[Partial splenectomy for hematological indication. Apropos of 40 cases].
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Biomedical subjects
Publications and source records attributed to A Khelif.
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Nineteen cases of B Thalassemia have benefited from partial splenectomy at the General Surgery Service of Farhat Hached Hospital in Sousse (Tunisia). The partial splenectomy indication was to reduce hypersplenism, thus transfusion needs, to suppress splenic pain and to conserve a splenic remnant, which preserves patients' immunity. The operation was in reality a subtotal splenectomy keeping the lower pole in all cases. We had no per-operatory complication. The preoperatory bleeding was not more serious than in total splenectomy. In all the patients, we noticed reduction of about half the transfusion need, except one who had also a chronic deficit in glyco-six phospho-dehydrogenase. The average hemoglobin rate increased from 60 g/L in the pre-operatory to 80 g/L after the operation. Consequently, this reduction of transfusion needs results in the decrease of the hemochromatosis, which is one of the main complications of hypertransfused thalassemia.
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Primary tumors of the vertebral column are rare: 20 per cent of all primary tumors of the spinal column. The distribution by type of tumor shows that the three most frequent primary tumors considered to be "radio and/or chemo resistant lesions" are the chordoma (15.5 to 24.5%), the chondrosarcoma (20%) and the giant cell tumor (10%). A second group with "chemo and/or radiosensitive lesions" include the Ewing sarcoma, primary lymphoma and plasmocytoma (5%). We consider a third group with the benign tumor: osteochondroma, chondroma, osteoid osteoma, osteoblastoma, aneurysmal bone cyst, hemangioma and eosinophilic granuloma (2 to 3%). The last tumoral group agrees with sarcomatous tumors: osteogenic sarcoma, fibrosarcoma, malignant fibro-histiocytoma, angiosarcoma and hemangio-pericytoma (1 to 3% of primary tumors of the vertebral column); they are most frequently secondary to Paget's disease, giant cell tumor or to radiation therapy and their prognosis is poor.
Complete surgical excision of a primary spinal tumor without contamination of the surgical wound is not always possible, and adjuvant therapy by chemotherapy and/or radiation therapy is important to improve local and survival disease free. The efficacy of radiation therapy is limited above L2 because of the risk of radionecrosis of the spinal cord if one exceeds 45 Gy over 4.5 weeks. According to drugs and radiation sensitiveness, three groups can be distinguished: i) malignant tumors with low sensitiveness: chordoma, osteo-, chondro-, and fibro-sarcoma; ii) malignant tumors with high sensitiveness: Ewing's sarcoma, plasmocytoma, and non Hodgkin lymphoma; iii) benign tumos, occasionally aggressive like giant-cells tumor. For each type of tumor, a literature review and our personal experience is presented.
A higher incidence of hemoglobinopathies in Central Tunisia is probable in view of the socioeconomic conditions of the high risk population, frequency of consanguinity and decreased early death in children. Given our limited therapeutic possibilities in severe forms, we have undertaken a community prevention program based on genetic counselling.
A 22-year-old woman had an idiopathic pure red cell aplasia that failed to respond to high doses of corticosteroids. After a series of 10 plasma exchange procedures, bone marrow erythropoiesis and reticulocyte blood count returned to normal; the haematological remission has been now persistent for 12 months. The place of plasma exchange in the management of pure red cell aplasia and its mode of action will be discussed.
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A complete blood analysis with a careful morphologic examination of peripheral blood and bone morrow smears completed by cytochemical reaction will help to classify the most acute myeloid leukaemia (AML). Actually, the study of other cytogenetis and immunophenotypic markers are now necessary to confirm diagnosis. The World Health Organisation WHO classification (2001) incorporates theses approaches. The purpose of this study is a bio-clinical review according to the WHO recommendations in 153 cases of LAM diagnosed between January 1998 and December 2003. The patients were aged 2 months to 90 years with sex ratio (M/F) of 1,22. The morphologic conclusion was difficult in 12% cases. Presence of dysplasia is noted in 50% of cases with multilineage dysplasia in 42% of cases. Our results showed cloned chromosomal abnormalities in 57% of cases (t(8;21): 12%, t(15;17) : 10%, Inv16: 1,3%, 11q23: 2,6% et complex karyotype: 14,3%). In 69% of cases with multilineage dysplasia, the karyotype was normal. 3 cases of LAM were noted at patients treated for breast cancer with chirurgic chemotherapy and radiotherapy 3, 4 et 5 years after treatment (LAM3 with t(15;17), LAM4 with genetic abnormalities of chromosomes 3, 5, 7, 8, 9, 14 et 16 et LAM 6 with genetic abnormalities of chromosomes 4, 7, 12, 14, 19 et 21). In WHO classification, cytology is essential in diagnosis of LAM even if the karytype have an important prognostic value. Research of signs of dysplasia lineage after lineage constitutes an important microscopic work and it is difficult to quantify dysplasia when the lineage is poor.