SIMULTANEOUS OCCURRENCE OF VIRAL-ASSOCIATED HEMOPHAGOCYTIC SYNDROME AND LANGERHANS CELL HISTIOCYTOSIS - A CASE-REPORT

Langerhans cell histiocytosis (LCH) is a class I histiocytosis charact erized by the presence of the pathologic Langerhans cell, an unique hi stiocyte. In contrast to LCH, class II histiocytosis is characterized by the proliferatian of mononuclear phagocytes other than Langerhans c ells and includes sinus histocytosis with massive lymphadenopathy, vir al-associated hemophagocytic syndrome, and familial hemophagocytic lym phohistiocytosis. Until now, these two classes have been considered se parate, if related, entities. We report a 2O-month-old girl who presen ted with pyrexia, hepatosplenomegaly, an eczematous skin rash, anemia, thrombocytopenia, and a markedly elevated serum IgG and IgM antibody level to cytomegalovirus. Histologic proof of both hemophagocytosis in the liver and bane marrow and LCH in the skin was obtained at present ation. The clinical course and response to treatment over 6.5 years is recorded. Although the etiology of both class I and class II histiocy tosis remains unknown, we speculate that the monocytic/macrophage diso rder, as well as the LCH, were both triggered by virus or viral-relate d monokines secreted by activated macrophages.