Lenalidomide-Associated Hemophagocytic Lymphohistiocytosis With Plasma Cell Phagocytosis

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a severe systemic inflammatory syndrome that is often fatal. In the adult population, it is believed to develop secondary to immune dysregulation due to rheumatologic, infectious, malignant, and recently, immunomodulatory drugs. It's co-occurrence with phagocytosis by nonmacrophage cells has not been previously well defined. We present a case of lenalidomide-associated HLH with concurrent plasma cell hemophagocytosis in a patient with controlled multiple myeloma (MM).

Secondary Hemophagocytic Syndrome in a Patient with Plasma Cell Myeloma and CNS Involvement Treated with Lenalidomide

“…In this patient, the major pathophysiologic mechanism, thought to be responsible for triggering secondary HLH, was the immunomodulatory drug lenalidomide, which could alter T cell function and aggravate immune dysregulation. However, data supporting a causal relationship between lenalidomide and HLH are limited [ 12 ]. The fact that HLH resolved after definite withdrawal of lenalidomide further supports this assumption.…”