A 54-year-old woman was admitted with general fatigue and dyspnea on exertion. Her serum LDH level was markedly elevated to 2145 IU/L, and chest CT revealed diffuse centrilobular opacities. Total cell counts in bronchoalveolar lavage fluid were elevated, and lymphocytes accounted for 98% of the cells. A transbronchial lung biopsy demonstrated numerous CD20-positive atypical cells in the alveolar capillaries, so intravascular lymphoma (IVL) was diagnosed as having. Lymphoma cells were also present in the bone marrow sinusoids, while there was no sign of hemophagocytosis. Combined chemotherapy (CHOP with rituximab) successfully induced complete remission, and she has been free of recurrence for 40 months. In cases with diffuse centrilobular opacities on chest CT, accompanied by elevated serum LDH, it is important to rule out IVL by performing TBLB.
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