摘要:

AbstractBetween January 1985 and July 1989 we diagnosed asymptomatic stage I multiple myeloma according to Durie and Salmon [Durie and Salmon: Cancer 36:842, 1975] in 91 patients. All patients were followed without chemotherapy. Disease progression occurred in 41 patients and the median time to progression for all patients was 48 months. In the Cox multivariate regression analysis, hemoglobin levels<12 g/dl (P25% (P<.01), and M‐component size ≥30 g/l for lg G or 3 = 25 g/l for lg A (P<.01) were the only significant prognostic factors for progression. The 38 patients without any harmful factor remained free of progression for a median of more than 50 months. The 18 patients with two or three of these characteristics (high‐risk group) had the shortest median time to progression of 6 months. Despite different times to progression, the response rate and survival after chemotherapy were similar for all groups of patients. Patients in the high‐risk group for progression have to be frequently monitored for disease progression and might benefit from early tr

ISSN:0361-8609    

DOI:10.1002/ajh.2830480201

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

年代:1995

数据来源: WILEY

摘要:

AbstractThe granulocyte colony‐stimulating factor (G‐CSF) has been shown to accelerate recovery from severe neutropenia and to decrease the incidence of documented infections after intensive chemotherapy in cancer patients. However, the routine prophylactic use of G‐CSF is expensive. This study was conducted to determine the role of G‐CSF as adjunct therapy for septicemia following neutropenia caused by chemotherapy in children with acute leukemia. Fifty consecutive episodes of septicemia were studied involving 34 episodes of Gram‐negative, 7 episodes of Gram‐positive, 5 episodes of polymicrobial bacterial septicemia, one episode of fungemia, and 3 episodes of disseminated fungal infection. In the first 25 episodes, G‐CSF was not used (group A). For the next 16 episodes, G‐CSF 200 μg per square meter per day subcutaneously was given immediately after the septicemia was documented until the absolute neutrophil count was maintained at more than 1,500 per cubic millimeter (group B). Thereafter, G‐CSF at the same dose as that of group B was prophylactically used in all the children who received high‐dose cytosine arablnc‐side‐containing regimens. Nine episodes of septicemia occurred (group C). The incidences of mortality per episode of septicemia in groups A, B, and C were 12.0% (3/25), 12.5% (2/16) and 0% (0/9), respectively. Statistically, there was no difference between the three groups overall and in pair‐wise comparisons (allP>0.5). The durations of G‐CSF administration in group B ranged from 6 to 26 days with a median of 12 days and the durations of G‐CSF administration in group C ranged from 10 to 23 days with a median of 19 days. With or without G‐CSF, there may be no significant difference in the mortality of septicemia following neutropenia caused by chemotherapy

ISSN:0361-8609    

DOI:10.1002/ajh.2830480202

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

年代:1995

数据来源: WILEY

摘要:

AbstractIn order to verity the genetic factors influencing the clinical expression of β‐thalassemla we have studied 292 Kalian patients, 165 with thalassemia intermedia and 127 with thalassemia major. The β‐globin gene mutations were defined in all cases. The number of α‐globin genes and the integrity of specific control regions of the β‐globin cluster—γ promoters and β‐Locus Control Region (β‐LCR)—were studied in selected cases.Homozygosity for mild mutations (group I) accounts for 24% of the intermedia patients and it is not represented among major patients. Forty‐four percent of intermedia patients had combinations of mild/severe (group II) mutations and 32% had homozygosity or double heterozygosity for severe mutations (group III). Seventy‐six percent of patients with thalassemia major were classified in group III end 24% in group II.Deletion type —α3.7thalassemia, assessed in a part of the cases, was found in 5% of thalassemia major and 19.5% of Intermedia patients in groups II and III. Structural analysis of γ promoters and β‐LCR HS2 and HS4 regions, carried out in order to look for alterations associated with Hb F increase, did not reveal new mutations. Only rare polymorphic changes were observed at the HS2 and HS4 level. The − 158 γ C T change was found with an increased incidence in intermedia patients in groups II and III.A subset of 10 β‐thalassemia heterozygotes with mild intermedia phenotype resulted from coinheritance of a triplicated α‐locus. We have been unable to find a molecular basis for the benign clinical course in approximately 20% of patients with thalassemia intermedia. Other genetic or acquired factors must be hypothesiz

ISSN:0361-8609    

DOI:10.1002/ajh.2830480203

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

年代:1995

数据来源: WILEY

摘要:

