A case of angiotropic large cell lymphoma is reported in which the patient presented with only pulmonary symptoms. It suggests that this rare and highly malignant lymphoma should be considered in the differential diagnosis of interstitial lung disease, and shows the value of open lung biopsy in unexplained interstitial lung disease. This patient responded well to treatment with cyclophosphamide, doxorubicin, vincristine, and prednisone.
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