[Epidemiologic, clinical and therapeutic aspects of chronic lymphoid leukemia: apropos of 120 cases]. / Aspects épidémiologiques, cliniques et thérapeutiques de la leucémie lymphoïde chronique: à propos de 120 cas.

We retrospectively studied 120 cases of chronic lymphocytic leukemia diagnosed between January 1988 and December 1998. The median age of our patients was of 66 years, 75% among them were male. The discovery of the illness was fortuitous in 20% of the cases, the peripheral adenopathy and the splenomegaly were noted respectively in 72 and 48% of the cases. The blood lymphocytosis was on average 51.109/1 with extremes of 5 and 818.109/1. Anemia was noted in 71% of the cases and a thrombopenia in 42%. Fifty patients were classified C stage of BINET and sixty elevated risk according to RAI. The therapeutic attitude was according to patient's age and the CLL stage. Thus, 94 patients received a chemotherapy and a complete or partial response was observed in 58 of the cases. The overall survival at 5 years were 47%. The retained prognostic factors were the stage according to the classifications of BINET and RAI, the thrombopenia and the lymphocytosis blood overhead 100.109/1.

[Gastric adenocarcinoma secondary to Hodgkin's disease treatment]. / Adenocarcinome gastrique secondaire au traitement d'une maladie de Hodgkin.

UNLABELLED: Second malignant neoplasms are a major cause of late morbidity and mortality following treatment for Hodgkin's disease. Gastric carcinoma belong to the rare secondary malignancies induced by radiation-therapy and it is associated with a poor prognosis. We report a patient treated for Hodgkin's disease by 6 ABVD and total lymphoid radiation therapy, who developed a gastric carcinoma 9 years after completing treatment. Our case fits the criteria for radiation induced malignancies reported from the literature: IN CONCLUSION: recommendations are presented for both prevention and early detection of the tumours we recommend a strict follow-up for patients treated for HD to detect second cancers.

[Acute lymphoblastic leukemia with Philadelphia chromosome: eight case reports]. / Leucémie aiguë lymphoblastique avec chromosome Philadelphie: à propos de huit observations.

Philadelphia chromosome-positive (Ph+) acute lymphoblastic leukemia (ALL) is an aggressive form of acute leukemia that children ALL. Between 1991 and 1998, eight cases Ph+ ALL (7 males and one female) were diagnosed in our institution by successful cytogenetic studies. Median age was 37 years (range, 1-60 years). Leukocyte count was more than 50 x 109/l in 5 cases. According to the French-American-British (FAB) criteria, six patients were classified L1 and two L2. The Ph+ as sole anomaly was seen in 2 patients (25%), while additional chromosome changes were observed in 6 cases. Complete remission was achieved in 5 cases (62%) and relapse was observed in all cases? The 2-year survival rae was 25% confirming the worse prognosis of this leukemia when treated with standard chemotherapy.

[Invasive aspergillosis in the leukemic patient]. / Aspergillose invasive chez le leucémique.

Invasive pulmonary aspergillosis (IPA) remains a life threatening complication in immuno-compromised and especially in neutropenic patients. We report our experience in the diagnosis and therapeutic management of IPA in 8 patients with acute leukemia. All patients were neutropenic (PNN < 100/mm3, mean duration = 37 days) when IPA was diagnosed. Clinical signs included fever above 39 degrees and cough in all cases, chest pain in 4 cases, hemoptysis in 3 cases, rales in 5 cases. Chest x ray showed one lesion in 4 cases and multiple lesions in 4 cases. The diagnosis of IPA was established by bronchoalveolar lavage (BAL) in 5 cases, tissue biopsy in one case, positive sputum in one case and it was highly probable in one case. Thoracic computed tomographic (CT) scans were preformed after diagnosis confirmation of IPA and showed one or multiple lesions with air crescent signs. Serological tests were positive in 4 cases late in the course of IPA. All patients were treated with i.v. Amphotericin B. Outcome was favorable in 5 cases and three patients died by massive hemoptysis (in two cases) and systemic aspergillosis (in one case). Early diagnosis and appropriate treatment are essential to improve IPA prognosis.

[Childhood acute myeloblastic leukemias. Report of 21 cases]. / Leucémies aiguës myéloblastiques de l'enfant. A propos de 21 cas.

