摘要:

AbstractIn order to investigate the cause of the anemia concomitant with rheumatoid arthritis (RA), we examined, using the erythroid colony assay of human bone marrow colonyforming units‐erythroid (CFU‐e) and burst‐forming units‐erythroid (BFU‐e), the effects of the patients' serum and peripheral blood T lymphocytes on the CFU‐e‐derived colonies.The counts of erythroid colonies of RA patients were markedly lower than those of human control subjects [CFU‐e: control 152.9 ± 30.6 (n = 19), RA 51.1 ± 13.6 (n = 7), t = 7.66567, p<0.01; BFU‐e: control 25.2 ± 5.9 (n = 5), RA 12.6 ± 2.6 (n = 7), t = 4.574, p<0.01]. The serum from two out of seven RA patients slightly inhibited the formation of CFU‐e‐derived colonies of human control subjects (t = 2.31, 0.05

ISSN:0361-8609    

DOI:10.1002/ajh.2830250102

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

年代:1987

数据来源: WILEY

摘要:

AbstractMany immunologic studies of acute lymphocytic leukemia (ALL) during the past decade have demonstrated the close correlation of immunologic phenotypes of ALL subclasses with the clinical presenting features and prognosis. However, the clinical application of conventional immunologic techniques had been very limited because of the requirement of a fresh sample to prepare the mononuclear cell suspensions for study. We studied 81 cases of ALL using immunoperoxidase stain for nuclear terminal deoxynucleotidyl transferase (TdT) and immunoalkaline phosphatase stain for surface markers (using monoclonal antibody J5for common ALL antigen [CALLA], Leu‐1 for pan‐T antigen, and B1for pan‐B antigen) on air‐dried smears. The cases were classified as common ALL (TdT+, CALLA+, pan‐T−, and pan‐B−) (41 cases), null‐ALL (TdT+, CALLA−, pan‐T−, and pan‐B−) (19 cases), T‐ALL (TdT+, CALLA−, pan‐T+, and pan‐B−) (nine cases), B‐ALL (TdT−, CALLA−, pan‐T−, and pan‐B+) (six cases), pre‐B‐ALL (TdT®, CALLA+, pan‐T−, and pan‐B+) (four cases), or pre‐T‐ALL (TdT+, CALLA+, pan‐T+, and pan‐B−) (two cases). This subtyping of ALL correlated well with known clinical presenting features, prognosis, chromosome analysis in 35 cases with an abnormal clone, and conventional immunologic typing in 38 cases. The data suggest that these simple and practical immunocy

ISSN:0361-8609    

DOI:10.1002/ajh.2830250103

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

年代:1987

数据来源: WILEY

摘要:

AbstractAn immunoperoxidase technique was used to examine the distribution of lymphocyte subsets in bone marrow biopsies of 15 patients with neutropenia and seven non‐neutropenic controls. The bone marrow of most patients and controls had similar distributions of immune effector cells characterized by a diffuse array of predominantly cytotoxic/suppressor T‐cells and occasional nodular aggregates of helper T‐cells. Cells displaying the natural killer cell marker HNK‐1 were sparse in controls and most neutropenic patients. However, marked increases in marrow HNK‐1 + cells were identified in four of the 15 patients. Three of these patients had diffuse HNK‐1 + infiltrates associated with increased Leu 4+ (OKT‐3 +) T‐cells while one had a nodular HNK‐1 + infiltrate associated with small B‐cell follicles. Each of these patients had clinical features similar to those described in the large granular lymphocyte (LGL) lymphocytosis (leukemia) syndrome, but only one of four demonstrated persistently increased numbers of LGLs in the peripheral blood. Thus, this study extends the association of neutropenia and increased numbers of cells with a T/NK phenotype to include patients whose bone marrow is the only demonstrable site of involvement. Since morphologic examination of the bone marrow could not identify the bone marrows with increased HNK‐1 + cells, immunulogic techniques are required

ISSN:0361-8609    

DOI:10.1002/ajh.2830250104

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

年代:1987

数据来源: WILEY

摘要:

AbstractLow‐dose Ara‐C (10 mg/m2subcutaneously bid) has been used as an alternative therapy for acute nonlymphocytic leukemia (ANLL) and myelodysplastic syndromes. We sought to define its therapeutic mechanism by assessing clinical and cytogenetic responses to treatment in conjunction with careful in vitro study of both morphologic and functional characteristics of bone marrow cells cultured with Ara‐C. Sixteen patients (12 ANLL, four myelodysplastic syndrome) were treated. All developed pancytopenia and 11 of 12 had bone marrow hypoplasia during treatment. Four had a meaningful clinical response while five more showed in vivo leukemic cell sensitivity to low‐dose Ara‐C. Seven showed no reponse. Cells with cytogenetic abnormalities were either decreased in number or eradicated during clinical improvement. Liquid culture of marrow mononuclear cells with Ara‐C (.033‐.333 μg/ml × 7 days) produced little evidence of morphologic or functional differentiation (ten of 11 studied). No functional maturation was observed in cells from clinically responding patients. We conclude that low‐dose Ara‐C is modestly effective for some patients with ANLL or myelodysplasia. However, no evidence for in vivo leukemic differentiation is suggested by either in vitro culture studies or cytogenetic correlates of clinical response. In vitro marrow culture studies failed to predict clinica

ISSN:0361-8609    

DOI:10.1002/ajh.2830250105

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

年代:1987

数据来源: WILEY

摘要:

