Compressive myelopathy. An unusual presentation of B-thalassaemia intermedia.
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Biomedical subjects
Publications and source records attributed to G Garewal.
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Deoxyuridine (dU) suppression test (i.e. ability of exogenous dU to suppress the incorporation of subsequently added 3H-thymidine into DNA) and the incorporation of 3H-thymidine (3H-TdR) alone without dU were studied in bone marrow cultures from 10 patients with erythroleukaemia, 10 patients with vitamin B12/folate-deficient megaloblastic anaemia and 10 haematologically normal subjects. Despite morphological resemblance between megaloblastosis in erythroleukaemia and nutritional megaloblastosis, the dU suppression values in erythroleukaemia were within normal range in contrast to abnormal dU suppression in vitamin B12/folate-deficient megaloblastic bone marrows. The incorporation of 3H-thymidine alone was significantly lower in erythroleukaemia than in normal or vitamin B12/folate-deficient megaloblastic bone marrows. Autoradiographic studies showed that 3H-TdR labelling indices as well as mean grain count (MGC) of basophilic and polychromatic erythroblasts were significantly lower in erythroleukaemia than in normal or vitamin B12/folate-deficient bone marrows. The reduced incorporation of 3H-TdR in erythroleukaemia erythroblasts was probably not due to deficiency of the salvage pathway enzyme, thymidine kinase, since MTX (10(-5) M) which blocks the de novo pathway of thymine-DNA synthesis, enhanced the incorporation of 3H-TdR into erythroblasts in erythroleukaemia as well as in normal bone marrows. A high intracellular pool of thymidine-triphosphate (dTTP) due to defective DNA synthesis may allosterically inhibit thymidine kinase and 3H-TdR incorporation.
Sea-blue histiocytes are described in the bone marrow of a 15-year-old male patient suffering from idiopathic thrombocytopenic purpura. Cytochemically the storage material in these cells appeared to be glycophospholipid. Lipid analysis of blood leucocytes and nucleated cells of bone marrow showed a significant increase in the phospholipids, triglycerides and cholesterol as compared to age-matched controls. Serum lipid analysis showed a mild increase of phospholipids as compared to age-matched controls.
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The percentage of T-cells was studied in pregnant patients with a previous history of spontaneous abortions, in patients without such a history and in nonpregnant controls. The percentage of T-cells remained unaltered during the three trimesters of normal pregnancy. In patients with a previous history of spontaneous abortions, the T-cell percentages were lower in the second and third trimesters than in non-pregnant controls, but the values did not differ statistically from corresponding values in those patients with no previous history of spontaneous abortion (normal pregnancy). The phytohaemagglutinin (PHA)-induced transformation of lymphocytes was depressed in the second and third trimesters of both patients with a normal pregnancy and those with a previous history of spontaneous abortions. Thus, while the percentage of T-cells remained unaltered during pregnancy, their response to PHA falls in the second and third trimesters.
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Ten children with megaloblastic anemia and a hemorrhagic diathesis are reported. Four of them had life-threatening bleeds necessitating an emergency blood transfusion. Seven had platelet counts of less than 30,000/cu mm, and nine had hemoglobin values of less than or equal to 5.2 g/dl at initial presentation. Deficiency of vitamin B12 was more frequently encountered. Response to therapy was excellent and hemostasis was possible within 12 to 24 hours of initiating treatment.
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The clinical and hematological characteristics of ten children with myelodysplastic syndromes diagnosed and followed up over a 3 year period are presented. All of them had anemia and a low platelet count whilst the white blood cell count was variable. Presentation with bilateral proptosis and acute febrile neutrophilic dermatosis (Sweet's syndrome) were unique features observed in one case each. None of these cases could afford specific therapy and thus serve to illustrate the natural history of the disease in pediatric practice.