Familial erythrophagocytic lymphohistiocytosis: Late relapse despite continuous high‐dose VP‐16 chemotherapy

Publisher Website
Google Scholar
Abstract
Familial erythrophagocytic lymphohistiocytosis (FEL) is a rare disease of infancy and early childhood. Transmitted in an autosomal recessive manner, FEL is characterized by infiltration of lymphocytes and histiocytes into visceral organs, the bone marrow, and the central nervous system (CNS) [1,2,3]. Epipodophyllotoxin (VP‐16) is currently the most effective chemotherapeutic agent against FEL; complete clinical remissions have been attained with this agent alone [4,5]. Prolonged remission has been achieved in only a few patients, usually those in whom aggressive systemic and CNS therapy was initiated early in the course of the disease [6]. For these children, the necessary duration of therapy has not been defined.