L. Hessissen

Determinants of severe oral mucositis in paediatric cancer patients: a prospective study

OBJECTIVE To analyse the incidence and the determinants of severe oral mucositis (OM) in young cancer patients treated by standard chemotherapy. METHODS The study was carried out at the Pediatric Hemato-Oncology unit of Childrens Hospital of Rabat. Patients under 16 years of age with malignant disease treated by chemotherapy between January 2001 and December 2006 were recorded. RESULTS Consecutive patients (n = 970) with malignant disease were studied. The age ranges from 2 months to 16 years (mean, 6.8 ± 4.1 years). OM occurred in 540 (55.6%) patients, and 17.9% of them encountered severe grades. Mean time to onset of the lesions was 10.5 ± 6.8 (range, 1-22 days) and mean duration was 6.8 ± 3.1 (range, 2-23 days). All chemotherapeutic protocols were associated with OM development (range, 20-100%). Patients with severe OM were more likely to have undifferentiated carcinoma of nasopharyngeal type (RR = 2.6, 95% IC 1.1-6.1), non-Hodgkin lymphoma (RR = 2.1, 95% CI 1.2-2.4) and acute leukaemia (RR = 1.7, 95% CI 1.5-3.6). Methotrexate-based therapies were also associated with the worsening of OM (RR = 1.7, 95% IC 1.2-2.6). CONCLUSION Underlying disease and chemotherapy regimens are the principal risk factors of OM development. This model can help in the identification of patients at risk for adequate preventive and therapeutic measures.

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.

Cytogenetic Profile of Moroccan Pediatric Acute Lymphoblastic Leukemia: Analysis of 155 Cases With a Review of the Literature

&NA; The purpose of the present study was to define the frequency of chromosomal abnormalities in 155 Moroccan patients with acute lymphoblastic leukemia referred to the BIOLAB Laboratory from the Children’s Hospital of Rabat and compare our findings with those from reported studies. We identified chromosomal aberrations in 66% of the cases, of which 70% revealed recurrent abnormalities with high prognostic value that correlated with the reported data and their lineage. Background: Acute lymphoblastic leukemia (ALL) is the most common malignancy in children, with a peak incidence at 2 to 3 years of age and accounting for almost 30% of all cancers in this age group. It is well established that the identification of cytogenetic abnormalities is highly relevant for the prognosis of and therapeutic decisions in ALL. The purpose of the present study was to define the frequency of recurrent chromosomal abnormalities of ALL in Moroccan patients referred exclusively to the BIOLAB Laboratory of the Childrens Hospital of Rabat during a 4‐year period and compare our findings to the reported data. Patients and Methods: We performed conventional karyotyping of 155 ALL cases, with a successful cell culture rate of 94%. Results: We identified chromosomal abnormalities in 66% of the total studied cases, of which 70% revealed important recurrent abnormalities with high prognostic value, such as hyperdiploidy, hypodiploidy, t(9;22), t(8;14), t(1;19), and MLL rearrangements. In total agreement with the reported data, most of the patients (56%) in the present study were aged 1 to 5 years, with a male predominance, and B‐ALL was the most common blast phenotype (85%). Conclusion: The frequency of most chromosomal rearrangements successfully identified in our study and their lineage correlated with those reported in the published data.