摘要:

AbstractThe coagulant content and thrombin generating potential of synovial fluid from patients with osteoarthritis were studied as a model of extravascular coagulation. The concentrations of individual coagulant proteins were partially correlated with their molecular weight. The levels of the very large coagulants factor V, factor VIII and von Willebrand factor antigen (vWF:ag) are less than 1% of the activities found in a normal pooled reference plasma while smaller coagulants including factors IX, XI and prothrombin range between 9 and 30%. The protease inhibitors antithrombin‐III (AT‐III) and Alpha‐2 macroglobulin in synovial fluid were present at levels of 74% and 13% of plasma, higher than expected based on their molecular weights. Prothrombin was more rapidly activated by tissue thromboplastin than by aPTT reagent. The thrombin activity formed in synovial fluid decreased more rapidly than that formed in dilute plasma. The addition of recombinant factor VIII or bovine factor V to synovial fluid accelerated the thrombin production by APTT but not by tissue thromboplastin. Indicating that the low levels of factor VIII and factor V did limit the rate of thrombin production. The addition of specific antibodies to factor VIII or factor V strongly inhibited thrombin production by aPTT.These data confirm a roughly inverse relationship between the concentrations of coagulation proteins and their molecular weight in synovial fluid and indicate that thrombin can be generated in synovial fluid. The inactivation of thrombin in synovial fluid may be more dependent on antithrombin‐III than in plasma because of the increased AT‐III/alpha‐2 macroglobulin ratio seen in syn

ISSN:0361-8609    

DOI:10.1002/ajh.2830500202

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

年代:1995

数据来源: WILEY

摘要:

AbstractThrombotic thrombocytopenic purpura (TTP) is a rare disease and the epidemiologic features have been incompletely characterized. Because of the historically high case‐fatality rate for TTP, we analyzed U.S. multiple cause‐of‐death mortality data with TTP listed on the death record for the period 1968–1991, in order to estimate the incidence of TTP, to characterize demographic features of the decedents, and to determine if trends in mortality correlate with findings from clinical studies showing improved survival in recent years.There were 4,523 TTP‐associated deaths during the 24‐year study period. The annual age‐adjusted mortality rate decreased initially and reached its lowest point at 0.4 per 1,000,000 residents for the years 1970 through 1973, and then increased steadily to 1.1 during the last 4 years of the study period, 1988 through 1991. We estimate the current incidence of TTP to be approximately 3.7 cases per 1,000,000 residents. Deaths were rare below the age of 20 years, but the age‐specific mortality rate for those 20 years and older increased steadily with increasing age. Regardless of age, females were affected more often than males, and the overall female‐to‐male age‐adjusted rate ratio was 1.9 (95% confidence interval (Cl), 1.8 to 2.0). The greatest age‐specific difference was between females and males in their twenties (rate ratio 3.2; 95% Cl, 2.6 to 3.9). The mortality rate for blacks, and especially black females, was higher than the mortality rate for whites (black‐to‐white age‐adjusted rate ratio 3.4; 95% Cl, 3.2 to 3.6; black female‐to‐white female age‐adjusted rate ratio 3.6; 95% Cl, 3.3 to 3.9), although the majority of deaths were among whites (71.5%). Infection with the human immunodeficiency virus (HIV) or an HIV‐related diagnosis was reported in 61 (1.3%) decedents overall and in 51 (4.4%) decedents from 1988 through 1991.The TTP mortality rate has increased over time despite reports of significant improvement in survival associated with clinical use of plasma infusion and plasma exchange. This trend in mortality suggests that the incidence of TTP is increasing. Blacks, and black females in particular, are affected at a disproportionately high rate. The increased incidence of HIV infection and related disease may have contributed to some of the increase in TTP mortality in recent years, but it does not explain the majority of the increase, which began

ISSN:0361-8609    

DOI:10.1002/ajh.2830500203

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

年代:1995

数据来源: WILEY

摘要:

