Biomedical subjects
Publications and source records attributed to B Sztern.
In case of anaemia, the author insists on the three key tests, hemoglobin measurement, mean corpuscular volume evaluation and reticulocyte count determination. In the general practice setting, the more frequent causes of anaemia are iron, B12 and folate deficiencies.
Hereditary spherocytosis (HS) is a congenital hemolytic anemia which affects one person out of 5000 in Northern Europe. HS is caused by defects of the red cell membrane proteins involving mainly ankyrin or band 3, and less frequently, protein 4.2 or spectrin. The reduction of red cell osmotic resistance and the recognition of spherocytes on the peripheral blood smear are the primary laboratory tests necessary to evocate a diagnosis of hereditary spherocytosis. Analysis of the red cell membrane proteins using polyacrylamide gel electrophoresis is used to quantify individual proteins and to identify the protein defect related to a diagnosis of HS. Samples from 47 patients and 25 controls were studied by electrophoresis of the red cell membrane proteins. Protein deficiencies related to HS were demonstrated for 21 patients. In 4 other cases, abnormalities of membrane proteins unrelated to HS were also demonstrated. Electrophoresis of the red cell membrane proteins allows the identification of the protein deficiency related to HS and thus confirms the diagnosis of HS, but also points to the underlying molecular defect, the inheritance pattern and the clinical aspects of the disease.
We report an unusual observation of Yersinia enterolitica (YE) bacteremia with subcutaneous with subcutaneous abscesses on the scalp and, by gradual extension, cerebritis in a diabetic patient. All clinical signs disappeared after surgical drainage of the abscesses and protracted antibiotherapy. The well-known affinity of YE for iron led us to demonstrate an unrecognized hemochromatosis.
Idiopathic acquired sideroblastic anemia is not a common disease. We studied the renal tubular function in such patients. Patients have lower calcium than controls (8.46 mg/dl +/- 0.59 vs 9.16 +/- 0.53). We have highlighted the multiple renal anomalies observed in patients with this type of hemopathy. The serum phosphate levels are lower in patients than in controls (2.73 +/- 0.36 vs 3.3 +/- 0.55 mg/dl, p = 0.0048). We found higher glycosuria (21:43 +/- 42.58 vs 0.0 +/- 0.0 mg/dl), total aminoaciduria (6280 +/- 3943 vs 4138 +/- 2269 microMol/g creatinine, p = 0.19) and lower maximum capacity for phosphate reabsorption by the renal tube (TmPO4) (2.11 +/- 0.38 vs 2.9 +/- 0.73 mg/100 ml GFR, p = 0.0027) in the patients. The association between idiopathic acquired sideroblastic anemia and the multiple tubular anomalies corresponding to the syndrome initially described by Fanconi has not been reported to date. The underlying mechanism is not understood, but, taken separately, these two anomalies commonly present heme metabolic anomalies in the mitochondria. We hypothesize that this syndrome could represent the clinical expression of a mitochondrial cytopathology.
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A case of chronic lymphocytic leukemia (CLL) is described during the course of which paraplegia appeared caused by epidural compression of the tissue by leukemic cells. This complication in CLL is rare. The disorders of the nervous system during the course of leukemia are summarized, and, in particular, the circumstances pertaining to the occurrence of paraplegia during chronic lymphocytic leukemia.
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We report a case of a double M component appearing in the course of angioimmunoblastic lymphadenopathy with dysproteinemia (AILD). Lymphocyte function, especially the decrease in T-suppressor cells, could play an essential role in the mechanisms of the disease. In the presence of an M component, the prognosis for AILD seems to be more pessimistic. Arguments for considering AILD as a premalignant disease are reviewed.
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Seven patients with recurrent brain gliomas were treated by a single dose of CCNU 390 mg per m2. In five cases, chemotherapy was followed by autologous bone marrow transfusion containing 1.5 to 3 X 10(8) nucleated cells, 2.8 to 18 X 10(4) clusters plus colonies and 0.4 to 5 X 10(4) colonies forming cells per kg of body weight. Two patients were not grafted. None of these patients showed a clear cut response to the treatment as judged by clinical improvement and changes of the brain CT-scan. In 3 patients blood toxicity occurred early and was severe. In 4 others, it was milder and delayed. The duration and the severity of blood toxicity were modified by bone marrow transfusion but only slightly.
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