摘要:

AbstractThe clinical and hematological features of 202 Sicilian subjects with sickle cell disease are reported, 41 being homozygous for betaS(βSβS), 64 with betasthal betaS(βsβS), and 97 beta+thal betaS(β+βS). Analysis of the findings showed that the disease observed in Sicilians is of intermediate severity and falls between the severe form observed in patients of African origin and the milder one seen in subjects of Arabian o

ISSN:0361-8609    

DOI:10.1002/ajh.2830330202

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

年代:1990

数据来源: WILEY

摘要:

AbstractAn alternating VCAD‐VAD regimen, combining vincristine‐doxorubicin by continuous infusion with cyclophosphamide and pulse dexamethasone, or VAD alone, was given to 175 previously untreated patients with multiple myeloma. The response rate with primary VAD‐based regimens of 55% was virtually identical to the 54% in comparable patients treated previously with similar programs by using bolus vincristine‐doxorubicin. Despite responses to VAD that were more rapid in onset than any previous treatment, remission and survival times were similar. This may be due to major differences in drug sensitivity between progenitor and differentiated plasma cells. A VAD‐based regimen seems better for newly diagnosed patients when rapid control of multiple myeloma is

ISSN:0361-8609    

DOI:10.1002/ajh.2830330203

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

年代:1990

数据来源: WILEY

摘要:

AbstractIn order to assess the thrombin and plasmin generation in vivo in disseminated intravascular coagulation (DIC), plasma levels of thrombin‐antithrombin III (ATIII) complex (TAT) and plasmin‐alpha 2‐antiplasmin (a2AP) complex (PAP) were measured together with standard coagulation and fibrinolytic parameters in 80 patients with DIC. Both TAT and PAP were markedly elevated in patients with DIC. When plotted by the underlying disease categories, differences in the magnitude of the elevations of these complexes were recognized among groups. Patients with acute promyelocytic leukemia (APL) had the highest PAP, the lowest TAT/PAP ratio, low a2AP, and low fibrinogen, indicating that the most excessive fibrinolysis can occur in APL. Similar profiles, although less marked, were observed in patients with other leukemias and vascular diseases. Patients with sepsis showed the highest TAT/PAP ratio and the lowest PAP with no decrease in a2AP or fibrinogen, demonstrating a relatively impaired fibrinolysis. Patients with cancer had a relatively high TAT and high TAT/PAP ratio. In addition, both TAT and PAP were markedly elevated in patients with shock. From these, it was suggested that, although laboratory manifestations in DIC are extremely variable from patient to patient, underlying disorders are, at least in part, responsible for the observed variations. Recognition of this variable activation of coagulation and fibrinolysis would be helpful for the proper management of patients wit

ISSN:0361-8609    

DOI:10.1002/ajh.2830330204

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

年代:1990

数据来源: WILEY

摘要:

AbstractElevation of the cytoplasmic Ca2+concentration ([Ca2 +]i) by epinephrine and epinephrine‐induced inhibition of prostaglandin E1(PGE1)‐stimulated cyclic adenosine monophosphate (CAMP) accumulation were assessed in platelets from three groups of subjects: normal controls (NS, n = 11) and patients with myeloproliferative disorders whose platelets were either sensitive (ES, n = 9) or specifically insensitive (El, n = 7) to the aggregatory effect of epinephrine. The inhibition by epinephrine of CAMP accumulation in the platelets exposed to 500 nM PGE, was not significantly different between the three groups. Therefore, despite the detective aggregation response to epinephrine, platelets from the El group seemed to retain normal response, which was attained through cα2‐adrenergic receptors, guanine nucleotide binding regulatory protein, and the adenylate cyclase system. However, in aequorin‐loaded, washed platelets, the epinephrine‐stimu‐lated rise in [Ca2+]i showed significant decrease in the El group compared with the other groups (P<0.01). Thus the mechanism for the impaired aggregation response to epinephrine in platelets from the El group could include the defect that exists in the pathway from receptor binding of epinephrine to the aggregation response through [Ca2+

ISSN:0361-8609    

DOI:10.1002/ajh.2830330205

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

年代:1990

数据来源: WILEY

摘要:

AbstractDual‐parameter flow cytometric analysis of B‐cell antigens and DNA content was used to determine the phenotypes of proliferating tumor cells (S‐phase cells) from 30 patients with multiple myeloma. B4 (CD19), J5 (CALLA, CD10), B1 (CD20), and monotypic surface immunoglobulin (Sig) were expressed heterogeneously in 24 patients. J5 and monotypic Slg were found most frequently but were always expressed on a significantly lower percentage of cells than the antigens typically associated with plasma cells, cytoplasmic immunoglobulin (Clg) and T10 (CD38). S‐phase cells were found in each antigen(+) subset. B antigen(+) cycling cells were demonstrated in 16 patients whose marrow or blood cells expressed B antigens exclusively in the hyperdiploid fraction and therefore were certainly part of the myeloma clone. Similar to the low level of proliferative activity of the T10(+), Clg(+), and PCA1(+) subsets, the percentages of cycling cells of the preplasma cell B‐antigen‐bearing myeloma subsets ranged from<1% to 12%. The tumor cells of four patients were also studied with dual‐color surface antigen analysis and demonstrated independent expression of B antigens, with only rare coexpression of T10 and monotypic Slg, J5, or B4. These findings are consistent with the presence of distinct myeloma subsets bearing differing B phenotypes in the same tumor and provide evidence that the proliferation in myeloma is occurring at various developmental stages in the malignant B lineage. These antigens may be important targets for immunologic therapy aimed at eliminating the entire proliferating compartment of this

