FAMILIAL HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS AND VIRAL-INFECTIONS

A retrospective study was performed in 32 children with hemophagocytic lymphohistiocytosis, 16 of whom had affected siblings. Altogether 22 of these children, of whom the majority (13/22) were familial cases, h ad clinical or laboratory signs of infection. Laboratory analysis demo nstrated Epstein-Barr virus in five children, cytomegalovirus in three and human parvovirus in two. Two siblings with onset of familial hemo phagocytic lymphohistiocytosis within one month of each other, both of whom demonstrated serological indications of a recent human parvovira l infection at onset, are described. It is concluded that a viral infe ction cannot serve as the sole criterion for distinguishing a virus-as sociated hemophagocytic syndrome as an entity separate from familial h emophagocytic lymphohistiocytosis. Instead, it is suggested that viral infections may elicit a bout of the familial hemophagocytic lymphohis tiocytosis disorder in genetically predisposed individuals.