Hemophagocytic lymphohistiocytosis: A rare, potentially fatal complication in subcutaneous panniculitis like T cell lymphoma

ABSTRACT

Subcutaneous panniculitis-like T cell lymphoma is a rare subtype of cutaneous lymphomas with distinct clinical, histological and immunophenotypic characteristics, as well as an indolent clinical course. Rarely, it may be complicated with hemophagocytic lymphohistiocytosis a hyperinflammatory syndrome which, if not diagnosed early, carries a dismal outcome. In this article, we describe a case of subcutaneous panniculitis-like T cell lymphoma in a middle-aged female patient which was complicated with secondary hemophagocytic lymphohistiocytosis with a favorable outcome following etoposide-based therapy. The various histological mimics of subcutaneous panniculitis-like T cell lymphoma and the management options are also briefly discussed.