Intrasinusoidal bone marrow infiltration revealing intravascular lymphomatosis

Intravascular lymphoma is a rare, often fatal disease characterized by a widespread intravascular proliferation of neoplastic lymphoid cells. Dermatological and bizarre neurological manifestations usually predominate. We report a case of intravascular lymphomatosis with an exceptional clinical presentation showing splenomegaly combined with early bone marrow involvement. The diagnosis was made on bone marrow biopsy examination using both immunohistochemistry and molecular biology analysis. We stress the histopathological features of bone marrow involvement by intravascular lymphoma which allow the prompt recognition of this disease. Early systemic chemotherapy, which represents the only chance of remission in such an aggressive disease, can then be initiated.