AbstractWe investigated the deformability of sickle (SS) cells from 25 patients and mixtures of these SS cells with blood type‐compatible normal (AA) cells, using a nickel mesh filtration system, with the aim of determining optimal goals for exchange therapy. We found that for air‐equilibrated SS/AA cell mixtures the fraction of dense cells (MCHC>37 g/dl) is the determinant factor in filterability and that the dense cells contribute in a linear fashion to the loss of filtration up to 15% dense cells (y = ‐4.41 × + 98.23, r = 0.945,P<0.0001). The slope of this effect is approximately 25 times steeper than that of the relationship between filtration and percent nondense (MCHC<37/g/dl) SS cells (y = ‐0.17x +106.53, r = 0.772,P<0.0001). A comparison of the proportion of high fluorescence reticulocytes to total reticulocytes (HFR ratio), indicating an elevation of immature reticulocytes, between six nontransfused patients and six exchange‐transfused patients showed significant higher values in the nontransfused individuals (0.154 ± 0.051 versus 0.070 ± 0.054,P<0.003). These results may have implications regarding targets for exchange transfusion therapy. Further studies of the effect on transfusion, both simple and exchange, on the numbers of dense cells and the proportions and populations of reticulocytes and the rheological characteristics of the erythrocyte subpopulations see

ISSN:0361-8609    

DOI:10.1002/ajh.2830500204

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

年代:1995

数据来源: WILEY

摘要:

AbstractThe concentrations of interleukin‐5 (IL‐5), granulocyte‐macrophage colony‐stimulating factor GM‐CSF, and interleukin‐3 (IL‐3) in serum and in IL‐2‐stimulated lymphocyte culture medium (L‐IL2‐CM) prepared from patients with reactive eosinophilia were measured by enzyme‐linked immunosorbent assay (ELISA). Serum IL‐5 levels were increased in 16 out of 42 cases. GM‐CSF and IL‐3 were below the detectable levels in all sera examined. The concentrations of IL‐5 and GM‐CSF in L‐IL2‐CM were increased in 10 out of 29 patients. IL‐3 was belo

ISSN:0361-8609    

DOI:10.1002/ajh.2830500205

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

年代:1995

数据来源: WILEY

摘要:

AbstractIn the past 10 years, we have observed 112 cases of EDTA‐dependent pseudothrombocytopenia (PTCP) due to in vitro platelet clumping at room temperature. 93 patients had antiplatelet antibodies (48 IgM, 30 IgG, 3 IgA, and 12 had two different isotypes concomitantly). In 20% of patients, the presence of IgM antibodies characteristically accompanied platelet agglutination also at 37°C, and in citrated blood. The phenomenon was not age or sex related, nor was it associated with any particular pathology or use of specific drugs, and was present in both healthy subjects and patients with various diseases.Flow cytofluorimetric analysis of CD5‐positive B cells, which are responsible for autoantibody production, did not demonstrate any changes in the percentage and absolute number of this lymphocyte subset.Average follow‐up was 5 years (6 months‐10 years); however, previous clinical records disclosed that PTCP was present for more than 15 years in four cases, and more than 20 years in three others, with no clinical manifestation of disease.This study confirms that EDTA‐dependent PTCP is a phenomenon related to the presence of natural autoantibodies with antiplatelet activity, devoid of pathological significance. Its clinical interest resides in the need for its prompt and certain recognition in order to avoid unnecessary examinations and therapeutic interventions.The best and most rapid technique for obtaining accurate platelet counts in PTCP subjects is to collect and examine EDTA blood at 37°C; however, clumping will still be present in about 20% of these cases, and even in citrated blood. To obviate this phenomenon, blood should be collected in ammonium oxalate, and platelets counted in a Bur

ISSN:0361-8609    

DOI:10.1002/ajh.2830500206

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

年代:1995

数据来源: WILEY

摘要:

AbstractWe report three patients with pulmonary disorders associated with myelodysplastic syndromes (MDS). All three patients had symptoms of pyrexia and respiratory discomfort. One patient had pulmonary eosinophilia with bilateral pleural effusion, one had interstitial pneumonia, and one had bilateral pleural effusion caused by systemic vasculitis. Elevated C‐reactive protein (CRP) levels, polyclonal hypergammaglobulinemia, and morphological abnormalities in peripheral blood were observed in all three patients. The bone marrow of these patients revealed trilineage dysplasia and eosinophilia. Cytogenetic analysis showed [46,XY,‐7,+der(1q;7p)]. Antibiotic treatment was not effective. However, improvement was dramatic after corticosteroid treatment; CRP levels were reduced and the hypergammaglobulinemia was improved. These cases suggest that MDS with [‐7,+der(1q;7p)]may be correlated with bone marrow eosinophilia and that an immunologic abnormality may be involved in the pulmonary diso

ISSN:0361-8609    

DOI:10.1002/ajh.2830500207

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

年代:1995

数据来源: WILEY

摘要:

