摘要:

Ten cases of chronic myeloproliferative diseases with myelofibrosis and circulating megakaryoblasts have been studied. The megakaryoblasts were recognized by the presence of megakaryocyte-platelet-related cell surface antigens, detected by an immuno-electron-microscopic method. They showed a tendency to develop from ‘lymphocyte-like’ mononuclear cells to relatively mature megakaryocytes. In these latter cells, the cytoplasm was abundant and contained specific organelles and cytoplasmic blebs. Several characteristic features were observed during the maturation of the megakaryoblasts: (1) cells of the megakaryocytic lineage represented by microforms, (2) dysplastic platelets (giant, nucleated and agranular platelets), (3) the shedding of dysplastic platelets brought about by cytoplasmatic membrane inclusions and (4) naked nuc

ISSN:0001-5792    

DOI:10.1159/000205029

出版商:S. Karger AG    

年代:1990

数据来源: Karger

摘要:

An anti-idiotypic IgGlκ murine monoclonal antibody (MoAb) Y7 against purified monoclonal IgMλl, derived from a patient with Waldenström’s macroglobulinemia, has been generated. This antibody cross-reacted with the tumor-derived idiotypes of patients with B cell nonHodgkin’s lymphoma as measured by competitive inverse solid radioimmunoassay using unpurified serum samples. Our results with the inhibition curves of 10 sera of normal donors and 60 sera of lymphoma patients indicate that 21 lymphoma patients revealed cross-reactivities > 7%, the mean value observed in normal donors. Of these, 5 sera cross-reacted strongly, in the range of 43–163%, revealing a frequency of positive cross-reactivity for MoAb Y7 of 1/12 sera of lymphoma patients. The generation of a panel of anti-idiotypic antibodies which cross-react with different tumor-derived Ig in serum may be valuable for monitoring the disease in a high proportion of NHL

ISSN:0001-5792    

DOI:10.1159/000205030

出版商:S. Karger AG    

年代:1990

数据来源: Karger

摘要:

The classic analysis of metal transfer between ligands suggests that the metal-binding ligand (M-L1) and the uncomplexed ligand (L2) form a mixed complex (L2-M-L1), and that transfer is effected with the dissociation of this complex to L2-M and L1. Spectroscopic data suggested that such mixed complexes formed when pyrophosphate-Fe and acetohydroxamate-Fe were added to apotransferrin: the initial species had a different absorbance maximum than the final transferrin-iron complex. We now show that similar spectroscopic changes are seen when free ferrous iron or iron liganded to ATP, citrate or nitrilotriacetate are added to apotransferrin. The evolving spectrum on the addition of iron to apotransferrin may thus reflect iron binding per se rather than the formation of a mixed ligand complex.

ISSN:0001-5792    

DOI:10.1159/000205031

出版商:S. Karger AG    

年代:1990

数据来源: Karger

摘要:

Sixty transfusion-dependent thalassemic patients were studied by simultaneous measurement of circulating thyroid hormones, basal thyroid-stimulating hormone (TSH) and TSH response to thyrotropin-releasing hormone with the aim of evaluating the frequency of hypothyroidism in such patients, and the relationship between hypothyroidism and compliance with treatment and iron overload. Thyroid failure was present in 31 of the 60 patients. A correlation was found between impairment of thyroid functions, duration of chronic hypoxia and the activities of various transaminases. The results of this study emphasize the importance of early evaluation of thyroid function in thalassemic patients and suggest that anemia and hypoxia may potentiate the toxicity of iron deposition in endocrine glands.