AbstractMonosomy 7 is a non‐random cytogenetic abnormality that is frequently associated with myelodysplastic syndromes (MDS). Twenty‐four bone marrow samples from five pediatric patients with MDS were analysed using both traditional and Interphase cytogenetic analysis. The majority of the metaphases were monosomic for chromosome 7 while interphase cytogenetic analysis consistently detected a disomic cell population in nondividing cell populations. This suggests that the monosomy 7 cells have a distinct proliferative advantage compared to the disomic cell population. The results demonstrate that interphase cytogenetic analysis provides important cytogenetic information about non‐dividing cell subpopulations, enhancing our understanding of the cell dynamics of normal and monosomy 7 cells i

ISSN:0361-8609    

DOI:10.1002/ajh.2830480204

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

年代:1995

数据来源: WILEY

摘要:

AbstractThe in vitro effect of IL‐6 on platelet activation was investigated. When human platelets were incubated with high (1,000 ng/ml) or low (1 ng/ml) dose IL‐6, expression of GMP‐140 was enhanced by 42% (N = 6;P<0.009) and 46% (N = 6;P<0.061) in 1 hr low and high dose IL‐6‐platelet incubations, respectively, as assessed by flow cytometry. In platelet specimens incubated with high dose IL‐6 for 3 hr, a 70% (N = 6;P<0.009) increase in GMP‐140 expression over control was observed. Parallel high dose IL‐6 incubations subjected to scanning electron microscopic studies revealed a 3.4‐fold increase (N = 6;P<0.001) in spheroid morphologic platelet forms in 1 hr incubations in comparison to control platelet preparations, whereas in 3 hr IL‐6‐platelet incubations, a 96% uncrease in dendritic platelet forms was observed (N = 6;P<0.001). Significant increases in platelet ATP levels were observed in both 1 min and 1 hr high dose and low dose IL‐6 platelet incubations. In 3 hr high dose‐IL‐6 platelet incubations, a significant 18% (N = 8;P<0.001) decrease in platelet ATP was parallelled by a significant 40% increase (N = 8;P<0.014) in plasma ATP in the same specimens. This increased plasma ATP was highly correlated with a reduction in platelet ATP when analyzed by bivarlate regression analysis. Lastly, transmission electron microscopic analysis demonstrated a significant reduction in dense granule number and ratio of dense granule surface area/cell surface area in 3 hr high dose IL‐6 incubations. These findings suggests that IL

ISSN:0361-8609    

DOI:10.1002/ajh.2830480205

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

年代:1995

数据来源: WILEY

摘要:

AbstractWe studied 21 filgrastim (G‐CSF)‐mobilized peripheral blood stem cells (PBSC) apheresis products obtained from seven patients, and stored at 4°C for periods of up to 96 hr prior to their reinfusion, to rescue high‐dose chemotherapy. The apheresis products contained a median of 106 × 10/L mononuclear cells (MNC), 14.6% of them displaying the CD34 antigen; the viability was over 90% in all samples studied at 24, 48, and 72 hr after harvesting. These PBSC were successfully used to rescue high‐dose chemotherapy; patients received a median of 4.8 × 10/Kg MNC; the median time to achieve>500 granulocytes was 14 days (range 11–26) and the median time to achieve>20,000 platelets was 20 days (range 11–40). Since autologous transplants with nonfrozen PBSC are feasible and less costly than those using frozen PBSC, restrictions to PBSC autotransplant programs may be overcome and costs may

ISSN:0361-8609    

DOI:10.1002/ajh.2830480206

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

年代:1995

数据来源: WILEY

摘要:

AbstractThe majority of children with idiopathic thrombocytopenia (ITP) have an acute self‐limiting course and no diagnostic test has been identified which will predict the course of thrombocytopenia and detect those with the chronic autoimmune form. The detection of autoantibodies directed against the platelet glycoprotein complex llB/llla, may identify patients with chronic ITP. Serum anti‐GP llb/llla antibodies were assessed by the indirect MAIPA assay in 54 children with immune thrompocytopenla at initial presentation along with an additional 7 children previously diagnosed with chronic ITP, to determine if there was a difference in antibody positivity between acute and chronic ITP patients, and whether the identification of antibodies could be used as a predictive test at diagnosis. There was no significant difference in the percentage of antibodies detected in children classified with acute ITP (27/40–68%) compared to children with chronic ITP (13/21‐62%,P>0.05). Patients with acute ITP had significantly lower mean platelet counts at diagnosis compared to the chronic ITP group (16,225/mm3vs. 32,250/mm3,P<0.05), though there was no significant difference in the bleeding manifestations between the acute and chronic ITP groups. Serum anti‐GP llb/llla antibodies are detected in a high percentage of children with ITP and autoantibodies appear to be involved in the pathogenesis of both acute and chronic ITP. The detection of anti‐GP llb/llla antibodies at diagnosis, however, does not appear to be a useful prognostic test in childhood ITP. © 1995 Wil

