[Isolated extramedullary adnexal relapse of acute lymphoblastic leukemia: a case report]. / Rechute extramédullaire annexielle isolée d'une leucémie aiguë lymphoblastique : à propos d'un cas.

The occurrence of an isolated ovarian or pelvic relapse of acute lymphoblastic leukemia (ALL) in complete remission after chemotherapy has rarely been described. We report the case of a 12-year-old girl, treated for ALL, who developed an isolated left ovarian and fallopian tube localization without medullary or blood relapse 4 years after the end of the initial treatment. She presented with an isolated pelvic mass. The diagnosis was established by a CT-guided biopsy. The treatment consisted of a second course of chemotherapy and complementary surgery; a second complete remission was obtained.

[Evaluation of a prospective therapeutic protocol in adult patients with nonsevere aplastic anemia in South Tunisia]. / Evaluation d'un protocole thérapeutique prospectif des aplasies médullaires acquises non sévères de l'adulte dans le Sud tunisien.

INTRODUCTION: Immunosuppressive drugs are usually used in the treatment of acquired aplastic anemia (AAA). The aim of this study was to evaluate the efficiency of a prospective therapeutic protocol using cyclosporine and androgens in the treatment of adult patients with nonsevere AAA. METHODS: Twenty-nine patients diagnosed and treated at the University Hospital of Sfax (Tunisia), during a 10-year period (1991-2000) were included. In addition to symptomatic treatment (transfusion, antibiotics), all the patients received a specific treatment including two drugs: cyclosporine 5mg/kg per day and androgens 0,5mg/kg per day. RESULTS: The response rate at three, six and 12 months were 48, 75, and 87%, respectively. Survival rate was 52% at one year, and 37% at five and 10 years. The main toxicities were hepatic, renal, and hypertension, observed in 53, 16 and 15%, respectively. These toxicities were reversible in 65, 87 and 100% of the cases, respectively. CONCLUSION: The response and survival rates in our series are quite satisfactory when compared to those obtained with other immunosuppressive drugs (cyclosporine and antilymphocyte serum) in the literature. The addition of androgens in our patients seemed to potentiate the immunosuppression induced by ciclosporin, but secondary toxic effects were more common.

[Congenital factor XIII deficiency in the south of Tunisia]. / Déficit congénital en facteur XIII de la coagulation dans le sud tunisien.

Factor XIII deficiency is a rare autosomal recessive congenital disorder of haemostasis characterised by a plasmatic factor XIII level less than 1% in homozygote and bleeding as of the youth. We report a study about ten patients with congenital factor XIII deficiency from seven south-Tunisian families, there are seven females. Umbilical bleeding was common and only two patients had intracranial bleeding. The standard screening tests are normal. Factor XIII activity was less than 1% in all patients. A sub-unit A deficit was detected for the ten patients. Out hemorrhagic context, five patients receive regular prophylactic transfusion with fresh frozen plasma.