L'hemophagocytose lymphohitiocytaire familiale: Apropos d'un cas

ABSTRACT

Familial hemophagocytic lymphohistiocytosis is still a rare serious disease. It is usually suspected on clinical and biological features but its diagnosis relies on histology. The authors report a case of a little boy, aged 3 months with a cutaneous rush, fever and a hepatosplenomegaly. The fine needle hepatic biopsy found a periportal infiltration by histiocytes engaged in active hemophagocytosis. The treatment was based on chemotherapy and corticoids but evolution was marked by the death of the patient