M. N. Nachef

Pediatric rhabdomyosarcoma in Morocco.

Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in the first two decades of life. There is, however, a paucity of reports on the pattern of its occurrence in Africa. This study analyses the epidemiological pattern, clinical features, histology, and outcome in Moroccan children presenting with RMS.

Pediatric Hodgkins disease in Morocco

To the Editor: Hodgkin disease (HD) is one of the common malignancies in childhood [1]. With current treatments, 80–90% of all pediatric patients will be surviving without evidence of disease [2]. There is limited information regarding the management of HD in developing countries [3]. The purpose of our study was to analyze the epidemiological pattern, histology, staging, and treatment results on Moroccan children presenting with HD. We retrospectively studied 118 cases of histologically proven HD in previously untreated children under 15 years of age admitted at the Hematology and Oncology Unit of Rabat from 1995 to 2000. The protocol of treatment was the MDH 90 from the French Society of Pediatric Oncology [4]. The mean age of the patients was 8 years (range 2–15 years) with 28 patients (24%) less than 5 years. The male/female ratio was five. Consanguineous marriage was found in 10% and in two cases it was a history of familiarly HD. Histopathologic classification according to Lukes and Buttler was nodular sclerosis (NS) in 45% and mixed cellularity (MC) in 41%. The median delay of diagnosis was 6 months. Cervical lymphadenopathy was present in 116 patients (89.8%). In 59 patients (54.6%) at least one B symptom was present. Splenomegaly was found in 35% and hepatomegaly in 17% of cases. Mediastinal enlargement was found in 45%, pulmonary nodule in 4%, and pleurisies in 5%. Abdominal adenopathy was found in 43%. Bone marrow was involved in 14%, the kidney in 2.5%, the bone in 3.7%, and two patients had cutaneous nodule. Erythrocyte sedimentation rate was over 70 mm in 63%. Median of white blood cell was 8,200 element/mm and median of hemoglobin 10.2 g/dl. According to the Ann Arbor staging system 55% of patient where stage III–IV. Among the 118 patients, 117 were treated and 34% received treatment conforming to the protocol. Involved field radiotherapy was done in 69%. Thirty-two patients had an event, 12 were in progressive disease, 19 relapsed, and 1 died. The 7 year event-free survival was 43 9%. Among 118 patients, 57 were alive and followed-up, 3 patients died, and 58 (49%) were LOF. Age distribution of the HD differs among region and ethnic groups. In developing countries, the peak incidence is usually in the group of 5–9 years [5]. In Turkish study, the median age was 8 year [6]. Our male to female ratio was similar to that in India [3]. In western countries, NS subtype is the most common subtype [7]. In Iran and Turkey, the majority of tumors were classified as MC [5,6]. Combined-modality treatment approach has yielded excellent results, with long-term disease-free survival of 85–100% in patients with early-stage disease [8]. Many problems in managing HL have been identified and we are proposing several strategies to enhance HL management in our unit, the use of hybrid protocol according to drugs available in Morocco; a dedicated team for HL to insure an accurate assessment; and a social program to address social and financial issues. Laila Hessissen, MD* N. Oulhiane, MD M. ElKhorassani, MD M.N. Nachef, MD M. Khattab, MD F. Msefer Alaoui, MD Pediatric Hematology and Oncology Unit Children’s Hospital of Rabat Rabat, Morocco

A hemophagocytic syndrome revealing a Griscelli syndrome type 2

Griscelli syndrome type 2 is a rare autosomal recessive disorder, due to a mutation in RAB27A gene. It associates partial albinism, silver hair and immune deficiency. We report the case of a 6 year-old boy who was admitted to the Emergency department with severe sepsis complicated by hemophagocytic syndrome. Many clinical and biological criteria leads to the diagnosis of type 2 Griscelli syndrome: consanguineous family, recurrent infection, absence of psychomotor retardation, oculocutaneous albinism, silver hair, occurrence of hemophagocytic syndrome and especially the pathognomonic appearance on microscopic examination of the hair. The absence of giant organelles inclusion in all granulated cells eliminated Chediak-Higashi syndrome.