Intravascular Lymphoma Presenting as Multiple Endocrine Failure, Transforming Into the Hemophagocytic Variant, and Relapsing as a Thrombotic Microangiopathy.

Abstract

Intravascular large B-cell lymphoma (IVLCBL) is a rare form of non-Hodgkin's lymphoma and is characterized by the growth of large B-cells within blood vessels and bone marrow sinusoids. A 55-year-old man presented with a multiple endocrine failure which progressed to a pancytopenia. A bone marrow biopsy revealed a diffuse infiltration by large B-cells in the sinusoids consistent with intravascular lymphoma. After 6 cycles of R-CHOP, complete remission was achieved. Six months later, the patient relapsed presenting with a thrombotic microangiopathy which progressed to multiple organ failure and death.