ISSN:0001-5792    

DOI:10.1159/000205032

出版商:S. Karger AG    

年代:1990

数据来源: Karger

摘要:

Recent molecular biological investigations have shown that genetic information on the short arm of chromosome 17 is frequently deleted or inactivated in acute myeloid leukaemias and a number of solid tumours. In earlier studies we have found clonal occurrence of a 17p deletion (17p-) in patients with refractory anaemia. In the present study we investigated the time patterns of 17p- and morphological characters of bone marrow cells in 14 myelodysplastic patients with clonal 17p- who have been reinveststigated within 3- to 6-month intervals for 15–56 (median 37) months. We found that the 17p- frequency increases significantly with time. Further, the deletion is positively correlated with bone marrow myeloblast frequencies and negatively with those of maturing granulocytes (myelocytes and later forms). These results suggest that 17p- interferes with and delays granulocyte maturation. Inactivation or deletion of genetic material in 17p in myelodysplastic syndrome, acute myeloid leukaemia, and a wide variety of solid tumours further suggests that genes present on 17p may have a central position in malignant transformation of cells in a number of different tissue

ISSN:0001-5792    

DOI:10.1159/000205033

出版商:S. Karger AG    

年代:1990

数据来源: Karger

摘要:

The spectrum of β-thalassemia mutations in Malaysia has been determined in 45 β-thalassemia chromosomes using dot blot hybridization of the polymerase chain reaction amplified DNA and direct DNA sequencing. Eleven different molecular defects, including those previously detected in Chinese, Asian Indians, and American blacks, and a novel frameshift mutation causing β°-thalassemia were detected. Since this novel mutation, a T deletion in codon 15 creates a new restriction site for EcoRllenzyme; the mutation could be detected by EcoRll digestion of the appropriate amplified fragment. The results of the present study provide additional information on the molecular heterogeneity of β-thalassemia in this population. We also demonstrated the nonradioactive detection method of the β-thalassemia mutation based upon the digoxigenin-labeled oligonucleotide p

ISSN:0001-5792    

DOI:10.1159/000205034

出版商:S. Karger AG    

年代:1990

数据来源: Karger

摘要:

To study the capacity of peripheral blood mononuclear cells (PBMC) from patients with sickle cell disease to synthesize antibodies in vitro, the levels of IgM, IgG, and IgA were quantitated in supernatants of cultured PBMC from a group of asymptomatic adults with sickle cell disease and from normal controls. The rates of spontaneous synthesis of IgM were similar in nonstimulated cultures of PBMC from patients and controls, whereas the amounts of IgG and IgA produced spontaneously by nonstimulated lymphocytes from the patients were significantly greater than those from controls. Similar levels of IgM, IgG, and IgA were detected in the supernatants of cultures stimulated with pokeweed mitogen from patients and controls. Thus, the capacity of PBMC to respond in vitro to pokeweed mitogen was preserved in the patients. The enhanced spontaneous synthesis of IgG and IgA suggests the presence of chronic polyclonal activation of B cells and/or defective regulation of the production of antibodies.

ISSN:0001-5792    

DOI:10.1159/000205035

出版商:S. Karger AG    

年代:1990

数据来源: Karger

摘要:

We report a case of polyarticular group B streptococcal infection in an HIV-negative 46-year-old alcoholic with factor IX deficiency. Septic arthritis occurs infrequently in the hemophilic population despite their chronic joint disease; indications for diagnostic arthrocentesis in these individuals are discussed. The group B streptococcus often behaves as an opportunist in adults.

ISSN:0001-5792    

DOI:10.1159/000205036

出版商:S. Karger AG    

年代:1990

数据来源: Karger

摘要:

A 60-year-old man receiving chemotherapy for an intermediate-grade non-Hodgkin’s lymphoma developed multiple papuloerythematous cutaneous lesions. Alternaria alternata was cultured from the lesions, and hyphae were seen in biopsy specimens. This is an unusual infection, without a well-established treatment, in patients with lymphoma. The use of amphotericin B resulted in cur

ISSN:0001-5792    

DOI:10.1159/000205037

出版商:S. Karger AG    

年代:1990

数据来源: Karger

摘要:

A hemophilia B patient, seropositive for HIV antibodies since 1984, came to us in March 1989 with a severe necrotizing lesion of the nose. It was an erythematous lesion and looked like rhinophyma. Microbiological examination of the skin biopsy showed the presence of Cryptococcus neoformans. At the time of the study, the patient was in partial remission after 2 weeks of therapy with fluconazole per os 400 mg/day. He will be treated with the same therapy at maintenance dose (200 mg/day) for a long period.

ISSN:0001-5792    

DOI:10.1159/000205038

出版商:S. Karger AG    

年代:1990

数据来源: Karger