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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.

Granulocytic sarcoma is a tumor composed of proliferating myeloblastic cells, generally found in the orbit. A brain localization is rare. We report the case of a 11-year-old boy treated in our unit for acute myeloblastic leukemia (AML 4 Eo. FAB). After 21 months of complete remission, he developed headache and facial palsy. The CT scan visualized the presence of two frontal and occipital masses. The spinal tap revealed blastic cells in the CSF. The study of the bone morrow showed medullar relapse. A new medullar and cerebro-meningeal remission was obtained with chemotherapy and radiotherapy. CSF and the bone marrow studies can help avoid stereotaxic biopsy can be avoided in this type of tumor

Pleuropulmonary blastoma is an uncommon malignant lung tumor observed in children. Outcome is often unfavorable. Two boys and two girls, mean age 4.5 years, were admitted for nonspecific respiratory signs. Oriented by radiology findings, the diagnosis of pleuropulmonary blastoma was confirmed at pathology examination of a pneumonectomy specimen. Three of the children were given postoperative adjuvant chemotherapy. There were three deaths and one child was lost to follow-up. We discuss the clinical features of pleuropulmonary blastoma. No optimal treatment has been defined for this often fatal tumor.

Vascular thrombosis remains severe complication in patients with nephrotic syndrome. Both venous and arterial thrombosis are observed. We report three new cases of arterial thrombosis in patients with nephrotic syndrome. The role of acquired hemostasis disorders, inducing hypercoagulability, is predominant. Extramembranous glomerulonephritis remains the most frequent cause of nephrotic syndrome, complicated by vascular thrombosis. Treatment is based on anticoagulation and corticosteroid therapy. Search for proteinuria should be part of the etiology work-up in all patients with vascular thrombosis of undetermined origin.