Familial lymphohistiocytosis is a genetically transmitted disease affecting infants and very young children with usually a fatal outcome. Cardinal symptoms are fever, hepatosplenomegaly, and pancytopenia. Histologic examination shows infiltration of all organs with phagocytosing histiocytes and lymphocytes as well as atrophy of the normal lymphoid tissue. The distinction from other histiocytic disorders, i.e., Letterer-Siwe disease or malignant histiocytosis, may be difficult. However, the familial occurrence and characteristic findings in the coagulation system and lipid pattern make familial lymphohistiocytosis a sufficiently distinct clinical entity. This report review 79 cases fron the literature and adds four of own observations.
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