Intestinal T-Cell Lymphoma With Lung and Lymph Node Involvement at Relapse

Patients with intestinal T-cell lymphomas (ITLs) usually present with perforation of the small intestine and colon at diagnosis. At relapse or in the advanced stage, ITLs involve in other extranodal sites, but biopsy-proven lung involvement has been rarely reported. A 76-year-old male presented with sudden-onset abdominal pain, which was found to be caused by the perforation of colon. Emergency operation was carried out, and histopathological examination of the resected colon led to the diagnosis of ITL, not otherwise specified (NOS). He achieved complete metabolic remission (CMR) after eight courses of CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisolone) chemotherapy. Two months later, computed tomography showed infiltration and ground-glass opacity in the left pulmonary area in addition to the enlargement of mediastinal and left subclavian lymph nodes, although he did not complain of any pulmonary symptoms. Histopathological findings of the biopsied samples from the pulmonary area were consistent with relapsed ITL, NOS. He achieved CMR after three courses of GDP (gemcitabine, dexamethasone, and cisplatin) chemotherapy; but 1 month after the completion of GDP chemotherapy, he relapsed again with involvement of multiple lymph nodes, not in the pulmonary area. He died owing to the progression of disease. This is the third case of ITLs with lung involvement. Active biopsy should be performed when pulmonary nodules, infiltration, or ground-glass opacity are found in ITLs. A regimen for salvage chemotherapy specifically for ITLs is not yet established, and GDP chemotherapy may be an alternative option for relapsed ITLs in addition to new agents, such as romidepsin and pralatrexate.