摘要:

A 10-year-old female with a complete selective IgA deficiency and recurrent autoimmune disease (chronic active hepatitis, focal glomerulonephritis, hemolytic anemia and thrombopenic purpura) is presented. Both serum IgA and saliva secretory IgA were below the detection limit. The small bowel biopsy using a peroxidase-antiperoxidase technique showed absence of plasma cells secreting IgA. Circulating antibodies against mitochondria, microsomal thyroid antigen were detected as well as rheumatoid factor. Circulating immune complexes were present. A positive Coombs’ test and a slightly positive reaction for cryoagglutinines were demonstrated. No alterations in cellular immunity were observed. Clinical and analytical improvement with prednisone and azathioprine was obtaine

ISSN:0001-5792    

DOI:10.1159/000206070

出版商:S. Karger AG    

年代:1986

数据来源: Karger

摘要:

Collagen synthesis stimulated by materials released from platelets (MRFP) of patients with chronic granulocytic leukemia (CGL) and polycythemia vera (PV) was measured in human bone marrow fibroblasts derived from normal individuals. The MRFP of patients with CGL and PV stimulated normal fibroblast collagen synthesis significantly compared to the response from normal MRFP. Platelets derived from patients with CGL and PV may have some factors that dramatically stimulate collagen synthesis which is relevant to the frequently observed myelofibrosis in these diseases.

ISSN:0001-5792    

DOI:10.1159/000206071

出版商:S. Karger AG    

年代:1986

数据来源: Karger

摘要:

The expression of the enzyme marker terminal deoxynucleotidyl transferase (TdT) was examined by immunofluorescence assay in the cells from 333 cases with various types and subtypes of leukemia or lymphoma. More than 90% of cALL and T-ALL, 70% of Null-ALL and 80% of pre-B-ALL were TdT-positive. One case in the commonly TdT-negative group of B-ALL showed TdT-positive cells. All cases of mature B-cell malignancies (B-CLL, hairy cell leukemia, B-cell lymphoma) have been TdT-negative. In the group of mature T-cell malignancies, T-CLL and mycosis fungoides were negative and 2 out of 6 mature T-cell lymphomas were TdT-positive. 13% of acute myeloid leukemias and 36% of CML in blast crisis expressed TdT. Therefore, these TdT-positive cases of CML in blast crisis also carrying the common ALL-antigen belong to the lymphoid subtype. CML and erythroleukemia were invariably TdT-negative. TdT has become an indispensable indicator of immature lymphoid leukemia cells and is particularly valuable as part of the panel of markers used in leukemia phenotyping.

ISSN:0001-5792    

DOI:10.1159/000206072

出版商:S. Karger AG    

年代:1986

数据来源: Karger

摘要:

Circulating CFU-GM, BFU-E and CFU-MIX were assayed in 21 infants aged between 1 day and 44 weeks. Compared to 15 adults, progenitor cells of all types were increased until 10 weeks following birth and approached the respective ranges of adults thereafter. The mean increases of progenitor cells in infants aged between 1 day and 10 weeks were 26-fold for CFU-GM, 7-fold for BFU-E and 5-fold for CFU-MIX. Our results demonstrate that not only committed progenitor cells (CFU-GM, BFU-E), but also early progenitor cells with the capacity for self-renewal (CFU-MIX), are increased in early infancy. These data further support the hypothesis that high levels of blood progenitor cells in very early stages of life reflect the colonization process of developing bone marrow by circulating progenitor cells and demonstrate the terminal phase of this process during the first 10 weeks after birth.

ISSN:0001-5792    

DOI:10.1159/000206073

出版商:S. Karger AG    

年代:1986

数据来源: Karger

摘要:

High-resolution chromosome banding studies were carried out on leukemic cells from a young patient with acute nonlymphocytic leukemia (ANLL), M2 of the FAB classification. A new chromosomal abnormality involving a translocation between chromosomes 3 and 21 was observed, i.e., t(3;21)(p14;q22). A complete remission was never obtained in spite of aggressive chemotherapy and the patient died 8 months after diagnosis.

ISSN:0001-5792    

DOI:10.1159/000206074

出版商:S. Karger AG    

年代:1986

数据来源: Karger

摘要:

Distribution of membrane-bound Gc protein (mGc) in normal lymphocyte subpopulations and leukemic lymphocytes was studied using a rabbit antiserum specific to Gc protein. Forty to fifty percent of the normal peripheral blood mononuclear cells were stained with anti-Gc antiserum by membrane immunofluorescence, and almost all of the B cells (Bl+) and NK cells (Leu 11+) were mGc-positive. Only a few resting T cells (T3+) reacted with anti-Gc, but mGc appeared in a significant number of the T cells after activation by PH A. The distribution of mGc on leukemic lymphocytes of various types nearly corresponded to that in normal lymphocyte subpopulations.

ISSN:0001-5792    

DOI:10.1159/000206075

出版商:S. Karger AG    

年代:1986

数据来源: Karger

摘要:

The activities of the antioxidative enzymes superoxide dismutase, catalase and glutathione peroxidase and the concentration of reduced glutathione were determined in CPD-adenine blood stored at 4°C for up to 20 days. Superoxide dismutase activity was found to be significantly reduced with storage beyond 10 days. The clinical significance of this finding is discussed

ISSN:0001-5792    

DOI:10.1159/000206076

出版商:S. Karger AG    

年代:1986

数据来源: Karger

摘要:

The first fatal case of AIDS in an hemophiliac observed in Italy is reported. The propositus is a 53-year-old hemophilia A patient who died on the 8th December, 1984. AIDS was documented clinically and in the laboratory by serum antibodies to HTLV-III detected by ELISA and Western blot assays. A progressive intellectual worsening of the patient due to diffuse cerebral atrophy was followed by CT scan, EEG and by evaluation of proper neurological signs and symptoms.

ISSN:0001-5792    

DOI:10.1159/000206077

出版商:S. Karger AG    

年代:1986

数据来源: Karger

摘要:

The case of a 42-year-old woman with clinical and histopathological features suggesting acute myelofibrosis is reported. The patient succumbed to massive intracerebral haemorrhage.

ISSN:0001-5792    

DOI:10.1159/000206078

出版商:S. Karger AG    

年代:1986

数据来源: Karger

摘要:

A case of leukemic mastocytoma terminating in metamorphosis is reported. Three types of mastocytoma cells appeared in the peripheral blood and bone marrow, each differing in cytochemical staining and maturation ability in both in vitro and in vivo cultures. Most immature mastocytoma cells, which contained no granules and were lymphoblast-like cells, differentiated to have basophilic and metachromatically positive granules in the cytoplasm after culture in the diffusion chamber, resembling more mature cells (type II). Mature cells (type II), which had diffusely dispersed and metachromatically positive granules in the cytoplasm, came to have vacuoles with basophilic substances after culture in vitro, resembling most mature cells (type I) which contained large and clumped granules with metachromasia. These findings together with clincial findings and results of morphological studies suggest that most immature cells originate from second mature or most mature cells and effect a blastic transformation.

ISSN:0001-5792    

DOI:10.1159/000206079

出版商:S. Karger AG    

年代:1986

数据来源: Karger