A retrospective study of leukemia epidemiology in Northern Tunisia.

A hospital-based epidemiological study of leukemia was carried out in the northern part of Tunisia during a 5-year period, from 1999 to 2003. Of 402 Tunisians diagnosed with leukemia, 344 (85.6%) had acute leukemia and 58 (14.4%) had chronic leukemia. Age-specific incidence rates for acute lymphoid leukemia (ALL), acute myeloid leukemia (AML), chronic lymphoid leukemia (CLL) and chronic myeloid leukemia (CML) are described. The distribution of leukemia in the governorate of Nabeul was established. These results are useful for the organization and follow-up of medical care.

Identification of new markers discriminating between myeloid and lymphoid acute leukemia.

BACKGROUND: The heterogeneity of acute myeloid leukemia (AML) with respect to biology and clinical course resides in the fact that patients belonging to the same group show marked differences in their response to chemotherapy, necessitating a refinement of AML classification. METHODS: In order to define molecular markers for AML, we performed microarray analysis on peripheral blood cells from two M5 AML patients, and selected four differentially expressed genes to validate their expression by real-time quantitative PCR (RT-PCR). RESULTS: We have shown that two downregulated genes in AML, those encoding guanine nucleotide-binding protein gamma11 (GNG11) and amphiregulin (AREG), are also downregulated in B-lineage acute lymphoblastic leukemia (B-ALL) and T-lineage acute lymphoblastic leukemia (T-ALL) patients. A second gene, that encoding ceruloplasmin (CP), is upregulated in AML but not in B-ALL and T-ALL. The level of expression of these genes varies from one patient to another. CONCLUSION: Since the number of patients studied is limited, further studies are needed with a larger series of patients to evaluate the potential utility of GNG11, AREG and CP as molecular markers for AML subtype classification. Our study is the first to analyze these genes in AML, B-ALL, T-ALL and chronic leukemia (myeloid and lymphoid) patients by RT-PCR. This rapid and sensitive method could be used to screen these genes in different types of leukemia.

[Hemoglobin C disease: report of 16 Tunisian cases]. / L'hemoglobinose C: a propos de 16 cas Tunisiens.

AIM: was to provide the clinical and biological patterns hemoglobine disease in Tunisia. METHODS: This retrospective study collected to 16 cases of hemoglobin C disease : 6 homozygotic Hb C and 10 heterozygotic Hb C/beta-thalassemia. RESULTS: The clinical profile is characterized by mild hemolytic anemia (Hb = 11.7 g/dl) associated with splenomegaly and hypersplenism. Contrary to homozygous state, the Hb C/beta-thalassemia is associated with microcytosis and pseudopolycythemia. The diagnosis is based on target cells, specific intraerythrocytic Hb C crystals in blood smear and Hb C level at 100%. CONCLUSION: The Hb C disease must be considered as a benign hemoglobinopathy which is associated with a long survival without major complications.

[Cytology and immunophenotype feautures of 80 acute myeloid leukemia]. / Caracteristiques cytologiques et immunophenotypiques de 80 leucemies aigues myeloides.

Acute myeloid leukemia (AML)'s diagnosis is clinical and biological. We report here 80 AML with cytology and immunophenotype features to establish correlations. 21 AML1, 23 AML2, 12 AML3, 2 AML4, 18 AML5 and 3 AML6 were diagnosed by cytology. Only one case of AML0 was diagnosed by immunophenotype. Myelogysplasia is present in 29.8% cases. CD19 and CD56 expression was significantly associated to AML +t(8;21). Additionally, concomitant negativity of CD34 and HLA-DR was discrimininatif to AML3 diagnosis. Prognostic value to expression some CD needs time backwards.

[Hemoglobin O Arab: about 20 cases]. / L'hemoglobine O Arab: etude à propos de 20 cas.

BACKGROUND: Hemoglobin O Arab is a rare abnormal hemoglobin. AIM: We report the Clinical and biological features of this disease METHODS: 20 patients.:16 were compound hétérozygous Hb O Arab/Béta thalassemia and 4 homozygous Hemoglobin O Arab. Patients are 7 men and 13 women. RESULTS: 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. CONCLUSION: Haemoglobin O Arab is an abnormal hemoglobin well tolerated except for heterozygous category which requires iterative transfusions. Spelenectomy is indicated in case of hypersplenious. The evolution is generally good with a long survival.

Megaloblastic anemia in North Africa.

We prospectively studied 478 patients with megaloblastic anemia living in Tunisia. Overall, 98% of patients had vitamin B12 deficiency. Pernicious anemia accounted for most of these cases, and median age at presentation was 45 years. Megaloblastic anemia occurred in 19 subjects under 15 years of age, and of these, nine had the Immerslund-Graesbeck syndrome.

[Alpha interferon in children with Philadelphia chromosome-positive chronic myeloid-leukaemia]. / Les resultats de l'interferon alpha dans le traitement de la leucemie myeloide chronique de l'enfant. A propos de 5 cas.

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/m2 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. Myelosuppression 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.

[Management of severe thrombopenia in pregnancies and labor. Report of 14 cases and review of the literature]. / La prise en charge des thrombopénies sévères au cours de la grossesse et de l'accouchement. A propos de 14 cas et une revue de la littérature.

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 weight at delivery (3 cases) and in one case cerebral bleeding was observed.

[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).

[Prognostic impact of karyotype in de novo acute myeloid leukemia: study of 139 cases]. / Impact pronostique du caryotype dans les leucemies aigues myeloides: etude de 139 cas.

A group of 139 patients with de novo acute myeloid leukemia were investigated to determine the prognostic significance of karyotype on early death, complete remission, continuous complete remission and survival. There were 27 children and 112 adults. Mean age was 32 years. t(15;17) was found associated with a high rate of early death and a diploid karyotype with long continuous complete remission. The presence of a structural change was predictive of shorter survivals. The study of the prognostic impact of recurrent anomalies reveals a good prognostic impact for normal karyotype (1 year survival probability: 40%), followed by t(8;21) (1 year survival probability: 24%), and by t(15;17) (1 year survival probability: 9%).