Biomedical subjects
Publications and source records attributed to A Najman.
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In November 1976, a 52 year old woman presented with a Moschowitz syndrome with clinical manifestations of continuous fever at 39 degrees and a transient Wernicke type aphasia. Laboratory findings included schizocytosis, a peripheral thrombocytopaenia and functional renal insufficiency. The ethanol tests was positive but there was no frank defibrination syndrome. After corticosteroid therapy failed, the patient was treated with Dipyridamole 400 mg/24 hours IV and acetylsalicylic acid 4 g/24 hours IV. Fever disappeared on the same day and the thrombocytopaenia was corrected in 48 hours. The patient was considered to be cured 15 days later. No precise aetiology to explain the Moschowitz syndrome was discovered apart from the recent ingestion of oestrogens. The authors emphasise the considerable progress which this use of a combination of Dipyridamole and aspirin represents, resulting in the cure of Moschowitz syndrome, a condition considered to be fatal up until a few years ago.
Gastro-intestinal involvement is a distinctive feature of non-Hodgkin's lymphomas. 31 cases are reported among 200 cases on NHL observed between 1960 and 1976. Multiple involvement appeared in 61%; a diffuse histological pattern is frequent (67%). The relapse of primary isolated gastro-intestinal localization (always) affected extra-digestive tissues (nodes, cavum). Chemotherapy is the mainstay of treatment COP, COAP and MOCA. Surgery is associated in localized involvement or in case of obstruction. High energy radiation therapy is indicated only in lymphosarcomas: -- to residual tumor after chemotherapy--in localized involvement diffuse on all the abdomen at 25 grays after surgery and a brief course of chemotherapy versus surgery and long course of chemotherapy alone.
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Sixty patients with non-Hodgkin's lymphomas were treated with a cyclophosphamide, vincristine, and prednisone (CVP) induction regimen, either alone (stage IV) or in combination with radiotherapy (stages I, II, III). The response rates for lymphocytic and histiocytic lymphomas were 82 and 86%. The complete remission (CR) rates were 66 and 71% with a median duration of 13 and 5.5 months respectively. Nodular types responded better than diffuse ones in both lymphocytic (CR rate 85% vs 45%; median duration 24+ months vs 2.5 months) and histiocytic lymphoma (CR rate 100% vs 0%). In lymphocytic lymphomas, survival in the responder group was 90% at 24 months vs only 20% in the nonresponder group (median survival 14.2 months). In the group with nodular lymphocytic lymphoma responding to therapy, there was a 100% survival rate at 24 months. The median survival for patients treated with chemotherapy alone (stage IV) and not responding to therapy, was 22 months vs 14.5 months in the whole nonresponder group (stages I, II, III, IV), suggesting a detremental effect of rediotherapy in the nonresponder group. In histiocytic lymphomas, the median survivals in the responder and nonresponder groups were 19 months and 3 months respectively. These results corroborate the excellent efficacy of the CVP regimen. They also indicate that, after CVP induction, 2 major prognostic factors are the histologic type and the nature of the response to therapy.
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60 patients with non Hodgkin's lymphomas were treated with a CVP induction regimen, either alone (stage IV) or in combination with radiotherapy (stages I, II, III). The response rates for lymphocytic and histiocytic lymphomas were 80 and 86%. The complete remission rates were 68 and 71% with a median duration respectively of 13 and 5.5 months. Nodular types did respond better than diffuse both in lymphocytic (CR rate 85% vs 44%; median duration 24 months +, vs 2.5) and histiocytic lymphoma (CR rate 100% vs 0%). In lymphocytic lymphomas, survival in the responding group was 90% at 24 months, vs 20% only in the non responding group (median survival 14.5 months). In the group with nodular lymphocytic lymphoma responding to therapy, there was a 100% survival rate at 24 months. The median survival for patients treated with chemotherapy alone (stage IV) and not responding to therapy, was 22 months, vs 14.5 months in the whole non responder group (stages I, II, III, IV) suggesting a detrimental effect of radiotherapy in the non responding group. In histiocytic lymphomas, the median survivals were respectively 19 months and 3 months in the responding and non responding groups. These results corroborate the excellent efficacy of the CVP regimen. They also indicate that, faced to a CVP induction, 2 major prognosis factors are the histologic type and the nature of the response to therapy.
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The molecular heterogeneity of congenital pyruvate kinase deficiencies becomes apparent from the results of immunological studies. In one case, a quantitative defect is plausible; in the second case, the most likely hypothesis is a molecular alteration of the binding site for the activator, with preservation of the antigenic specificity; in the third case an abnormal protein, extremely unstable and devoid of antigenic reactivity, carries catalytic activity. In no case can any cross-reacting material be detected.
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