AbstractEven though it is generally held that cryoprecipitate and fraction I‐0 correct the prolonged bleeding time (BT) in patients with von Willebrand disease (VWD), perusal of reported data indicates that the correction is usually short‐lasting and often partial. We decided to do a controlled study of the relationship between the multimeric structure of von Willebrand factor (VWF) and the BT in five patients with severe (type III) VWD after infusion of three plasma concentrates (“wet” cryoprecipitate, lyophilized cryoprecipitate, and fraction I‐0) given in random order. The dosage of concentrates was tailored from in vitro measurements to achieve post‐infusion levels of ristocetin cofactor above the lower normal limit (50 U/dL) for at least 3 hours. The postinfusion BT became transiently normal in only two of five patients treated with wet cryoprecipitate, whereas it remained prolonged in all five patients treated with lyophilized cryoprecipitate or fraction I‐0. For all the concentrates, the proportion of large VWF multimers calculated by scanning the electrophoretic gels were the same as those for normal standard plasmas. An intact multimeric structure was recovered in postinfusion plasmas of patients treated with wet cryoprecipitate, whereas there was a postinfusion loss of large multimers after lyophilized cryoprecipitate and fraction I‐0. These findings indicate that the attainment of a normal BT is the exception rather than the rule after the infusion of three plasma fractions used in the treatment of severe VWD, and that an intact multimeric structure in concentrates and postinfusion plasmas is necessary but not sufficient to sust

ISSN:0361-8609    

DOI:10.1002/ajh.2830250106

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

年代:1987

数据来源: WILEY

摘要:

AbstractChildhood chronic neutropenia with decreased numbers of chemotactic factor receptors as well as defective chemotaxis was first demonstrated in an 8‐month‐old girl. Chemotactic factor receptors on neutrophils were assayed using tritiated N‐formyl‐methionyl‐leucyl‐phenylalanine (3H‐FMLP). The patient's neutrophils had decreased numbers of the receptors: numbers of the receptors were 20,000 (<3 SD) as compared with those of control cells of 52,000 ± 6,000 (mean ± SD) (n = 10). The neutropenia disappeared spontaneously by 28 months of age parallel with the improvement of chemotaxis and increase in numbers of chemotactic factor receptors. These results demonstrate a transient decrease of neutrophil chemotactic factor receptors as one of the pathophysiological bases of a transient defect of neutrophil chemotaxis i

ISSN:0361-8609    

DOI:10.1002/ajh.2830250107

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

年代:1987

数据来源: WILEY

摘要:

AbstractWe evaluated the efficacy of alternate day, high dose prednisolone for the treatment of paroxysmal nocturnal hemoglobinuria (PNH). Nineteen patients were included. Thirteen were men and six were women, aged between 13–56 years. Eleven patients improved, eight with good response and three with fair response. Eight patients were non‐responders. Responders had gradual improvement in the hemoglobin level, but none achieved a normal hemoglobin level. Age at diagnosis, sex, initial hemoglobin, white count, and percentage of a positive Ham's test had no apparent bearing on treatment outcome. A prolonged interval from diagnosis to prednisolone treatment decreased the chance of a favorable hematologic response to therapy. Age at the treatment in non‐responders was higher than responders. Responders had higher numbers of colonies derived from BFU‐E and CFU‐GM both in the blood and bone marrow than non‐responders although the differences did not achieve statistical significance. These data indicate that alternate day, high dose prednisolone therapy is effective in some patien

ISSN:0361-8609    

DOI:10.1002/ajh.2830250108

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

年代:1987

数据来源: WILEY

摘要:

AbstractA 66‐year‐old man with peptic ulcer desease developed a paraprotein that resulted in a spontaneously prolonged prothromoin time, activated partial thromboplastin time, and thrombin clotting time. Although the reptilase time was normal, the thrombin clotting time failed to correct with the addition of normal plasma, calcium, or protamine sulfate. The patient's purified fibrinogen was normal, but his serum contained an IgG that inhibited the clotting of normal plasma and purified fibrinogen in the presence of thrombin. In contrast to previously described paraproteins, this patient's IgG appeared to inhibit the activity of thrombin per se rather than to interfere with fibrinogen cleavage or fibrin polymerization. Although immunoprecipitation between thrombin and the paraprotein could not be demonstrated, the patient's purified IgG, in the presence of thrombin, decreased the thrombin activity on a chromogenic substrate. Further, increasing concentrations of thrombin overcame the inhibitory effect of the patient's paraprotein. Thus, the patient's paraprotein appeared to possess antithrombin activ

ISSN:0361-8609    

DOI:10.1002/ajh.2830250109

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

年代:1987

数据来源: WILEY

摘要:

AbstractBone marrow transplantation is possible for only a minority of patients with severe aplastic anemia. There has been successful treatment in some patients with immunosuppressive agents: high‐dose 6‐methylprednisolone, antilymphocyte globulin, and antithymocyte globulin. We report the successful treatment oftwo patients with severe aplastic anemia with the simultaneous adminstration of antithymocyte globulin and high‐dose 6‐methylprednisolone after failure with antithymocyte globulin and low‐dose cortic

ISSN:0361-8609    

DOI:10.1002/ajh.2830250110

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

年代:1987

数据来源: WILEY

摘要:

AbstractSevere neutropenia due to selective loss from the bone marrow of cells of the entire neutrophil maturation sequence developed in a patient with Goodpasture's Syndrome and was associated with serious infections complicating continuous ambulatory peritoneal dialysis. Involvement of T‐lymphocytes in the process affecting the neutrophil series was implicated by the relation between recovery from neutropenia and treatment with antithymocyte globulin (ATG). Azathioprine and corticosteroid administration failed to sustain recovery from neutropenia induced by ATG. It is concluded that ATG can provide a nonmyelotoxic form of therapy for pure white cell aplasia whose effectiveness is independent of responsiveness to other immunosuppressive agent

ISSN:0361-8609    

DOI:10.1002/ajh.2830250111

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

年代:1987

数据来源: WILEY