Bezanilla Regato J L, Estébanez Ruiz S, Navajas Gutiérrez A, Piñán Garcés M A, Sojo Aguirre A, Moya Calderón E
Hospital de Cruces, Clínica Infantil, Baracaldo, Vizcaya.
An Esp Pediatr. 1990 Apr;32(4):368-70.
A case of familial hemophagocytic limphohistiocytosis (FHLH) is presented in a 16 months old infant, with clinical picture of prolonged fever, cutaneous purpura, generalized lymphadenopathy and progressive hepatosplenomegaly and laboratory of severe pancytopenia, hypofibrinogenemia and hypertriglyceridemia, with elevation of the pre-beta fraction in the lipidogram, all this compatible with this disease. She also showed bone marrow and ganglionar biopsy infiltrated by histiocytic cells with hemophagocytosis. The patient received chemotherapy with cycles VP-16, vincristine (VCR) and intrathecal methotrexate (MTX), alternating every two-three weeks with VACP, during one year, with resolution of clinical and laboratory parameters after two months of this treatment without relapses. The survival from diagnosis is 39 months.
