摘要:

AbstractTwo murine monoclonal antibodies (MoAbs), LAM3 and LAM7 of the IgG1isotype, which were produced by immunization with normal peripheral blood monocytes (PBM), were assayed in their specificity by indirect immunofluorescence against a panel of normal as well as leukemic cells.Both LAM3 and LAM7 were reactive with PBM while LAM3 also recognized platelets. Neither MoAb showed reactivity with erythrocytes, granulocytes, or resting and mitogen activated B and T lymphocytes. The reactivity with bone marrow cells correlated with the degree of monocyte contamination.Among the 62 cases of leukemia tested, which included three cases of B‐CLL, 19 cases of ALL, and 40 cases of ANLL, both MoAbs reacted highly homogenously only with M5b ANLL cells. These findings indicate that the two MoAbs, which recognize two distinct epitopes, represent useful markers in the differential diagnosis of M5b ANL

ISSN:0361-8609    

DOI:10.1002/ajh.2830250402

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

年代:1987

数据来源: WILEY

摘要:

AbstractWe studied the behavior in culture of megakaryocyte progenitor cells (CFU‐Mk) from peripheral blood (PB) and bone marrow (BM) cells in eight patients with myeloproliferative diseases (MPD). In seven patients we observed megakaryocyte (Mk) colony formation from PB cells, which were generated in the absence of any added stimulator and which did not increase after the addition of a source of Mk‐colony stimulating activity (CSA‐Mk). The number of BM CFU‐Mk was significantly higher in patients than in controls, and in seven out of eight patients the responsiveness to added CSA‐Mk was retained. Plasma obtained from six patients did not stimulate normal donors' BM target cells to form Mk colonies. These data demonstrate an expansion of the CFU‐Mk pool in MPD patients without increased plasma levels of CSA‐Mk, and suggest that PB and BM CFU‐Mk of MPD patients might have different kin

ISSN:0361-8609    

DOI:10.1002/ajh.2830250403

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

年代:1987

数据来源: WILEY

摘要:

AbstractTo assess the role of platelets in thrombohemorrhagic complications of acute respiratory failure (ARF), we studied platelet function in 13 ARF patients admitted for intensive care, in six acutely ill intensive care patients without evidence of acute lung injury (non‐ARF), and in 10 normal subjects. Platelet counts in ARF and non‐ARF patients were similar to the normal range. The bleeding time of the ARF patients (8.5 ± 0.9 min) was significantly longer (p<0.01) than the normal (4.8 ± 0.2 min) but similar to non‐ARF patients (5.4 ± 0.8 min). The bleeding time prolongations in ARF patients were unrelated to platelet concentration. Platelet aggregation induced by ADP and thrombin was normal in both ARF and non‐ARF patient groups. The epinephrine response was impaired in one non‐ARF patient and in three ARF patients; collagen‐induced aggregation was absent in two ARF patients, with a prolonged bleeding time. Levels of VIII:C and vWF in both groups of patients were similar to the normal level, but VIIIR:Ag levels in ARF patients (407 ± 45% of normal) were higher (p<0.01) than in both non‐ARF patients (210% ± 10%) and normal subjects (106% ± 4). The electrophoretic mobility of VIIIR:Ag was abnormal in ARF patients. The prolonged bleeding time in ARF patients appears to result from the qualitative and quantitative VIIIR:Ag defect. β‐Thromboglobulin levels were greater (p<0.01) in ARF patients (87.6 ± 6.9 ng/ml; p<0.001) than in non‐ARF patients (46.2 ± 3.1 ng/ml) or in normal subjects (25.3 ± 2.5 ng/ml p<0.0001). However, platelet factor 4 plasma levels in ARF patients (18 ± 1.6 ng/ml) did not differ from those in non‐ARF patients (15.0 ± 3.0 ng/ml), but both were significantly different from normal (6.1 ± 0.8 ng/ml). Plasma thromboxane B2(T × B2) levels were not different from normal values in either ARF or non‐ARF patients, but 6‐keto‐PFG1αlevels were significantly reduced (p<0.01) in ARF patients (215 ± 43 pg/ml) compared to normal values (381 ± 34 pg/ml). Non‐ARF patients had 6‐keto‐PGF1αlevels (285 ± 111 pg/ml) midway between the normal values and those of ARF patients. Our results suggest that in vivo platelet activation occurs in ARF. ARF patients have quantitative and qualitative platelet defects that may cont

