[Hemoglobin O Arab: about 20 cases]

Hemoglobin O Arab is a rare abnormal hemoglobin. We report the Clinical and biological features of this disease 20 patients: 16 were compound heterozygous Hb O Arab/Beta thalassemia and 4 homozygous Hemoglobin O Arab. Patients are 7 men and 13 women. Most of them are originated from the North West of Tunisia with a age average of 39. 7 years. Diagnosis was carried out at a relatively old age [26. 9 years old]. The homozygous form was not very symptomatic. The compound heterozygous form was more severe and characterized by a mild form of thalassemia with a moderate microcytic hypochromic anaemia [Hb =8. 8 g/dl]. It was often complicated of thrombopenia due to hypersplenism in 40% of the cases. Treatment was based on occasionally transfusion and splenectomy on event of hypersplenism. Evolution of this disease was generally good with a long lifespan of patients. Haemoglobin O Arab is an abnormal hemoglobin well tolerated except for heterozygous category which requires iterative transfusions. Spelenectomy is indicated in case of hypersplenism. The evolution is generally good with a long survival

[Alpha interferon in children with philadelphia chromosome-positive chronic myeloid-leukaemia]

The present work focuses on the therapeutic efficacy and the toxicity of alpha interferon in patients younger than age 18 years. 5 patients younger than 18 years were treated and followed up between 1990 and 1999 at the department of haematology [Aziza Othmana Hospital] Hydroxyurea was given as initial treatment to all patients. After a median period of 8 months. these patients received alpha interferon [5 millions units/m 2 once]. Six months after the beginning of the alpha interferon a complete hematologic response was obtained in all patients. The median overall survival was of 66 months: 3 patients are still alive [2 patients in an advanced stage and one patient in chronic phase] and 2 patients died after transformation. The most common reported side effects of alpha interferon were asthenia, weight loss, fever, myalgia, chills and headaches - these toxic manifestations were mild and were noticed in all our patients. MyeIosuppression was noted in two patients. Interferon is well tolerated in patients younger than age years 18 old, with CML.It may offer an alternative to bone marrow transplantation in children in the chronic phase of CML without histocompatible donor. The role of new agents such as STI 571 needs to be evaluated as well

prise en charge des thrombopenies severes au cours de la grossesse et de l'accouchement. A propos de 14 cas et une revue de la litterature

Thrombocytopenia occured in 14 of 5557 pregnant women [0,25%] in our hospital over 4-year interval. The patients with thrombocytopenia could be devided into two groups' healthy women 2 patients [14,28%] hypertensive patients and patients with immune thrombocytopenia 12 patients [85.72%]. Cesarian section rate for delivery was 35,7%. Six foetal loss were observed. Foetal morbidity is represented by prematurity and low weigh at delivery [3 cases] and in one case cerebral bleeding was observed.

[Long term survival and prognostic factors in multiple myeloma treated with conventional chemotherapy. About 109 cases]

Our study is retrospective. We report the results of conventional chemotherapy in previosly untreated patients with myeloma. Survival and progonstic factors were analysed in 109 patients diagnosed from 1983 to 1992. The median age was 65 years, 87 patients [80%] were including in the stage III according the Durie Salmon staging system. The median survival time was 27 months and 10 years survival rate is 3,66%. In the univariate analysis, two prognostic variables were retained namely the hemoglobin and creatinine level. The study suggest that conventional therapy is agood treatment for old patients. However, patients younger than 55 years, must benefit from intensive chemotherapy supported by autologus bone marrow, pheripherol blood stem cells, or allogenic bone marrow transplantation. A considerable encrace in duration of remission and survival is possible