ISSN:0361-8609    

DOI:10.1002/ajh.2830330206

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

年代:1990

数据来源: WILEY

摘要:

AbstractTo identify prognostic factors for patients with acute myeloid leukemia in first relapse we have analyzed the courses of 57 patients undergoing intensive reinduction therapy. The median duration of first remission was 9 months. A second complete remission was achieved by 31 patients (54%). The median duration of second remission was 5 months (range 1‐142 + months). Two patients are still disease‐free with 42 months and 142 months follow‐up. Clinical, laboratory, and treatment variables at the time of original diagnosis and at relapse were examined for possible associations with the outcome of reinduction chemotherapy. Only duration of first remission consistently correlated with ability to achieve a second remission, second remission duration, and overall survival from reinduction. The second complete remission rate was 69% for those with a first remission longer than 9 months but only 39% for those with a first remission less than 9 months (P<0.05). The patients with longer first remissions also had longer second remissions (8 months median vs. 4 months,P= 0.02) and a longer median survival following reinduction chemotherapy (8 months vs. 5 months,P= 0.02). We conclude that the duration of a patient's first remission should be considered a useful prognostic factor both in treatment planning and in the interpretation of clinical trials of patients in re

ISSN:0361-8609    

DOI:10.1002/ajh.2830330207

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

年代:1990

数据来源: WILEY

摘要:

AbstractSix patients, five with acute myeloid leukemia (AML) and one with a myelodysplastic syndrome (MDS), were found to have monosomy 7 by conventional cytogenetics at diagnosis. Repetitive DNA sequences from the heterochromatic region of human chromosomes 1 and 7 were used as probes for in situ hybridization experiments on interphase cells of these patients. A double hybridization protocol was used to reveal the particular chromosomes as distinct spots or clusters of signals within interphase nuclei. The chromosome 1 sequence served as an internal control. Simultaneous detection of the sequences showed the presence of two normal number 1 chromosomes and a missing 7 chromosome from individual cells. While cytogenetic preparations showed only −7 metaphases in 3 AML and 1 MDS patients, in situ hybridization of interphase cells showed many normal cells as well as the presence of ‐7 in fully mature granulocytes. One AML patient studied in remission showed only normal metaphases yet had 9% interphase cells with a missing 7 and relapsed within 3 months. We conclude that examination of interphase cells by in situ hybridization provides clinically useful data since every cell including mature granulocytes can be examined, the lineage of a cell can be determined, and efficacy of differentiation therapy can be evalua

ISSN:0361-8609    

DOI:10.1002/ajh.2830330208

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

年代:1990

数据来源: WILEY

摘要:

AbstractThe clinical and hematological parameters of a patient described here, who inherited the genes of both hereditary elliptocytosis (HE) and β‐thalassemia, seem to reflect a mutual enhancement of the two diseases. The coexistence of the two pathologies is probably also responsible for the observed changes in spectrin: the appearance of an extra spectrin band between tetramers and dimers on denaturing gel electrophoresis and the metabolic‐dependent reduction in spectrin amount. It is assumed that the instability of the skeletal network that results from the HE pathology caused increased exposure of the spectrin molecule to oxidative damage that usually occurs in thalassernic red cells. The products of such oxidation may have led to abnormal spectrin associations which finally resulted in the above cha

ISSN:0361-8609    

DOI:10.1002/ajh.2830330209

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

年代:1990

数据来源: WILEY

摘要:

AbstractThe objective of the study was to explore the risks and benefits of splenectomy in advanced agnogenic myelod metaplasia (AMM). We searched the literature (Medline, 1970‐1987) for studies of postoperative survival, operative mortality and effects of splenectomy on painful splenomegaly, and portal hypertension or transfusion requirements in patients with AMM. We employed formal decision analysis to determine the relative value of medical and surgical treatment of advanced AMM.Results of data synthesis showed that splenectomy in AMM is associated with an operative mortality of 13.4% (95% confidence intervals (Cl): 9.5‐1 ‐17.2%), an early morbidity of 45.3% (Cl: 39.6‐51.1%), and a late morbidity of 16.3% (Cl: 9.9‐22.5%). Almost all patients with portal hypertension and painful splenomegaly, but only about half of those with thrombopenia and anemia were reported to have experienced relief in their symptoms or signs after splenectomy. We found no evidence that splenectomy affects survival in AMM.We concluded that splenectomy in advanced AMM is a palliative procedure that carries a substantial risk. It may be considered for symptomatic patients after they have been informed about the operative mortality, morbidity, and chances of palliation. Decisions about treatment of advanced AMM should be guided predominantly by the patient's pr

ISSN:0361-8609    

DOI:10.1002/ajh.2830330210

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

年代:1990

数据来源: WILEY

摘要:

AbstractA 38‐year‐old man with severe factor IX and mild factor VIII deficiencies complicated by cirrhosis secondary to chronic non‐A non‐B hepatitis underwent orthotopic liver transplantation as treatment for both the cirrhosis and his congenital coagulopathy. Intraoperative hemostasis was obtained with factor VII‐depleted prothrombin complex concentrate and fresh frozen plasma. Factor VIII and factor IX levels were assayed frequently in the perioperative period, and both returned to normal within 24 hr and remained normal postoperatively. Liver transplantation can be considered as definitive therapy for hemophilia A and/or B with transfusion‐related li

ISSN:0361-8609    

DOI:10.1002/ajh.2830330211

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

年代:1990

数据来源: WILEY