ISSN:0361-8609    

DOI:10.1002/ajh.2830480207

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

年代:1995

数据来源: WILEY

摘要:

AbstractTo assess the effect of ABO‐identical, filtration leukodepleted transfusions on resource consumption and costs of care we performed a cohort study in consecutive adult patients admitted for induction therapy of acute myeloid or lymphoid leukemia during 1985–92 (n = 120) and consecutive adult patients admitted for autologous bone marrow transplantation for Hodgkin's or non‐Hodgkin's lymphoma during 1989–1991 in our university hospital. Patients with acute leukemia received either ABO unmatched, unfiltered transfusions (1985–89), ABO identical, unfiltered transfusions (1987–90), or ABO identical, filtered transfusions (1990–92). Patients with lymphoma received either ABO unmatched, unfiltered transfusions (1989–90) or ABO identical, filtered transfusions (1990–91). Mean platelet transfusion requirements per patient decreased with ABO identical platelets and filtered transfusions: from 143 to 71 units in the transplant setting; from 146 to 83 in acute leukemia (P<0.05). Mean hospital ancillary service charges in 1992 dollars decreased with ABO identical platelets and filtered transfusions approximately $14,000 per patient for acute leukemia and $26,000 for for lymphoma. Per patient actual costs for filters ($643 in transplantation for lymphoma and $875 in leukemia) were offset by savings in actual blood component purchase costs alone ($4,127 in lymphoma and $3,283 in leukemia). In our setting introduction of ABO identical platelets and filtration leukodepletion were implemented with substantial decreases in costs. © 19

ISSN:0361-8609    

DOI:10.1002/ajh.2830480208

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

年代:1995

数据来源: WILEY

摘要:

AbstractA case of adult T‐cell leukemia/lymphoma (ATL) with two different clones in the peripheral blood and lymph nodes is reported here. When cellular DNA from the lymph node was digested withEcoRI, one band larger than 9 Kb was detected. Digestion of the cellular DNA withPstIresulted in one clear band in addition to three internal fragments. In contrast, when cellular DNA from malignant peripheral blood lymphocytes (PBL) was digested with the same endonucleases, distinct bands at positions different from those observed in the lymph node were detected, indicating two separate malignant clones in the patient. Monoclonality of the tumor cells was shown by T‐cell receptor‐β (TCR‐β) gene rearrangement in both PBL and lymph node. Furthermore, there was a difference in the surface phenotype between tumor cells taken from peripheral blood (CD4+, CD8–) and lymph node (CD4+, CD8+). These findings suggest the presence of two different ATL clones in PBL and lymph node in a single patient simultaneously, which is distinguishable by the integration pattern of human T‐cell leukemia virus type I (HTLV‐

ISSN:0361-8609    

DOI:10.1002/ajh.2830480209

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

年代:1995

数据来源: WILEY

摘要:

AbstractWe have recently described an unusual situation which involved a combination of a factor IX and a protein C deficiency in a young child who presented, according to the bleeding tendency, as a hemophilia B patient in this particular hemophiliac, baseline prothrombin fragment F1+2levels were unexpectedly elevated and increased after an injection of a very high purity factor IX concentrate. This observation raised a question regarding the substitution schedule in the case of repeated injections of factor IX, since the thrombotic tendency has been a major concern with some factor IX concentrates. We monitored factor IX, prothrombin fragment F1+2and D‐dimer plasma levels before and during the 6 hr following the injection of an immunopurified factor IX concentrate. The results showed an increase in the F1+2levels after the factor IX injection, but an increase lower than previously observed with an ion‐exchange chromatography‐purified concentrate. Furthermore the F1+2level returned to baseline value 6 hr after administration. This factor IX concentrate seems to be best for use in the patient where repeated injections are involved (as employed during surgery). Moreover, the data point out the advantage of a monoclonal antibody‐purified factor IX concentrate over less purified concentrates in a specific situation, with regard to the thrombogen

ISSN:0361-8609    

DOI:10.1002/ajh.2830480210

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

年代:1995

数据来源: WILEY