Chronic myeloid leukemia patients in Tunisia: epidemiology and outcome in the imatinib era (a multicentric experience).

Data are limited in developing countries regarding the clinicopathologic features and response to therapy of chronic myeloid leukemia (CML) in the era of imatinib (IM). The objective of this study is to report on the clinicoepidemiologic features of CML in Tunisia, to evaluate the long-term outcome of patients in chronic (CP) or accelerated phase (AP) treated with IM 400 mg daily as frontline therapy, and to determine imatinib's efficacy and safety. From October 2002 to December 2014, 410 CML patients were treated with IM in six Tunisian departments of hematology. Response (hematologic, cytogenetic, and molecular responses) and outcome-overall survival (OS), event-free survival (EFS), and progression-free survival (PFS)-were evaluated. The following prognostic factors were analyzed for their impact on the European leukemia net (ELN) response, OS, EFS, and PFS at 5 years: age, sex, leukocyte count, Sokal score, European Treatment and Outcome Study (EUTOS) score, CML phase, time to starting IM, and impact of adverse events. The median age was 45 years (3-85 years). Two hundred ten (51.2%) patients were male. Splenomegaly was present in 322 of the 410 (79%). Additional cytogenetic abnormalities were encountered in 25 (6.3%) patients. At diagnosis, 379 (92.4%) patients were in CP, 31 (7.6%) were in AP. The Sokal risk was low in 87 (22.5%), intermediate in 138 (35.7%), and high in 164 patients (41.9%). The EUTOS risk was low in 217 (74%), and high in 77 (26%) patients. The rates of cumulative complete cytogenetic response (CCyR), major molecular response (MMR), and molecular response 4/5 log (MR4.5) in CP/AP-CML patients were 72, 68.4, and 46.4%, respectively. The median time to reach CCyR, MMR, and MR4.5 was 6 months (3-51), 18 months (3-72), and 24 months (3-100), respectively. According to the ELN criteria, optimal, suboptimal response, and failure were noted in 206 (51.8%), 61 (15.3%), and 125 (31.4%) patients, respectively. Five-year event-free survival (EFS), progression-free survival (PFS), and overall survival (OS) were 81, 90, and 90%, respectively. By multivariate analysis, AP, high EUTOS risk, and baseline WBC ≥ 150G/l remained independent predictive factors of non-optimal response to IM. The adverse events (AE) of IM were moderate and tolerable. With the caveats that the monitoring of the disease was not optimal, response rates were similar to those reported in previous studies. It is clear to us that improvements should be made in treatment of AP-CML and high Sokal risk group of CP-CML. The frontline use of second-generation tyrosine kinase inhibitor (TKI) is expected to improve the results of the first-line treatment of these high-risk Tunisian patients, but cost and accessibility of this therapy remain the problems in developing countries.

[Neuro-psychiatric disorders revealed pernicious disease without anemia. About three cases]. / Tableau neuro-psychiatrique révélant une maladie de Biermer en l'absence d'anémie. A propos de 3 observations.

In addition to an anemic syndrome, pernicious disease can also include neuropsychiatric manifestations. We report here three cases diagnosed as pernicious disease revealed by neuropsychiatric symptoms and even without anemia. Our patients were made of a man and two women with a mean age of 55 years. They consulted for progressively worsening troubles of step which were related to a combined degeneration of the cord. This degeneration was associated to a cerebellar syndrome in one case. An isolated macrocytosis with a mean MGV at 109 fl, was noticed allowing thus to evoke the Biermer pernicious disease. This diagnosis was confirmed by the marrow puncture which showed a medullar megaloblastosis in two cases and an erythroblastic nucleocytoplasmic maturation's asynchronism. Treated by vitamin B12, the evolution was favorable in two cases with a total neurological recovery after six months in two patients. One patient died after six days of treatment following an acute myocardial infarction. If faced to symptoms made of a combined degeneration of the spinal card, a peripheral neuropathy and/or psychiatric troubles, pernicious disease is a diagnosis that we must evoke even in absence of anemia.

[Association of systemic lupus erythematosis and myasthenia gravis. A new case report]. / Association lupus érythémateux systémique et myasthénie. A propos d'une nouvelle observation.

The association of myasthenia gravis (MG) and systemic lupus erythematosus (SLE) is a quite rare one. We report here one case and try to discuss the physiopathologic mechanisms and also the clinical, evolutive and therapeutic features of this morbid association. It's the case of a woman of 43 years old followed in our department since 1999. The patient has been treated by corticosteroids (prednisone) with monthly administered cyclophospamide pulses. Nine months lately, she develops a diplopia with a ptosis, a dysphagia and a muscular weakness. The treatment was mainly based on intravenous immunoglobulins. The later evolution was favourable. The coexistence of SLE and MG is not casual. It must be considered in every lupic patient developing neuromuscular troubles.