Frequency of specific coagulation inhibitors and antiphospholipid antibodies in Tunisian haemophiliacs.

Production of factor VIII or factor IX inhibitors is a major complication limiting the efficiency of substitutive therapy in haemophiliacs. Moreover, viral infections, the second serious complication of replacement therapy, may be associated to the occurrence of antiphospholipid antibodies which paradoxically lead to thrombosis. We investigated the prevalence of coagulation inhibitors (factor VIII and factor IX inhibitors, antiphospholipid antibodies) in Tunisian haemophiliacs, and we assessed concomitant coagulation factor deficiencies. Thirty-two previously treated haemophiliacs (20 haemophiliacs A; 12 haemophiliacs B) were screened for factor VIII and factor IX inhibitors by APTT mixing study, Bethesda test and modified Nijmegen method, and investigated for the presence of anticardiolipin, anti-ß2 glycoprotein I, lupus anticoagulant and associated coagulation factors deficiencies. The frequency of factor VIII and factor IX inhibitors was low (5%) in contrast to the high prevalence of antiphospholipid antibodies (28.1%). Four and nine patients were positive for anticardiolipin and anti-ß2 glycoprotein I, respectively. No lupus anticoagulant was detected. The prevalence of antiphospholipid antibodies was higher in patients with positive hepatitis C virus infection serology as compared to patients with negative serology (41.6% vs. 20%). Concomitant factor VII and/or factor V deficiency was found in 10 patients. In conclusion, the occurrence of factor VIII and factor IX inhibitors is rare among Tunisian haemophiliacs. The clinical relevance of antiphospholipid antibodies requires further investigations. We emphasize the importance of screening for concomitant deficiencies in haemophiliacs when the clinical presentation is suggestive.

Platelet and granulocyte alloimmunisation in multitransfused Tunisian patients.

We determined the frequency of post-transfusion alloimmunisation against platelet and granulocyte antigens in 51 Tunisian polytransfused patients with haematological diseases. Serum samples were analysed by a standard and an antiglobulin-augmented lymphocytotoxicity technique, a granulocyte agglutination test, a granulocyte immunofluorescence test, a platelet immunofluorescence test and the monoclonal antibody-specific immobilisation of platelet antigens assay. No granulocyte-specific antibodies were detected. HLA antibodies were found in 58.8% of patients. Platelet-specific antibodies were detected in four patients and were directed against human platelet antigen (HPA)-5b, HPA-1b and HPA-3a. The three patients with Glanzmann's thrombasthenia developed anti-GPIIb/IIIa antibodies. This study provides immunogenetic information that could improve the management of transfusion therapy in Tunisia.

[Adult primary myelodysplastic syndromes. Report of 36 cases]. / Syndromes myelodysplasiques primitifs de l'adulte. A propos de 36 cas.

Between 1989 and 1999, 36 cases with primary myelodysplastic syndromes were diagnosed. They were 15 male and 21 females, the median age was 62 years (range: 22 to 90 years). Eighty one per cent of patients were presented symptoms of anemia. Lymphadenopathy, splenomegaly and skin manifestations were noted in 25% of cases. Hemogram showed anemia, leucopenia and thrombocytopenia respectively in 97%, 44% and 55% of cases. Refractory anemia with excess blasts (AREB) is the most frequent FAB subtypes of MDS (17 cases). Cytogenetic study concerned 24 patients. In 13 cases the karyotype was pathological with deletion 5 q in 64% of cases. Seventeen patients have received a chemotherapy. Survival rate to 36 months is 11%. At the time, the only curative treatment is the bone marrow transplantation, which is proposed to young patients with HLA identical donor.

[Primary non-Hodgkin's lymphoma of the thyroid. A case report and review of the literature]. / Lymphome non hodgkinien primitif de la thyroïde. A propos d'une observation et revue de la littérature.

The authors report an observation of a non-Hodgkinien primitive lymphoma of the thyroid, developed on Hashimoto's thyroiditis. The 61 years old woman was operated on a total thyroidectomy for a multinodular goiter with dysphagia. The non-Hodgkinien high-grade lymphoma infiltrated the perithyroidal tissues. The extension' assessment was negative and the lymphoma was classified stage IE. The treatment was supplemented by 6 cures of standard chemotherapy CEOP. Based on this observation and on a literature' review, we will discuss the clinical and therapeutic characteristics of this thyroid cancer.

[Neoplastic manifestations of HIV infection]. / Les manifestations neoplasiques au cours de l'infection VIH.

The incidence of malignancies has increased in conjunction with epidemic of human immunodeficiency virus (HIV) disease and they are currently considered acquired immunodeficiency syndrome (AIDS)-defining conditions. Approximately 40% of all patients with AIDS have developed cancer during the course of HIV infections. Further, as survival has improved in HIV disease, the incidence of these malignancies has increased. The main malignancies noted are Kaposi's sarcoma, non-Hodgkin's lymphoma, Hodgkin's disease, rectal and anal cancer.

[Epidemiologic, clinical and cytohematologic characteristics of adult acute lymphoblastic leukemia in Tunisia]. / Caracteristiques epidemiologiques, cliniques et cytohematologiques des leucemies aigues lymphoblastiques de l'adulte en Tunisie.

Through a national retrospective study, the authors report the clinical and hematological characteristics of 124 acute lymphoblastic leukemia of the adult diagnosed during 5 years (1993-1997). The national prevalence is of 0.28/100.000 inhabitants/year. The sex-ratio is of 1.3. Sixty six per cent of patients were 16-35 years of age, and only 10% of them were more than 60 years of age. A tumoral syndrome was present at 71% of the cases with peripheral adenopathies in 55%, splenomegaly in 40%, hepatomegaly in 19% and a mediastinal tumor in 18% of the cases. The bone pain were rarely signaled (10%) and neuro-meningeal affection was found in only 3% of cases. There was no testicular lesions. The white blood cells count was less than 30.000/mm3 in 60% whereas an important hyperleucocytosis superior than 100.103/mm3 was observed in 20% of the cases. Anemia and thrombopenia were noted in 94% and 90% of the cases respectively. Acute lymphoblastic leukemia typing by cytological study of Bone marrow according to the Fransh-American-Britain criteria (FAB) had found 43%, 48% and 4% for type 1,2 and 3 respectively. In 5% of the cases the type of the acute lymphoblastic leukemia was not precised (diagnosis based on the Bone biopsy).

[Primary renal non-Hodgkin lymphoma]. / Lymphome non-Hodgkinien rénal primitif.

Primary renal non Hodgkin lymphoma is rare and unusual because the renal parenchyma does not have lymphatics. We report a case of bilateral primary lymphoblastic T lymphoma presenting with renal insufficiency in a 14 year old girl. We discuss clinical, pathological particularity and prognosis.