摘要:

AbstractMonclonal antibody (MoAb) GM 58/8 was earlier reported to be directed against an antigen expressed by myeloid progenitors (CFU‐GM), myeloid precursors, granulocytes, and monocytes [5]. Immunophenotyping of 216 cases of acute leukemia [acute myeloblastic leukemia (AML) = 147 and acute lymphoblastic leukemia (ALL) = 69]and 18 cases of chronic granulocytic leukemia in blast crisis (CGLBC) with this antibody showed that GM 58/8 reacted with 92% of AML cases (M1‐M5) and 100% of myeloblastic crisis in CGL cases. All cases of ALL, lymphoblastic crisis in CGL, erythroleukemia, and erythroblastic crisis in CGL were unreactive with GM 58/8. The antibody revealed the myeloid phenotype in an additional 15 cases of otherwise unclassifiable acute leukemia and six cases of CGLBC. Eleven cases of acute “mixed lineage” leukemia were also diagnosed with the help of GM 58/8. The high specificity (100%) and sensitivity (92%) of MoAb GM 58/8 for myeloblastic leukemia is unmatched by almost all previously described myeloid MoAb and proves its usefulness as a single diagnostic reagent for AML and myeloblastic crisis

ISSN:0361-8609    

DOI:10.1002/ajh.2830250202

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

年代:1987

数据来源: WILEY

摘要:

AbstractThe cause of hypogammaglobulinemia in patients with chronic lymphocytic leukemia (CLL) is unknown. Experiments were performed to determine if sera, monocytes, or non‐T cells from patients with CLL suppress the proliferative response and synthesis of immunoglobulin (Ig) following incubation with pokeweed mitogen (PWM) in cocultures with lymphocytes from normal individuals. The data indicate that sera and monocytes from patients with CLL did not suppress the proliferative response or synthesis of Ig by normal non‐T cells. When various numbers of normal non‐T cells and CLL non‐T cells were cocultured with a constant number of normal T cells, the proliferative response and the concentration of supernatant Ig decreased as the proportion of CLL non‐T cells increased. Since similar results were obtained when irradiated non‐T cells from normal individuals were substituted for non‐T cells from patients with CLL, we believe that the decrease in proliferative response and diminished synthesis of Ig is not the result of the suppressor non‐T cells but is related to the dilution of normal B cells by inert non‐ T cells. We conclude that these experiments serve as an in vitro model for patients with CLL and suggest that the hypogammaglobulinemia observed in this disease is related to the diluting out of normal B cells by the accumulation of neoplastic B cells in the peripheral blood, bone marrow, and lymphoid tissue

ISSN:0361-8609    

DOI:10.1002/ajh.2830250203

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

年代:1987

数据来源: WILEY

摘要:

AbstractThis study compared T‐cell colony formation in soft agar of lymphocytes from the peripheral blood and lymph nodes of patients with non‐Hodgkin's lymphoma (NHL) with T‐cell colony formation of peripheral blood lymphocytes from normal individuals. Mononuclear cells were separated from other blood and lymph node elements on density gradient columns, phenotyped for T‐ and B‐cell antigens using monoclonal antibodies, and then plated in soft agar cultures. Lymphocytes from peripheral blood and lymph nodes of patients with NHL exhibited less T‐cell colony formation (p<0.01) than did lymphocytes from normal individuals. This decrease in T‐cell colony formation was not the result of the number of T cells or null cells plated, or differences in proportions of T helper and T suppressor cells. However, when sera from patients with NHL were incubated with normal lymphocytes before plating in soft agar, a decrease in number of T‐cell colonies was observed (p<0.01). We conclude that peripheral blood and lymph node mononuclear cells from patients with NHL have a decreased ability to form T‐cell colonies in soft agar cultures and that this decrease is related, at least in part, to the presence o

ISSN:0361-8609    

DOI:10.1002/ajh.2830250204

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

年代:1987

数据来源: WILEY

摘要:

AbstractTo clarify the role of transferrin receptors in cases of altered iron metabolism in clinical pathological conditions, we studied: 1 number of binding sites; 2 affinity; and 3 recycling kinetics of transferrin receptors on human erythroblasts. Since transferrin receptors are mainly present on erythroblasts, the number of surface transferrin receptors was determined by assay of binding of125I‐transferrin and the percentage of erythroblasts in bone marrow mononuclear cells. 1 The number of binding sites on erythroblasts from patients with an iron deficiency anemia was significantly greater than in normal subjects (p<0.01). Among those with an aplastic anemia, hemolytic anemia, myelodysplastic syndrome, and polycythemia vera compared to normal subjects, there were no considerable differences in the numbers of binding sites. 2 The dissociation constants (Kd) were measured using Scatchard analysis. The apparent Kd was unchanged (about 10 nmol/L in patients and normal subjects. 3 The kinetics of endocytosis and exocytosis of125I‐transferrin, examined by acid treatment, revealed no variations in recycling kinetics among the patients and normal subjects. These data suggest that iron uptake is regulated by modulation of the number of surface transferrin receptors, thereby reflecting the iron demand of the erythrobl

ISSN:0361-8609    

DOI:10.1002/ajh.2830250205

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

年代:1987

数据来源: WILEY

摘要:

AbstractPlatelets from a patient with the Hermansky‐Pudlak syndrome were studied. These platelets had decreased amounts of serotonin and adenine nucleotides, and a decreased number of mepacrine‐labeled dense bodies. β‐Thromboglobulin and acid hydrolases contained in alpha‐granules and lysosomes respectively were present in normal amount. Platelets in platelet‐rich plasma did not respond to collagen, but arachidonic acid and ionophore A 23187 induced normal aggregation and normal thromboxane (TX) synthesis. Alpha‐granule release was found impaired and remained subnormal even with high doses of inducers. In response to thrombin aggregation, release and TX synthesis of isolated metrizamide gradient platelets were found at lower than normal levels. Phosphorylation of P20 and P43 proteins was normal. Only a combination of ADP plus thrombin could restore a normal aggregation, with normal alpha‐granule and lysosome release and normal TX synthesis. These results indicated that in the absence of dense bodies: 1) the release of other granules is impaired; 2) the TX synthesis is delayed except when induced by arachidonic acid and A 23187 ionophore; 3) the absence of dense bodies could be compensated for by the addition of ADP which restores the impaired release reaction and TX formation; and 4) P20 and P43 polypeptides were phosphorylated as rapidly as those in no

ISSN:0361-8609    

DOI:10.1002/ajh.2830250206

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

年代:1987

数据来源: WILEY

摘要:

AbstractPhosphoglycerate kinase deficiency is a rare, x‐linked glycolytic defect that, when severe, can be associated with hemolytic anemia, rhabdomyolysis, or neurological disorders. We report here a new phosphoglycerate kinase variant discovered in a boy with severe hemolytic anemia but no evidence of neuromuscular disease or developmental delay. The biochemical properties of the variant enzyme (greatly increased kmATP and km3‐phosphoglycerate; normal pH optimum, electrophoretic mobility, and substrate specificity; resistance to heat inactivation) establish its uniqueness. Separation of light and dense red cells by centrifugation showed no greater loss of phosphoglycerate kinase activity in dense (“old”) variant cells than in normal cells. We postulate that the striking stability of the variant enzyme allows cells capable of protein synthesis to accumulate sufficient enzyme to limit neuromuscular s

ISSN:0361-8609    

DOI:10.1002/ajh.2830250207

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

年代:1987

数据来源: WILEY

摘要:

AbstractIn view of their permeability to small peptides, it has been postulated that human erythrocytes may play a role in terminating the action of some circulating peptide hormones. Work using classical paper chromatographic techniques for detecting free amino acids indicated that the octapeptide, des‐(Arg9)‐bradykinin, enters these cells and its amino‐terminal arginine residue is released by cytosolic aminopeptidase‐P. We have used1H NMR to monitor directly the release of arginine from bradykinin. The hydrolytic reaction rate in hemolysates, with an initial peptide concentration of 13.0 mmol l−1was 6.5 mmol (1 packed red cell)−1h−1. But no reaction was evident after a 4.5‐h incubation with intact cells, thus contradicting the earlier suggestion that erythrocytes are involved in the primary inactivation of this hormone. This is consistent with our previous findings that the pentapeptide leu‐enkephalin fails to enter human erythrocytes but that its lower‐order degradation

ISSN:0361-8609    

DOI:10.1002/ajh.2830250208

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

年代:1987

数据来源: WILEY

摘要:

AbstractAccurate distinction between essential thrombocythemia and thrombocytotic polycythemia vera requires determination of the red cell mass in the presence of adequate iron stores, but this is not always possible. We therefore compared the clinical and laboratory features at the time of presentation of 50 patients with unequivocal essential thrombocythemia and 27 patients with thrombocytotic polycythemia vera. Univariate analysis failed to identify any single parameter capable of reliably separating the groups. A logistic regression algorithm incorporating hematocrit, white cell count, and spleen size markedly increased the diagnostic accuracy (92 %) compared with predictions based on the hematocrit alone (52 %). The algorithm's usefulness for patients with intermediate hematocrits was confirmed by analysis of independent samples of essential thrombocythemia and thrombocytotic polycythemia vera patients, and also by analysis of patients with probable essential thrombocythemia in whom the diagnosis could not be confirmed because of inadequate exclusion of polycythemia vera. Furthermore, comparison of survival data suggests that differentiating these disorders is prognostically important. The algorithm is recommended as an alternate method for differentiating essential thrombocythemia from thrombocytotic polycythemia vera whenever the red cell mass is unavailable or iron deficiency cannot be excluded.

ISSN:0361-8609    

DOI:10.1002/ajh.2830250209

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

年代:1987

数据来源: WILEY

摘要:

AbstractEarly reports suggested that hemophiliacs with factor IX deficiency (Christmas Disease) may be at less risk for developing the acquired immunodeficiency syndrome (AIDS) than patients with classic hemophilia. We evaluated 12 factor IX deficient patients for clinical and immunologic abnormalities related to infection with the human immunodeficiency virus (HIV). Antibody to HIV was not detected in these patients prior to 1982. By 1985, 66 percent (eight of 12) patients were seropositive. All three concentrates available commercially before 1985 were associated with seropositivity. Furthermore, seropositive hemophiliacs had received on average significantly more factor IX concentrate than seronegative hemophiliacs (27,825 ± 17,976 (S.D.) versus 1,250 ± 1,500 factor units/year, (p<0.02). Half of the seropositive individuals had generalized lymphadenopathy with splenomegaly. Two seropositive patients have developed AIDS, one with cryptococcal meningitis and another with a large cell immunoblastic lymphoma. Infection with HIV has occurred with high frequency in hemophiliacs who received unmodified factor IX concentrate

ISSN:0361-8609    

DOI:10.1002/ajh.2830250210

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

年代:1987

数据来源: WILEY

摘要:

AbstractTwo adolescent black females with sickle cell disease who developed systemic lupus erythematosus (SLE) are presented. The recognition of SLE was delayed because all new symptoms were initially attributed to their underlying sickle cell disease. Nine similar cases have been previously reported. The hypothesis that a deficiency of the alternative complement pathway in some patients with sickle hemoglobinopathies predisposes them toward immune complex disorders was not confirmed in our study. Our first patient had normal and our second increased activity of the alternative pathway of complement activation.

ISSN:0361-8609    

DOI:10.1002/ajh.2830250211

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

年代:1987

数据来源: WILEY