Systemic anaplastic Non-Hodgkin lymphoma in children: a 5 cases report

Anaplastic large cell lymphoma [ALCL] accounts for 10 to 15% of childhood non-Hodgkin's lymphomas. It is characterized by a high frequency of extranodal involvement, a wide morphological spectrum and the expression of CD 30. The therapeutic strategy is not yet well established. We report 5 cases of childhood ALCL diagnosed between 1999 and 2002 in the paediatric oncology and haematology department in Casablanca. Three patients presented with a bone lesion while the other 2 patients had both nodal and cutaneous involvement. The phenotype was T in 3 cases and null in 2 cases. All the patients were in stage III according to Murphy classification. The patients were treated according to the SFOP HM 91 protocol. Four patients are in CR with a follow-up of 41, 26, 18 and 11 months respectively. The fifth patient is lost to follow-up in CR. ALCL accounts for 10% of childhood NHL in our department. It is probably underestimated because of the lack of all monoclonal antibodies especially the ALK. All our patients achieved a CR and 4 of them are alive and disease free survivors. In conclusion, this type of childhood NHL seems to have a good prognosis in our context and more efforts have to be done in the diagnosis of this type of NHL

maladie de gaucher. Traitement enzymatique substitutif a props d'un cas avec revue de la lettirature.

Gaucher's disease, rare, hereditary and potentially mortal affection is characterized by the reduced concentration of the glucocerebroside lipid within the macrophage lysosomes. We report the case of a young 2 years old patient treated by transfusion since he was 9 months because of chronic anemia. According the clinical examination, the general state of the patients was bad with important delayed stanturoponderal growth, a cutaneomucous paller and enormous splenomegaly. The blood count formula showed anemia with major thrombocytopenia. The myelogram was poor and the osteomedullar biopsy showed the presence of Gaucher's cells. The diagnosis has been confirmed by enzymatic dosage [Leucocytar b-glucosidase]. The treatment of the patient has been substitutive enzymatic [inifucerase] with very favorable response. During Gaucher's disease, the enzymatic deficiency results in the pathologic accumulation of the substrate [glucocerebroside] in the lyososomes, this metabolic overloading may cause polyvisceral disease with spontaneous evolution after mortal. The recent discovery of a recombining glucocerebrosidase [imiglucerase] transformed the prognosis of this disease

Adrenocortical tumors in children: a 2 cases report

Adrenocortical carcinoma is a very rare malignant tumour in children. It has bad prognosis and the treatment protocols are not well established. We report 2 cases of adrenocortical carcinoma in children. Case 1: A 30-month-old girl was hospitalized for clitorimegaly and pubic hair. The clinical examination found an abdominal mass with paraplegia. Abdominal ultrasonography found a heterogeneous calcified mass localised in the right suprarenal region. The diagnosis was confirmed by elevated urinary 17-ketosteroids. Complementary investigations showed pulmonary, medullar and vertebral metastasis. The treatment consisted of chemotherapy [Cisplatinum + Vepeside] with no efficacy. She died after 7 months follow up. Case 2: An 8-year-old boy was admitted for a pubic and axillary's hair, with acne and abdominal mass. The abdominal ultrasonography found a heterogeneous mass in the right suprarenal region. The diagnosis was confirmed by histology after complete resection. The patient was lost from follow-up and was seen one year later with abdominal mass associated to pulmonary and liver metastasis. He received only a palliative treatment. Comments: The adrenocortical carcinoma is a rare tumour in children [0.2% of solid tumours]. The malignant criteria and treatment protocols are not well established. The 5-year OS is 49%. The most important prognosis factor is total excision of the tumour

[Viral contamination in hemophilia]

Viral contamination with blood products is a major problem in patients with hemophilia. The aim of this study is to look for viral infection ie hepatitis and immunodeficiency syndrome virus in patients with hemophilia in our institution. Hence, 40 patients have been studied. Viral hepatitis is found in 43% of cases, witch is in accordance with the results found by the majority of authors. On the other hand, since our patients have seldom been treated with factor VIII concentrate, no impaired immunodeficiency syndrome have been found