ISSN:0361-8609    

DOI:10.1002/ajh.2830250404

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

年代:1987

数据来源: WILEY

摘要:

AbstractThe hematology and pathophysiology of sickle cell disease during the postnatal development of younger hemoglobin (Hb) S homozygotes (SS) could be considerably affected by a variability of α globin gene numbers. We have documented longitudinal developmental changes of hematological values and hemoglobin composition on 147 patients with SS (αα/αα), 64 with SS (‐α/αα), and 9 with SS (–α/–α) between the ages of 1 and 15 years. Non‐steady‐state data were excluded from these analyses. The number and organization of α globin genes was established by gene mapping. As anticipated, mean corpuscular volume and erythrocyte counts correlated with α globin gene numbers throughout the 15‐year age interval. On the other hand, SS children with αα/αα, –α/, –α had similar hemoglobin concentrations up to the ages of 5–10 years. Around the age of 7, the SS patients with –α/–α developed a higher Hb concentration than that of the SS (–α/αα), which in turn was higher than that of the SS (αα/αα). The emergence of this difference coincided with a developmental increase of the mean corpuscular hemoglobin concentration (MCHC) in patients with SS (αα/αα) and the decline of Hb F % under 15%. This newly observed developmental change of the MCHC could lead to increased hemolysis and anemia after the age of 5‐10 years. It occurs to a smaller extent among SS (–α/αα) or not at all among SS (–α/αα) such that these two categories of patients have less severe hemolysis and higher hemoglobin levels at older ages. Although the proportion of Hb F was independent of α globin gene numbers, the absence of Hb Bart's suggested that α‐thalassemia promotes the intracellular assembly of Hb F over Hb S tetramers. Thus, the interaction of α‐thalassemia and Hb F in young SS patients may be more complex than revealed by Hb F levels in cell lysates. Among older SS children (>7 years) α‐thalassemia and Hb F levels exceed

ISSN:0361-8609    

DOI:10.1002/ajh.2830250405

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

年代:1987

数据来源: WILEY

摘要:

AbstractSeveral members of a Black family with a heterozygosity for anAγGβ+‐HPFH, shown in 1969 to have relatively low levels of Hb F and a low glycine to alanine ratio in the γ chain of this Hb F, were reinvestigated. Thirteen of 30 available family members in two generations had the heterozygous form of this condition, which was characterized by a decreased level of Hb A2, an average Hb FADvalue of 13.3%, an equal distribution of Hb F over the red cells, and normal hematological values. The γ chain composition of isolated Hb F was determined by reversed phase high performance liquid chromatography for all 13 heterozygotes and showed an averageAγ value of 84.5 %. Hybridization with synthetic oligonucleotides, specific for normal and mutant sequences at positions 111–129 5′ to theAγ globin gene, identified a G ± A base substitution at position 117, similar to that seen in subjects with the GreekAγ‐HPFH. Our data support conclusions by others [15,16,30] that this replacement is causative of the increasedAγ chain synthesis in this condition. Haplotype analysis supported the suggestion that the G ± A substitution occurred as an independent event in

ISSN:0361-8609    

DOI:10.1002/ajh.2830250406

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

年代:1987

数据来源: WILEY

摘要:

AbstractForty‐seven Chinese suffering from β thalassemia major and their parents were studied to establish linkage of the βthaland βAgenes with 11 restriction site polymorphisms. There is marked linkage disequilibrium at the BamH I site 3′ to the β globin gene, such that, in 31% of pregnancies, absence of the site in the fetus can exclude β thalassemia major. Using four restriction sites (Hinc II β, Ava II β, Hind III β, and BamH I β), prenatal diagnosis is feasible in all families. In 46% of all cases, a definitive diagnosis can be made, and in the remaining cases, a 50% chance of exclusion is possible. Fetal blood globin chain analysis would be required for the failures. Our experience in nine successive β thalassemia prenatal diagnosis is

