. Fourteen of 27 cases (52%) in whom karyotypic analyses were conducted had cytogenetic abnormalities. Five (19%) were simple (25% of all erythroblasts) and group 2 (38 patients with proerythroblasts and basophilic erythroblasts ≤25% of all erythroblasts). We observed no significant differences between groups 1 and 2 in regard to sex, age, race, performance status, percentage of blood erythroblasts or myeloblasts, percentage of bone marrow erythroblasts, and periodic acid‐Schiff (PAS) or myelodysplasia scores. Six of 6 (100%) patients of group 1, and 7 of 21 (33%) patients of group 2, had normal karyotypes (P = .006). Nine of 13 (69%) patients of group 1 and 15 of 33 (45%) patients of group 2 had a complete remission (CR) (P = .2). Eight of 11 (73%) cytogenetically normal patients achieved CR: 5 of 6 (83%) in group 1, and 3 of 5 (60%) in group 2. Five of 12 (42%) cytogenetically abnormal patients achieved CR. No difference in duration of survival (group 1, median = 4.6 months vs. group 2, median = 10.2 months; P = .93) was observed between the 2 groups. We conclude that AML‐M6 is typified by multilineage involvement of hematopoietic cells. The morphology of erythroblasts in patients with AML‐M6 may correlate with cytogenetic abnormalities and rate of CR.

ISSN:0361-8609    

DOI:10.1002/ajh.2830490106

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

年代:1995

数据来源: WILEY

摘要:

AbstractThirty‐nine Chinese patients presenting as essential thrombocythemia (ET) were analyzed retrospectively. The median age at presentation was 69 years and the M:F ratio was 1.35:1. At diagnosis, 33 cases (84%) were asymptomatic and the diagnosis was made incidentally, while 3 cases (8%) presented with small vessel, and 2 cases (5%) with large vessel, thrombosis. One patient (3%) presented with minor bleeding. The platelet count ranged from 0.9‐34 × 1012/1. Of 12 karyotypes done, 2 cases were abnormal, both showing the Philadelphia (Ph) chromosome. First‐line therapy was radiophosphorus (32P) in 3 cases, melphaian in 20 cases, and hydroxyurea in 12 cases; 4 cases did not receive specific therapy. During the follow‐up (mean = 4 years), no thrombotic or bleeding episodes were observed. One patient with the Ph chromosome underwent blastic transformation. These results indicate that bleeding and thrombosis occur significantly less in Chinese patients than in Western patients. The Ph chromosome appears to be a bad prognostic indicator. Because of the very low incidence of complications and good prognosis, the authors believe that cytoreductive therapy is best achieved by the use of hydroxyurea instead of alkylating agents or radiophorphours, as the latter agents are potentially leukemogenic. © 1995 Wiley

ISSN:0361-8609    

DOI:10.1002/ajh.2830490107

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

年代:1995

数据来源: WILEY

摘要:

AbstractA term infant presented with mild cyanosis without evidence of hypoxia. Cardiopulmonary disease, polycythemia, and methemoglobinemia were excluded. Standard hemoglobin electrophoresis, including isoelectric focusing, were normal. However, by reversephase C4HPLC, an abnormal globin chain was detected. Analysis of tryptic peptides and amino acid sequence showed that the patient had an amino acid substitution Phe→Ser at residue 41(C7) in theGγ chain. This was confirmed by DNA sequencing that demonstrated a point mutation at the expected site in exon 2 of theGγ gene, accounting for the appropriate change in the codon. This substitution, hemoglobin F‐Cincinnati, α2γ241(C7) Phe→Ser, not previously described, presumably decreased oxygen affinity of the hemoglobin. This substitution is very near the heme group and the α1β2interface and, hence, in a crucial area of the globin chain. Abnormalities of γ globin chains tend to be overlooked due to their transient presence and trivial clinical symptomatology, or due to “in utero” selection when physiologically abnormal. Mutant hemoglobins with altered oxygen affinity should be included in the differential diagnosis of newborns presenting with cyanosis, in whom all common causes have been excluded. © 199

ISSN:0361-8609    

DOI:10.1002/ajh.2830490108

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

年代:1995

数据来源: WILEY

摘要:

AbstractStandard intensive induction therapy is tolerated poorly by elderly patients with acute myeloblastic leukemia (AML). We treated 19 elderly patients with AML, including seven with a prior myelodysplastic syndrome (MDS) with a combination of low dose cytarabine, hydroxyurea, and GM‐CSF. The percentage of blasts in S‐phase was evaluated prior to and 24 hr after starting the GM‐CSF infusion. Cell cycle analysis was performed by flow cytometry using propidium iodine staining with fluorescein isothiocyanate‐conjugated monoclonal antibody to the myeloid antigen CD 33.Seven out of nineteen (37%) achieved a complete remission (CR) and six (31%) a partial remission (PR) for an overall response rate of 68% (13/19). There were three early deaths from infectious complications or organ filure. One patient died from disseminated fungal infection after attaining a PR. The medial overall survival was 9.5 months with a range of 1 to 23+ months. The projected median survival for the patients with de novo AML is greater than 23 months. The percentage of CD 33+ cells in S‐phase increased from a mean of 11.6+/‐2.7 (SEM) pre GM‐CSF to 19.0+/‐3.7 (SEM) post GM‐CSF (Pgrade 2 gastrointestinal toxicity. There was no neurologic or cardiac toxicity.These data suggest that the combination of hydroxyurea, GM‐CSF, and cytarabine is an effective remission‐induction regimen in elderly patients wit

ISSN:0361-8609    

DOI:10.1002/ajh.2830490109

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

年代:1995

数据来源: WILEY

摘要:

AbstractPrevious reports have alluded to variability in the aggregation response of normal human platelets to the neuropeptide arginine vasopressin (AVP). Since it has not been well documented, the current studies were undertaken to characterize this response. AVP (1‐100 nM) produced a concentration‐dependent aggregation response. Although the aggregation response to 100 nM AVP did not correlate with age or sex, there was a bimodal response distribution based on the presence or absence of a second wave of aggregation. In kinetic studies, the apparent km of AVP was 18.3 ± 5.4 nM. There was a significant inverse relationship between the maximal aggregation response to 100 nM AVP and the km (r = −0.82). One hundred nanomolar AVP increased the intracellular calcium concentration of platelets by 406 ± 120 nM in calcium free buffer and by 658 ± 233 nM in the presence of 1.0 nM CaCl2. The aggregation response to 100 nM AVP correlated most strongly with the transmembrane influx of calcium (r = 0.84). In individuals whom 100 nM AVP was able to generate a second wave of aggregation, the selective protein kinase C inhibitor bis‐indolylmaleimide significantly decreased the platelet aggregation response. Thus, there is significant heterogeneity in the aggregation response of normal human platelets to AVP. Based on our kinetic studies and the effects of PKC inhibition on the aggregation response to AVP, we would hypothesize that the variability of the aggregation response of normal human platelets to AVP is related to a polymorphism of the platelet AVP V1receptor. © 1995 Wlle

ISSN:0361-8609    

DOI:10.1002/ajh.2830490110

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

年代:1995

数据来源: WILEY

摘要:

AbstractThis study reports on the biological data of ten patients with acute venous thromboembolism. They were treated for 5 days with continuous intravenous infusion of a fixed dose (0.05 mg/kg/hr) of a recombinant hirudin (r‐H HBW 023 Behringwerke, Germany). The plasma level of r‐H (HBW 023), assessed by an anti‐factor Ila amidolytic activity, was stable after Day 2 and showed considerable individual variations. It correlated with APTT ratio, suggesting that this test is a reliable tool to monitor therapy. In contrast, thrombin time was constantly over 120 sec (control 15 sec) and consequently was not a useful parameter. Prothrombin time showed a slight, but significant, prolongation, which was correlated with the increase of APTT ratio. There was no bleeding time prolongation, platelet count, or ATIII level decrease. Levels of thrombin‐antithrombin III complexes, and D‐dimers, which were high in all patients on admission, decreased during the course of the treatment but remained abnormal on Day 5, showing an ongoing hemostasis and fibrinolysis activation: this is consistent with the delayed, but only slightly decreased thrombin generation evidenced by thrombin generation test performed on Day 3. These results suggest that thrombin inhibition by rH‐hirudin at this dosage is only partial, which allows the generation of traces of thrombin needed for the feed‐back thrombin production generated by factor V and VIII activation. © 1995 W

ISSN:0361-8609    

DOI:10.1002/ajh.2830490111

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

年代:1995

数据来源: WILEY