Angiotropic lymphoma: a rare hematological malignancy.

56-year-old woman was referred to us complaining of painful subcutaneous nodules in the lower extremities during the last three months. The nodules appeared a year ago. The physical examination disclosed palpable nodules in the medial surfaces of the thighs, each with a maximal diameter of 1.5 cm. The diagnosis was made after the biopsy of a nodule from the thigh (Figure 1). The skin and the subcutaneous tissue presented dilated small vascular ramifications, whose lumens were filled with aggregates of large, atypical lymphoid cells. These lymphoid cells were LCA + and L26 + , and there was no monoclonal k or l chain expression; also, in order to exclude T-cell lineage, CD3 and CD45RO antigens were assayed and found to be negative. The patient was treated with CHOP-bleomycin (8 cycles) plus methotrexate intrathecally (5 cycles). After the initial three cycles of CHOP-bleomycin the physical examination was negative for nodules and CT scans of the head, chest and abdomen showed no evidence of disease. The disease relapsed after the completion of the seventh chemotherapy cycle as shown in a new nodule biopsy, this time with bone marrow involvement. The patient received a single cycle of ESAP with no response and died following complications of septic shock. Angiotropic lymphomas (also referred to as intravascular malignant lymphomas, malignant endotheliomatosis or neoplastic angioendotheliosis) are rare and very aggressive intravascular non-Hodgkin’s lymphomas of B-cell lineage. 1 Clinical findings