Hepatosplenic T cell lymphoma responsive to 2'-deoxycoformycin therapy

Hepatosplenic T cell lymphoma (HSTL) is a rare condition usually with an aggressive course and a poor prognosis even after extensive treatment. We describe here a patient who presented with hemophagocytosis. The lymphoma had unusual phenotypic features, an indolent course and responded to 20-deoxycoformycin therapy as a single agent. We suggest that this therapy be used in further cases as part of the treatment strategy.