摘要:

Erythrocyte volume and haemoglobin concentration in individual red cells from 62 patients with Hb H disease: 37 with H genotype (α-thalassaemia 1/α-thalassaemia 2) and 25 H/CS genotype (α-thalassaemia 1/Hb Constant Spring) were measured using the H*l haematology analyser. All 25 cases with H/CS genotype, the more severe genotype, had microcytes (red cells with a volume smaller than 60 fl) less than 35% and hypochromic red cells (red cells with haemoglobin concentration less than 28 g/dl) more than 35%. A discriminant function, the ratio between the percentage of hypochromic red cells and the percentage of microcytes (Hypo/Micro), was proposed. Most of the H/CS patients (76%) had Hypo/Micro greater than 2.5 whereas those of H patients (82%) were below 2.5. Red cell volume histograms were also characteristically different between the two genotypes: the H/CS had a peak between 60 and 90 fl while the H genotype showed a peak at or very close to 60 fl, indicating a greater degree of microcytosis. Increased hypochromia with a slight decrease in cell size of H/CS red cells suggests that the poor degree of haemoglobinization has no linkage or very little role in disturbing the synthesis of membrane proteins and their assembly to the plasma membra

ISSN:0001-5792    

DOI:10.1159/000204704

出版商:S. Karger AG    

年代:1992

数据来源: Karger

摘要:

Nucleolar organizer regions (NORs) are loops of DNA which transcribes to ri-bosomal RNA. The NOR-related protein becomes visible in nucleus by a silver-staining technique under a light microscope, and it has been named argyrophilic protein of NOR (Ag-NOR). In various malignancies, the correlation between the proliferation potential of tumor cells or histological grade and the number of Ag-NORs has been reported. In this study, we investigated the Ag-NOR of acute leukemic cells and its relation to the in vivo proportion of bone marrow leukemic cells in DNA synthetic phase. The number of Ag-NORs in bone marrow leukemic cells was more than that in peripheral blood (means values 2.78 and 2.48, respectively, p < 0.01). This result shows that the number of Ag-NORs reflects the vigorous proliferative potential of bone marrow leukemic cells. However, no significant correlation was obtained between the number of Ag-NORs and the bromodeoxyuridine-labeling indices (r =0.2064). These results suggest that Ag-NOR might be one of the markers for cellular proliferation in leukemia, while DNA synthesis of leukemic cells do not seem to be directly related to Ag-NOR. In order to clarify the role of Ag-NOR in leukemia, further studies are needed.

ISSN:0001-5792    

DOI:10.1159/000204705

出版商:S. Karger AG    

年代:1992

数据来源: Karger

摘要:

We have identified three types of α-thalassemia in 28 members of an Indian family from Durban, South Africa. The rare South African (SA) type of α-tha-lassemia-1, which is characterized by an ∼23-kb deletion involving the ψζ, ψα2, ψαl, α2, α1, and θl genes, was present in 13 members [6 simple heterozygotes, 5 with Hb H disease of the ∼(S A)/-α(-3.7 kb) type, and 2 with Hb H disease of the --(SA)/-α(-4.2 kb) type]. Seven others were heterozygotes for α-thalassemia-2 (-3.7 kb), 1 was homozygous for this deletion, and 1 was a compound hetero-zygote [-α(-3.7 kb/-α(-4.2 kb)]. Hematological and hemoglobin composition data indicated a moderate anemia in all 7 patients with Hb H disease with severe microcytosis and hypochromia, no elevation of γ-chain synthesis, low levels of Hb A2 (0.3-0.7%), and low levels of Hb H. The most severe disease was present in 2 teenagers with the --(SA)/-α

ISSN:0001-5792    

DOI:10.1159/000204706

出版商:S. Karger AG    

年代:1992

数据来源: Karger

摘要:

Functional analyses were performed on neutrophils isolated from 6 patients from two institutions who displayed features of chronic neutrophilic leukemia (CNL). These neutrophils demonstrated a consistent deficiency (44 ± 8% of control values) in superoxide anion (O-2) production in response to the phorbol ester, phorbol myristate acetate (PMA). O-2 production in response to chemotactic peptides was near normal (82.3 ± 10.7% of control values). Bacterial killing was normal in the two patients studied, and chemotaxis was diminished in response to zymosan-activated plasma and to high concentrations of chemotactic peptides in the patients studied. Cytosolic C kinase activity was decreased in one of the two patients studied. These results suggest that a deficient O-2 release in response to PMA is a hallmark of neutrophils in CNL and may provide a diagnostic indicator of this conditio

ISSN:0001-5792    

DOI:10.1159/000204707

出版商:S. Karger AG    

年代:1992

数据来源: Karger

摘要:

The toxicity and hematologic effects of Escherichia coli- derived recombinant human granulocyte-macrophage colony-stimulating factor (rhGM-CSF) were studied in 58 treatment cycles in Japanese patients with advanced malignancy as a phase I/II clinical trial. rhGM-CSF in doses from 30 to 250 μg/m2/day was administered by 24-hour continuous intravenous infusion, 8-hour intravenous, or a daily subcutaneous injection for 14 days. The most common adverse drug events (ADE) were fever, nausea/vomiting, diarrhea, skin eruption, and phlebitis. The frequency of moderate and severe ADE was 2.9,14.7, 35.3 and 47.1% at 30, 60,125, 250 μg/m2/day, respectively. In terms of administration routes, the frequency of ADE was 69% with 24-hour continuous intravenous infusion, 39.1% with 8-hour intravenous infusion and 16.7% with subcutaneous injection. Regarding the hematologic effects of rhGM-CSF, leukopenia improved in a dose-dependent manner. The appropriate dose level to be used in the phase II study was estimated to be in the range between 60 and 250 μg/m2/d

