Intrasinusoidal bone marrow infiltration revealing intravascular lymphomatosis

Intravascular lymphoma is a rare, often fatal disease characterized by a wi despread intravascular proliferation of neoplastic lymphoid cells. Dermatol ogical and bizarre neurological manifestations usually predominate. We repo rt a case of intravascular lymphomatosis with an exceptional clinical prese ntation showing splenomegaly combined with early bone marrow involvement. T he diagnosis was made on bone marrow biopsy examination using both immunohi stochemistry and molecular biology analysis. We stress the histopathologica l 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 agressive disease, can then be initiated.