AbstractLong‐term survival following chemotherapy or autologous bone marrow transplantation in adults with relapsed/refractory non‐Hodgkin's lymphoma was evaluated. English language articles published from January 1, 1988 to September 1, 1993 were obtained from a broad‐based MEDLINE search retrieving 3,854 citations regarding therapy for lymphomas.Citations were evaluated using both computer‐based evaluation and manual review. Articles were included if they addressed the disease of interest (non‐Hodgkin's lymphoma, Working Formulation D‐H), the population of interest (adults with either relapsed or refractory disease), and the therapies of interest (chemotherapy or autologous bone marrow transplantation). Articles were excluded if they did not provide convincing information on long‐term survival (as evidenced by either survival analysis or individual patient data) or if they reported a small number of patients (N<15).No randomized trials of the two therapies were found. Nine case series were found reporting on 444 eligible patients receiving chemotherapy; eight were found reporting on 256 patients undergoing autologous marrow transplantation. After weighting by sample size, the mean 3‐year survival rate was 25% (95% Cl, 20–30%) following chemotherapy and 40% (95% Cl, 33–47%) following marrow transplantation. The reporting of potentially relevant prognostic factors was inconsistent among articles.Despite our comprehensive synthesis and evaluation of currently available data, the survival advantage of marrow transplantation in relapsed/refractory non‐Hodgkin's lymphoma that we report must be viewed as tentative, given the limitations of the case series data. In addition, establishing the comparability of patients treated with these therapies is made more difficult by the inconsistent reporting of potentially relevant prognostic factors. The results of an international randomized trial of these two therapies is forthcoming and may address some

ISSN:0361-8609    

DOI:10.1002/ajh.2830500208

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

年代:1995

数据来源: WILEY

摘要:

AbstractA B‐cell lymphoma cell line, designated KML‐1, was established from pleural effusion of a patient with non‐Hodgkin's lymphoma of large‐cell type. The lymphoma arose in the pelvis and ran an aggressive clinical course. Chromosome analysis of the cell line exhibited a complex karyotype including the loss of chromosome 18. To evaluate the molecular events in the cell line that may be associated with the development of the lymphoma, we investigated the expression and/or alterations of several classes of human genes, including oncogenes, tumor suppressor genes, and cytokine genes. The expression of theDCC(deleted in colorectal cancer) gene, located on the chromosome 18q21, was extremely reduced in KML‐1 cell line, as compared with that in a normal spleen tissue and other 4 lymphoma cell lines by the reverse transcription‐polymerase‐chain‐reaction (RT‐PCR) method. This finding suggests that inactivation of theDCCgene might play a role in the pathogenesis of the

ISSN:0361-8609    

DOI:10.1002/ajh.2830500209

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

年代:1995

数据来源: WILEY

摘要:

AbstractExpansion of the natural killer (NK) subset of lymphocytes represents a rare leukemia phenotype with variations in clinical presentation, morphology, surface phenotype, and effector function. This paper reports on a 5‐year‐old male patient who had an unusual presentation of an NK cell leukemia that was initially diagnosed as neuroblastoma. A bone marrow (BM) aspirate showed clumps of undifferentiated cells with the following phenotype: CD56bright+, CD33dim+, CD45‐, CD2‐, CD19‐, CD16‐, and CD57‐. Cytochemistry was noncontributory. The patient, having failed to respond to conventional neuroblastoma chemotherapy, was subsequently diagnosed as having NK cell leukemia based on functional in vitro assays. The patient responded to acute lymphoblastic leukemia (ALL) chemotherapy but relapsed 4 weeks into treatment and eventually died 25 weeks after initial presentation. The cell surface phenotype observed is consistent with a rare NK cell subset, the biology of which has not been well defined. Freshly isolated BM cells killed K562 cells in a conventional51Cr‐release assay. Both interleukin‐2 (IL‐2) and interferon‐α (IFN‐α) induced LAK activity against the Daudi cell line. IL‐2 induced proliferation of the leukemic cells. TNF‐α, IFN‐γ, IL‐6, IL‐1ra, and TGF‐β levels were assessed and found to be concentrated in BM, in contrast to plasma samples. TNF‐α was present at a high concentration in BM (150.9 pg/ml), probably a reflection of the associated disease pathology of severe bone pain and pyrexia. In summary, this paper details clinical and laboratory investigat

ISSN:0361-8609    

DOI:10.1002/ajh.2830500210

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

年代:1995

数据来源: WILEY

ISSN:0361-8609    

DOI:10.1002/ajh.2830500211

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

年代:1995

数据来源: WILEY