ISSN:0001-5792    

DOI:10.1159/000204708

出版商:S. Karger AG    

年代:1992

数据来源: Karger

摘要:

Favism is a potentially fatal manifestation of glucose-6-phosphate dehydrogenase (G6PD) deficiency, and it is therefore a public health problem in areas where this genetic abnormality is common. In the district of Sassari (northern Sardinia), the frequency of G6PD male hemizygotes is approximately 7.5%, and therefore all newborns since 1971 have been screened for G6PD deficiency. We have analyzed the incidence of favism in this community in two 10-year periods: (1) 1961-1970; and (2) 1981-1990. In period 1, there were 508 cases of favism, of which 76% occurred in boys. In period (2) there were 144 cases of favism, of which only 52% in boys. Thus, between the two periods there was an overall decrease in the incidence of favism of 75%, whereas the proportion of girls affected has approximately doubled. These data suggest that neonatal screening and health education programs can produce a substantial decrease in the number of cases of favism, and that the relative increase in favism in girls is possibly due to failure of the screening method used to detect all the heterozygotes for G6PD deficiency.

ISSN:0001-5792    

DOI:10.1159/000204709

出版商:S. Karger AG    

年代:1992

数据来源: Karger

摘要:

Autologous mixed lymphocyte reaction (AMLR)-induced suppressor function was studied in 12 patients with chronic idiopathic thrombocytopenic purpura (ITP). The function was found to be significantly impaired (20 ± 55%; p < 0.005) compared with normal subjects (69 ± 25%). AMLRs in these patients were significantly decreased (p < 0.05) compared with normal subjects. There was no significant correlation between AMLR-induced suppressor function and platelet counts. Nine patients were studied for AMLR-induced suppressor function before and after splenectomy. The platelet counts increased significantly as a result of splenectomy, but the AMLR-induced suppressor function showed no significant improvement. The results of this study suggest that suppressor dysfunction in ITP may be an immunologic defect irrespective of disease activity. We consider that this abnormality may reflect in vivo failure of the immunoregulatory (feedback) mechanism in IT

ISSN:0001-5792    

DOI:10.1159/000204710

出版商:S. Karger AG    

年代:1992

数据来源: Karger

摘要:

To clarify the components of cellular immunity responsible for defense against the clonal development of myeloma cells, we tested the capacity of human peripheral blood lymphocytes (PBLs) to inhibit the growth of 3 human myeloma cell lines (RPMI 8226, OPM-1 and OPM-2). RPMI 8226 was found to be sensitive to PBLs, showing almost complete growth arrest when cultured with PBLs for 72 h. Inhibition of the growth of RPMI 8226 cells required direct cell-to-cell contact but not presensitization of the PBLs to the target cells, and did not depend on the generation of soluble factors. CD3+, CD4-, CD8- and CD16- cells were found to be the major subset contributing to inhibition of the growth of RPMI 8226 cells, and this growth inhibition was cytostatic rather than cytotoxic. These characteristics distinguished it from growth inhibition mediated by the natural killer system. Impaired PBL-mediated growth inhibition of RPMI 8226 cells was found in patients with various hematologic diseases, including myeloma. It therefore appears that the CD3+, CD4-, CD8- and CDI6- cell subset might be involved in tumor immunity in myeloma.

ISSN:0001-5792    

DOI:10.1159/000204711

出版商:S. Karger AG    

年代:1992

数据来源: Karger

摘要:

Peripheral blood mononuclear cells (PBMCs) from patients with Chediak-Higashi syndrome (CHS) exhibit minimal natural-killer-like cytotoxicity by standard 51Cr-release assays in vitro. However, a CHS-derived γδ-T-cell clone maintained in long-term culture with low-dose interleukin-2 (IL-2) does exhibit cytotoxic activity. PBMCs from 2 patients with CHS were stimulated with low-dose IL-2 in short-term culture and compared with a control for induction of cytotoxicity against a panel of target cell lines. The CHS-derived PBMCs exhibited IL-2-induced cytotoxicity, though the magnitude of induction was uniformly less than in controls. This was accompanied by no significant change in the proportion of PBMCs bearing the γδ-T-cell receptor antigens. The inducibility of cytotoxicity with low-dose IL-2 in vitro suggests a possible in vivo therapy for patients with

ISSN:0001-5792    

DOI:10.1159/000204712

出版商:S. Karger AG    

年代:1992

数据来源: Karger

摘要:

In a comparison of 47 patients with Philadelphia-chromosome (Ph)-positive chronic myeloid leukemia (CML) in the Nagasaki University School of Medicine and 64 patients with the same disease in the Roswell Park Memorial Institute, the correlation between the modal number of chromosomes and the therapeutic response and/or survival after the onset of the blastic phase (BP) was evaluated. The patients were divided into four groups on the basis of the modal number of chromosomes of the cells in the bone marrow: those with hypodiploidy (group 1), those with pseudodiploidy carrying a Ph chromosome (group 2), those with 47 chromosomes (group 3), and those with 48 or more chromosomes (group 4). The results revealed similar trends in the two institutes. Namely, the therapeutic response and the survival after the onset of the BP in groups 1 and 4 were more unfavorable and shorter than those in groups 2 and 3, although the former (group 2) had a better prognosis than the latter (group 3). Thus, the statistical analysis revealed that the numerical chromosome findings at the BP are useful parameters for assessing the therapeutic response and survival after the onset of the BP of CML.

ISSN:0001-5792    

DOI:10.1159/000204713

出版商:S. Karger AG    

年代:1992

数据来源: Karger