Intravascular lymphoma (IVL) is a rare lymphoproliferative disorder characterized by a proliferation of lymphoma cells within the lumen of small blood vessels. Clinical features are nonspecific and the lung involvement as a primary site is reported to be less than 10%. Usually the disease progression is rapid and antemortem diagnosis is reported to be difficult.
We report a case of a 73-year-old woman presenting with dyspnea on exertion. Chest X-ray and contrast-enhanced computed tomography showed diffuse homogeneous ground-glass opacification. Pulmonary function tests showed mild restrictive ventilatory dysfunction. Bronchoscopic examination revealed no abnormality. Neither segmental nor partial perfusion defect was observed on a lung perfusion scan using technetium‑99m macroaggregated albumin (^99ｍTc-MAA). Cardiac catheterization showed pulmonary hypertension without increase of capillary wedge pressure. These findings suggested pulmonary alveolar involvement, especially of the alveolar microvessels. As her blood examination showed a remarkable increase of CRP and LDH, we suspected that she had IVL and performed random skin biopsy on the third hospital day. A diagnosis of IVL was made. As she did not show neurological or skin involvement, she was diagnosed as having the Asian variant of intravascular lymphoma (AIVL). She was successfully treated with CHOP followed by R-CHOP.