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A 50-year-old woman is presented here with natural killer (NK) cell type lymphoproliferative disorder of granular lymphocytes. She was admitted to the hospital because of dyspnea on exertion. Chest X-ray revealed bilateral reticular shadows. Open lung biopsy demonstrated usual interstitial pneumonia (UIP). Her white blood cell count was 3,900/mm3, of which 55% was large granular lymphocytes (LGLs). The LGLs were CD3- CD16+CD56+, and the clonality of them was not confirmed. Despite steroid therapy, she died from exacerbation of UIP complicated with opportunistic infection. The patient, her father and son had pancytopenia. Congenital immunological abnormality might cause both large granular lymphocytosis and UIP.

Lymphoproliferative Disorder of Granular Lymphocytes (Natural Killer Cell Type) with Interstitial Pneumonia in a Patient with Familial Pancytopenia.