Between 1989 and 1996, 21 cases with acute non lymphoblastic leukemia (11 males and 10 females) were diagnosed in our institution. Median age was 9 years (range, 2-15 years). Leukocyte count was more than 50,109/l in 47% of cases. According to the French-American-British (FAB) criteria, 7 cases were classified M1, 10 cases were classified M2, 1 classified M4Eo and 3 classified M5. All patients were treated with "7 + 3" protocol and complete remission was achieved in 17 cases (80%), 2 cases (10%) failed to respond and 2 (10%) died during induction. Relapse was observed in 15 cases. The 3-year survival rate was 20% and the relapse-free-survival rate was 12% confirming the worse prognosis of this leukemia when treated with standard chemotherapy.

[Acute lymphoblastic leukemia in adults. Apropos of 42 cases]. / Leucémie aiguë lymphoblastique de l'adulte. A propos de 42 cas.

Between 1989 and 1995, 42 cases with acute lymphoblastic leukemia (18 males and 24 females) were diagnosed in our institution. Median age was 38.5 years (range, 16-88 years). Leukocyte count was more than 30.10(9)/l in 54% of cases. According to the French-American-British (FAB) criteria, 67% were classified L1 and 33% L2. Sixteen patients were treated with 12LA80 protocol, 14 patients with LALA 85 protocol, 6 patients with LALA 87 protocol and 6 patients with EORTC protocol. Complete remission was achieved in 22 cases (52%), 8 cases (20%) failed to respond and 12 (28%) died during induction. Relapse was observed in 10 cases. The 4-year survival rate was 28% confirming the worse prognosis of this leukemia when treated with standard chemotherapy.

[Acquired amegakaryocytic thrombocytopenic purpura treated with intravenous immunoglobulins]. / Le purpura thrombopénique amégacaryocytaire acquis traité par des immunoglobulines intraveineuses.

Acquired amegakaryocytic thrombocytopenic purpura is a rare disorder characterized by severe thrombocytopenia due to the absence of bone marrow megakaryocytes. The pathogenic mechanisms of this disorder have not well defined; consequently, several empirical therapies are used. We reported the case of a 38-year-old mean who was hospitalized for serious bleeding syndrome. The platelet count was 10 yen10(9)/L. The bone marrow aspirate and biopsy showed the absence of megakaryocytes but otherwise normal granulocyte and erythroid precursors. No definable etiology has been found. After the unsuccessful use of prednisone, intravenous immunoglobulin therapy was started and resulted in favorable reponse.

[Gougerot-Sjögren syndrome disclosed by MALT lymphoma of the salivary glands. Report of 3 cases]. / Syndrome de Gougerot-Sjögren révélé par un lymphome MALT des glandes salivaires. A propos de trois cas.

Sjögren's syndrome is characterized by an increased risk of developing non-Hodgkin's lymphoma. The lymphoma is most frequently extra-nodal, preferentially affecting the salivary gland: low-grade MALT lymphoma. Conversely, underlying Sjögren's syndrome has been recently identified by some authors in patients with non-Hodgkin's lymphoma. In the present report, we present three cases of Sjögren's syndrome disclosed by low-grade salivary gland MALT lymphoma. The patients were all women aged 33, 38 and 52 years. Extension work-up revealed nodal and bone marrow involvement in one case and no evidence of disseminated disease in the two others. Using the European criteria, all of our patients had certain Sjögren's syndrome. Labial salivary gland biopsy and immunopathological studies in newly diagnosed low-grade MALT lymphoma would be helpful in identifying the real frequency of this association.

[Acute leukemia in the elderly patient. Forty case reports]. / Leucémies aiguës du sujet âgé. A propos de 40 cas.

Between 1989 and 1996, 40 cases with acute leukemia (16 males and 24 females) were diagnosed in our institution. Median age was 65 years (range, 56-88 years). Leukocyte count was more than 30.109/l in 42% of cases. According to the French-American-British (FAB) criteria, 11 cases were classified lymphoblastic and 29 myeloblastic. Sixteen patients have received palliative treatment because of there age and there bad performance status. Only 24 patients have received curative treatment. Complete remission was achieved in 12 cases (50%), 5 cases (20%) failed to respond and 7 (30%) died during induction. Relapse was observed in 8 cases. The 2-year survival rate was 10% confirming the worse prognosis of the acute leukemia in elderly.

Contribution of flow cytometry to acute leukemia classification in Tunisia.

