PubMed Health⌕ Search

Biomedical subjects

J Tanzer

Publications and source records attributed to J Tanzer.

At least 37 records · Page 2Linked to original sources
PubMed1991

[Prognosis of chronic myeloid leukemia].

The prognosis of chronic myelocytic leukemia is a current topic owing to the new treatments that have been proposed for this malignant blood disease which, a few years ago, was lethal within 3 to 4 years due to the inescapable occurrence of the terminal acute transformation. Beside bone marrow allograft, which is known to have cured a non-negligible number of patients, the most recent use of interferons offers a reasonable therapeutic alternative to those patients who cannot be allografted. The identification of prognostic variables at the time of diagnosis has become necessary for a better determination of therapeutic indications. The analysis of large published series has made it possible to construct mathematical models which have proved efficient and have been confirmed by prospective studies. The value of new techniques, such as molecular biology, to refine the mathematical models remains to be demonstrated.

Actuarial Analysis↗
PubMed1991

Recurrent ctb(7)(q31.3) and possible laminin involvement in a neonatal cutis laxa with a Marfan phenotype.

A 6-week-old girl presented with cutis laxa, emphysema, heart anomalies and a diaphragmatic hernia. She died at 22 weeks. A recurrent ctb(7)(q31.3) was found and the laminin gene was suspected to be involved in the disease. Anti-human laminin antiserum showed that this protein was absent from the skin. This case, together with 17 other similar cases, could represent a new type of connective tissue disease.

Cells, Cultured↗
PubMed1991

Two additional cases of isochromosome 21q or translocation 21q21q in hematological malignancies.

We report on 2 patients with isochromosome 21q [i(21q)] or translocation 21q21q [t(21q21q)] in myeloid disorders. Of 18 available cases of i(21q) or t(21q21q), 15 were found in myeloid malignancies, often secondary to a previous carcinogen exposure. Complex karyotypes were found in most cases. Four cases presented with i(21q) or t(21q21q) as the sole anomaly, and this might represent a specific entity.

Aged↗
PubMed1991

Nosocomial legionnaires' disease in a bone marrow transplant unit.

We report the outcome of nosocomial legionnaires' disease in three patients who were isolated in the same sterile unit after allogeneic bone marrow transplantation. In all three cases the disease presented with dramatic pulmonary symptoms, and diagnosis was ascertained by direct immunofluorescence on bronchoalveolar fluids. None of the patients underwent seroconversion. This report draws attention to: (1) the fact that bacteriological filters do not ensure absolute security; (2) the need for frequent monitoring of the two factors governing legionella growth, water temperature and chlorination; and (3) the effectiveness of quinolones as a curative and prophylactic treatment of legionnaires' disease in transplanted patients avoiding pharmacological cyclosporin interaction.

Adult↗
PubMed1991

[Early acroparesthesia in females: a sign disclosing heterozygote Fabry disease].

Heterozygous Fabry's disease has an inconstant expression and very few complications. The theory of X-chromosome inactivation which, according to Lyon, occurs hazardly, is illustrated by the fact that the disease is expressed even in hemizygous women. Ophthalmic manifestations, as detected by the slit lamp method, are almost constant, 80 p. 100 of women with the disease having a verticillate cornea. Angiokeratoma is present in 20 p. 100 of the cases. Episodes of paraesthesia of the hands and feet are less common; in most cases they are attributed to the disease retrospectively, during family investigations. In two girls aged 10 and 11 years respectively and without history of Fabry's disease the only symptom suggestive of the diagnosis was paroxysmal acroparaesthesia. In one of the girls acroparaesthesia was associated with acrocyanosis, livedo and acro-osteolysis, but concordance was the only argument in favour of a link with Fabry's disease. Alterations of the extremities have been reported in this disease, including palmar erythema and a bluish discoloration of the palms due to dilatation of the superficial veins. Only two cases of livedo have been published. Acrosteolysis has never been documented in Fabry's disease, and its presence must be confirmed in further cases. The diagnosis of heterozygous Fabry's disease in these 2 girls was confirmed by the finding of ceramide trihexoside in urine and by leucocyte alpha-galactosidase levels that were 25 to 30 p. 100 of values obtained in controls. A study of the family of one of the girls showed that the father was involved; this hemizygous type of the disease with a 10 p. 100 alphagalactosidase level was totally asymptomatic.(ABSTRACT TRUNCATED AT 250 WORDS)

Acrodermatitis↗
PubMed1991

Soluble CD23 displays T-cell growth enhancing activity.

Soluble CD23 (sCD23) enhances, in a dose-dependent manner, the number of secondary T-cell colonies generated by peripheral blood-derived agar T-colony cells in the presence of phytohaemagglutinin (PHA) and interleukin-2 (IL-2). This effect is not affected by IL-1 or IL-4 but is abolished by an anti-CD23 monoclonal antibody (mAb) or by IgE. No colonies were observed when sCD23 was added to PHA- or IL-2-free cultures. sCD23 also enhanced the cloning frequency of primary T-colony cells in a limiting dilution assay. These data provide the first direct evidence that sCD23 recruits T-cell clones in peripheral blood-born T cells and may be involved indirectly in the regulation of IgE response.

Antigens, Differentiation, B-Lymphocyte↗
PubMed1991

Chronic myelocytic leukaemia with unusual (27 years) complete remission terminating in acute undifferentiated leukaemia: a clinical and karyotypic study.

A case of clinically typical CML (300 x 10(6)/l leukocytes, 400 x 10(6)/l platelets, splenomegaly) is presented. After complete remission induced by busulphan, no clinical or haematological abnormalities were observed for 27 years until the development of acute leukaemia (type M1), which was rapidly fatal after a brief chemotherapy-induced remission. The cytogenetic findings were also original: no chromosome Ph1 (during remission 3 years after the onset of the disease), no translocation (banding study 5 years later), and no bcr/abl rearrangement (during the terminal phase).

Busulfan↗
PubMed1990

Cytogenetics and molecular analysis in chronic myelogenous leukemia patients treated by interferon and chemotherapy.

A new combination regimen including chemotherapy and interferon was started in 1986 for the treatment of 24 chronic myelogenous leukaemia patients in chronic phase. Complete cytogenetic remission were noted in 10 patients and 6 of them are in sustained remission. The overall survival was 70% at 40 months. However, a minimal residual disease was detected in the 3 patients tested with the polymerase chain reaction. In addition no correlation was detected between the site of the breakpoint and response to therapy.

Adult↗
PubMed1990

Two additional cases of t dic(9:12) in acute lymphocytic leukemia (ALL): prognosis in ALL with dic(9:12).

We report two occurrences of dic(9;12) in acute lymphoblastic leukemia and review previous cases. Cases of dic(9;12) share common features with cases of 9p and 12p rearrangements, but prognosis seems particularly good in cases of dic(9;12). The persistence of a specific dicentric in stable clones is remarkable and points to unusual centromeric behavior and/or marked selective advantage of the anomaly.

Adult↗