摘要:

AbstractTerminal‐deoxynucleotidyl‐transferase (TdT) activity determinations, using the indirect immunofluorescence technique, were performed on 28 consecutive leukemia patients. Fifteen patients with acute lymphoblastic leukemia had 38 bone marrow smears examined. TdT‐positive cells were a good indicator of disease activity in acute lymphoblastic leukemia in that numbers were increased at the time of diagnosis, normal during remission, and increased during relapse. Ten patients with acute myeloid leukemias had 42 smears studied. The number of TdT‐positive cells did not reflect the activity of the disease, except for two patients with acute myelomonocytic leukemia in which TdT‐positive cells were mildly increased during relapse. Three patients with chronic myelogenous leukemia had eight smears studied. The number of positive cells seemed meaningful only in following the course of lymphoid blast crisis. It would therefore appear that TdT determinations seem to be of value in lymphoblastic leukemia, but of limited value in myeloid

ISSN:0361-8609    

DOI:10.1002/ajh.2830100102

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

年代:1981

数据来源: WILEY

摘要:

AbstractSixty consecutive patients with chronic lymphocytic leukemia (CLL) were studied from both a clinical and laboratory standpoint. Hypoimmunoglobulinemia was found in 45% of patients; many of these patients suffered from severe bacterial infections. Second primary malignancies were diagnosed in 20% of patient's; most of these predated the diagnosis of CLL. HLA‐B17 typing was found in unexpectedly high frequency in a small group of Coombs'‐positive patients. The Rai‐staging system was found to be generally useful for determining prognosis of groups of patients, although less useful in any individual patient. Findings relating to surface membrane immunoglobulin‐positive (B) and E‐rosetting (T) lymphocytes are described. A patient with null‐cell CLL is described. All patients with proliferation of gamma heavy‐chain‐bearing cells were diagnosed in early Rai stages suggesting that this marker may identify a subset of patients who present early and have a good prognosis. These data suggest that lymphocyte marker studies augment the Rai criteria in evaluating prognosis and may eventually be of value to the clinician in evaluating stage of disease and respon

ISSN:0361-8609    

DOI:10.1002/ajh.2830100103

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

年代:1981

数据来源: WILEY

ISSN:0361-8609    

DOI:10.1002/ajh.2830100104

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

年代:1981

数据来源: WILEY

摘要:

AbstractSurface markers, terminal transferase, cytochemical staining, and function of peripheral blood lymphocytes were examined in nine patients with T‐cell lymphoproliferative disease. Anti‐T‐cell antiserum proved useful in characterizing the phenotype of the abnormal cells when only a minority of the abnormal cells formed E‐rosettes. Anti‐B‐cell antiserum was consistently unreactive with the abnormal cells. Terminal transferase was elevated in T‐cell ALL but low in T‐cell CLL, lymphoma‐leukemia, or Sézary syndrome. Lymphocyte proliferative response to nonspecific mitogens was generally depressed as was the response to allogeneic cells. The cells also failed to stimulate in mixed lymphocyte culture, implying a lack of Ia antigens on their surface. Acid phosphatase stain was positive in the three patients tested. Peroxidase and esterase stains were negative, whereas PAS was positive in five of eight patients tested. Prognosis in general, regardless of the clinical picture, (ie, acute, chronic, or lymphoma‐leukemia), was poor with seven of the nine patients dying within 14 months of diagnosis. Therefore, the use of anti‐T‐cell antisera and the E‐rosette surface marker should be used to characterize the phenotype of the malignant cells in lymphoproliferative disease to enable more vigorous treatment of those that are of T‐cell origin and that ther

ISSN:0361-8609    

DOI:10.1002/ajh.2830100105

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

年代:1981

数据来源: WILEY

摘要:

AbstractAmong a cohort of 142 patients with Hodgkin disease (HD), pathologic stages (PS) IA through IVB, 84 remained in complete remission (CR) at least 3 years after the completion of initial protocol therapy. Eight of these patients subsequently developed a recurrence of HD and were defined as having a late relapse. The disease‐free interval ranged from 37 to 76 months (median 53 months). Six of the relapse patterns were those of dissemination. All patients were salvaged and are in CR from 14 to 50 months after relapse. The actuarial survival, measured from the time of relapse, of the eight late‐relapsing patients was superior to the actuarial survival of 38 patients with early relapse (disease‐free interval less than 36 months), but as yet this difference is not statistically significant (P = 0.07, G

ISSN:0361-8609    

DOI:10.1002/ajh.2830100106

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

年代:1981

数据来源: WILEY

摘要:

AbstractColonies derived from erythroid burst‐forming units (BFU‐E) synthesize fetal hemoglobin (HbF) in amounts that far exceed in vivo levels. There is some evidence that HbF synthesis is controlled at the level of a primitive erythroid precursor cell. Dexamethasone may potentiate the development of BFU‐E. Since a means of augmenting HbF production in sickle cell anemia or severe β‐thalassemia would be of great therapeutic value, we studied the effects of dexamethasone on HbF and γ‐globin chain synthesis in BFU‐E from patients with sickle cell anemia and controls. HbF was measured by radioimmunoassay of BFU‐E lysate and γ‐chain synthesis by the incorporation of3H‐leucine into globin, which was then purified by gel filtration and column chromatography. Dexamethasone (10−9M) produced an increase in the number of BFU‐E in 16 of 19 subjects when compared with numbers of BFU‐E cultured with only erythropoietin. The individual BFU‐E were larger and contained more subcolonies. Dexamethasone did not increase HbF or γ‐chain synthesis, and there was no relationship between increased proliferation of BFU‐E and augmented HbF production. Thus, although dexamethasone augmented the development of erythroid burs

ISSN:0361-8609    

DOI:10.1002/ajh.2830100107

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

年代:1981

数据来源: WILEY

摘要:

AbstractA familial erythrocytosis is described. The propositus is a 52‐year‐old man who was found, on routine testing, to have an erythrocytosis with a Hb level of 19.8 g/dl. Physical examination and laboratory findings revealed an associated hemolysis. The P50of the stripped hemolysate was normal. The 2,3‐DPG level was reduced to 15% of normal but that of ATP was increased more than twofold. Red cell diphosphoglycerate mutase activity was normal as were the levels of all red cell glycolytic and hexose monophosphate shunt enzymes with the exception of pyruvate kinase (PK). The level of the latter was elevated to 24.5 U/g(normal:6.3 ± 1.1). The electrophoretic pattern of hemolysate PK was abnormal, showing three additional bands; one of these migrated as the M2isozyme. The findings were similar in the propositus's daughter and her daughter and in one of the two brothers of the propositus. The second brother and the son of the first brother also exhibited erythrocytosis but their red cell 2,3‐DPG levels were normal. In addition, the level of their RBC PK was reduced to between 50% and 60% of normal and the abnormal electrophoretic bands were absent. The erythrocytosis appears to be inherited as an autosomal‐dominant trait. The relationship between the PK abnormalities appea

ISSN:0361-8609    

DOI:10.1002/ajh.2830100108

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

年代:1981

数据来源: WILEY

摘要:

AbstractThe most common abnormality of the platelets in 43 patients with a variety of myeloproliferative syndromes was impaired or absent aggregation when stimulated with collagen and adrenaline. Eight unselected cases studied in more detail showed a normal prostaglandin synthesis pathway as evidenced by normal aggregation with arachidonic acid and the production of normal amounts of malonyldialdehyde. Mixing experiments with aspirin‐tested platelets showed correction of the abnormal adrenaline and collagen responses and confirmed that the nature of the defect was different from that induced by aspirin. Stimulation of “myeloproliferative” platelets with thrombin after blocking the prostaglandin pathway with aspirin resulted in reduced aggregation, indicating either a deficiency of the storage pool of adenine nucleotides in the platelets or an abnormality of a membrane receptor for thr

ISSN:0361-8609    

DOI:10.1002/ajh.2830100109

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

年代:1981

数据来源: WILEY

摘要:

AbstractGranulocyte antibodies are important in the pathogenesis of neonatal granulocytopenia, in some varieties of idiopathic granulocytopenia, and in transfusion reactions, particularly since the development of leukocyte transfusion therapy for granulocytopenic patients. Previous assays for the detection of granulocytespecific antigens and antibodies were cumbersome, complex, and unreliable. Thus we have now described a new microimmunofluorescent method to assay human granulocyte antigens and their antibodies. This sensitive and reliable technique allows for the preservation of human granulocyte antigenicity for at least 24 hours. The granulocyte‐specific antigens of a Caucasian population, residing in metropolitan New York, were studied by this microimmunofluorescent method. We report the distribution of granulocyte‐specific antigens in this population, together with the corresponding gene frequency. This microimmunofluorescent test should improve our understanding of antigenic systems restricted to single cell populati

ISSN:0361-8609    

DOI:10.1002/ajh.2830100110

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

年代:1981

数据来源: WILEY

摘要:

AbstractA 27‐year‐old woman with β‐thalassemia/hemoglobin E disease presented with an intracranial mass of 10 × 9 × 1.2 cm firmly attached to the dura and the falx cerebri extending over the central part of both hemispheres, with pressure atrophy of the underlying brain. Histopathologic examination revealed that the mass consisted entirely of hematopoietic cells. We have seen 1,315 cases with β‐thalassemia/hemoglobin E, but this was the first case with intracranial extramedullary hematop

ISSN:0361-8609    

DOI:10.1002/ajh.2830100111

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

年代:1981

数据来源: WILEY