The precision of immunological characterization of leukemias was improved by a certain number of technical innovations, particularly hybridoma production and standardization, resulting in monoclonal antibodies and definition of recognised cellular antigens (designated by CD: Cluster of Differentiation). The aim of this work was to determine the immunophenotyping profile of patients with leukemia, by means of a flow cytometric method: 66 blood samples coming from leukemic persons in the Sahel region were studied by flow cytometry, using about thirty monoclonal antibodies all marked with a fluorochrome, in one or two colour systems to assess their distribution according to type (lymphoid B or T / myeloid) and age, and to search for possible co-expressions of markers of different lineages. The marked preponderance of childhood B-ALL in our series is, at least partly, attributable to the age distribution of the Tunisian population. In agreement with studies from other countries, the majority of AML cases occurred among adults. A high proportion of AML cases in our series co-expressed markers of other lineages. Overall, accurate classification of acute leukemias was possible from a simple peripheral blood sample in 62 of 66 cases (93.9%).

[Hemorrhagic syndrome and isolated alpha 2-antiplasmin deficiency. Apropos of a case]. / Syndrome Hémorragique et déficit isolé en alpha 2 antiplasmine. A propos d'un cas.

An isolated alpha 2 plasmin inhibitor deficiency is reported in a 33 years old male, presenting repeated intramuscular hematomas since 5 years, spontaneously or after minor traumas. None of other family members were suffering from abnormal bleeding. Screening hemostatic examinations were normal except for a moderately shorted euglobulin lysis time (2 hours). Evaluation of fibrinolysis parameters (plasminogen, plasminogen activator inhibitor type 1, tissue plasminogen activator, fibrin and fibrinogen degradation products, alpha 2 plasmin inhibitor) showed normal values except for alpha 2 plasmin inhibitor which is markedly decreased (activity: 14%, antigen: < 5%). Familial hemostasis investigations have not been performed. This isolated alpha 2 plasmin inhibitor deficiency has been confirmed by two repeated control prelevments. Bleedings episodes were treated with antifibrinolytics agents (tranexamic acid). This case report shows the importance of the diagnostic approach in the laboratory to detect such rare hemostatic abnormalities associated with bleeding tendency.

First study of immunoglobulin and T cell receptor gene rearrangements in chronic and acute lymphoblastic leukemias from Tunisia.

We report the first characterization at the immunological and molecular level of 12 cases of chronic lymphocytic leukemia (CLL) and acute lymphoblastic leukemia (ALL) from Tunisia. Our results show biallelic IgH gene rearrangement in B-CLL (6/6). A high ratio of T-ALL (4/6) was observed in Tunisian ALL leukemias. One T-ALL expressed CD10 (common ALL) which has already been found in some other cases of T-ALL. We report the occurrence of T cell receptor (TCR) beta and/or gamma gene rearrangements in two precursor B-ALL patients who had normally rearranged Ig genes. In one precursor B-ALL case, multiple rearranged IgH and TCR gamma bands allowed the identification of three clones. Such an oligoclonal ALL is interesting since only rare biclonal TCR beta or gamma gene rearrangements have been described.

[Screening of hemoglobinopathies and molecular analysis of beta-thalassemia in Central Tunisia]. / Dépistage des hémoglobinopathies et analyse moléculaire des beta-thalassémies en Tunisie centrale.

BACKGROUND: Previous investigations have permitted to locate 16 beta-thalassemic mutations in different samples of the Tunisian population. One of them (IVS I nt 2: T--G) had been found only in the central region of Tunisia. Our research was carried out in this part of the country to estimate the prevalence of this mutation and to establish a prenatal diagnosis using appropriate probes. POPULATION AND METHODS: One thousand one hundred and five blood samples taken from 1987 to 1990 from healthy blood donors and 346 samples taken from 1985 to 1992 from patients were analysed. Detection of hemoglobinopathies was carried out by means of specific hematological tests and different electrophoretic and chromatographic techniques. Mutations were detected by means of the usual techniques of molecular biology. RESULTS: Sickle cell anemia and beta-thalassemia were the most frequent in the samples studied. The molecular analysis carried out on eight patients native of the Essouassi-El-Djem region point out that all these patients carry the same point mutation (IVS I nt 2: T-G) detected for the first time in 1988 in a patient native of the same region. One of these patients, aged 43, who did not suffer from anemia and did not show the usual symptoms of beta O thalassemia, had one hemolytic attack at the age of 17. CONCLUSIONS: The high number of persons carrying Hb S and beta-thalassemia trait increase the risk of appearance of homozygous forms. The presence of the same mutation IVS I nt 2: (T-G) in all beta O-thalassemic patients from Essouassi-El Djem region may indicate that it may have its origin there. The heterogeneity of clinical phenotype of these patients shows the difficulty of establishing a unique strategy of prenatal diagnosis by DNA analysing which can be applied in all cases.

[Partial splenectomy in thalassemia major. Apropos of 19 cases]. / La splénectomie partielle au cours des thalassémies majeures. A propos de 19 cas.

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.