ISSN:0361-8609    

DOI:10.1002/ajh.2830250407

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

年代:1987

数据来源: WILEY

摘要:

AbstractA study was done to find out whether apotransferrin receptors are involved in the release of iron from reticuloendothelial cells. To this end, human macrophages which had been obtained by culturing blood monocytes for 7 days were incubated with either diferric or apotransferrin at the physiological pH of 7.4 or at an acidic pH (6.0). While specific diferric transferrin receptors (Kb1.3 × 10−8M) were demonstrated at pH 7.4, no apotransferrin receptors were found. In contrast, both diferric receptors (Kd2.1 × 10−8M) and apotransferrin receptors (Kd2.8 × 10−9M) were found at pH 6.0. The finding of specific apotransferrin binding at acidic pH fits in with the current understanding of iron uptake by cells, in which the iron‐transferrin complex is endocytosed and the iron is released at acidic pH. The present results suggest that the apotransferrin remains attached to its receptor in the endocytosed vesicle at this acidic pH but that it becomes detached at the cell surface where the pH is neutral. No evidence was found to indicate that iron is transported out of macrophages via apotransferrin receptors at the physio

ISSN:0361-8609    

DOI:10.1002/ajh.2830250408

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

年代:1987

数据来源: WILEY

摘要:

AbstractThis report describes a patient who developed a malignant proliferation of granular lymphocytes following Epstein‐Barr virus (EBV) infection. For many months, his illness resembled prolonged infectious mononucleosis with persistent fatigue, fever, leukocytosis, and serologic evidence of recent primary EBV infection. After approximately I year, however, he developed progressive granular lymphocytosis and extensive lymphocytic infiltration of the bone marrow and liver. Tests for EBV DNA in pre‐ and postmortem tissue samples using a sensitive DNA hybridization technique were negative. Southern blot analysis of DNA prepared from blood mononuclear cells demonstrated clonal T‐cell antigen receptor gene rearrangement. Despite increased numbers of circulating lymphocytes with the morphology and surface phenotype of normal donor natural killer (NK) cells, the patient's NK activity was consistently depressed in a standard in vitro assay. However, in vitro incubation with interleukin‐2 (IL‐2), but not with α‐ or ‐γ‐interferon, increased the NK activity of the patient's lymphocytes. Intravenous recombinant IL‐2 treatment transiently increased the patient's blood NK activity and was associated with seroconversion to EBV nuclear antigens but failed to affect the progression of his disease. Our findings indicate that clonal granular lymphocytic proliferation may develop after EBV infection and confirm the utility of DNA hybridization analysis in distinguishing monoclonal from benign immunoreactive lymphoproliferation. Furthermore, our results suggest that certain functionally inert neoplastic granular lymphocytes acquire NK activity w

ISSN:0361-8609    

DOI:10.1002/ajh.2830250409

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

年代:1987

数据来源: WILEY

摘要:

AbstractThe phenotypic marker profile of a case of acute leukemia is described; its immunophenotype is unique in that the blast cells were initially negative for a wide panel of monoclonal antibodies (McAbs) to surface and intracytoplasmic antigens and for the nuclear enzyme terminal deoxynucleotidyl transferase. Morphological and cytochemical examination suggested an acute myeloid leukemia (AML), but the cells were unreactive with anti‐myeloid McAbs. Treatment with the phorbol ester 12‐0‐tetradecanoylphorbol 13‐acetate (TPA) induced the cells to differentiate morphologically to macrophage‐like cells and led to the expression of surface antigens that could be detected by antimyeloid McAbs. It is not clear whether these cells represent a rare subclass of leukemic cells that are void of any characteristic surface markers but have the potential to differentiate along the myeloid axis, or whether the antigens were masked by an unknow

ISSN:0361-8609    

DOI:10.1002/ajh.2830250410

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

年代:1987

数据来源: WILEY

ISSN:0361-8609    

DOI:10.1002/ajh.2830250411

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

年代:1987

数据来源: WILEY