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면역성혈소판감소증을 동반한 사르코이드증

Immune thrombocytopenia associated with sarcoidosis

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초록

Sarcoidosis is a systemic disease of unknown cause involving multiple organs and is characterized by noncaseating granuloma. Immune thrombocytopenia (ITP) is an autoimmune disease characterized by increased peripheral platelet destruction due to the presence of an antibody to the platelet and abnormal platelet production. There is no known pathogenesis that occurs concurrently with ITP and sarcoidosis. However, considering together of 2 known pathogenesis, abnormal immune response triggers either ITP or sarcoidosis. The disease that develops first stimulates secondary disease. After development of secondary disease, they stimulate each other. A few cases of ITP associated with sarcoidosis are well documented in English; however, the disease has rarely been reported in Korea. Here, we report on a case of ITP with sarcoidosis in a 29-year-old man. He suffered from easy bruising. The chest X-ray and the contrast-enhanced computed tomography scan showed bihilar lymphadenopathy and reticulonodular infiltrates. Bone marrow study and fluoroscopy-guided percutaneous needle biopsy were performed and the patient was diagnosed with sarcoidosis and ITP. He was put on 400 mg/kg of intravenous immunoglobulin for 5 days and administered oral steroids and further follow-up will be carried out. He has shown a good response without significant bleeding event. However, administration of more oral steroid and additional follow-up is required than for single disease, whether sarcoidosis or ITP.

Abstract

Sarcoidosis is a systemic disease of unknown cause involving multiple organs and is characterized by noncaseating granuloma. Immune thrombocytopenia (ITP) is an autoimmune disease characterized by increased peripheral platelet destruction due to the presence of an antibody to the platelet and abnormal platelet production. There is no known pathogenesis that occurs concurrently with ITP and sarcoidosis. However, considering together of 2 known pathogenesis, abnormal immune response triggers either ITP or sarcoidosis. The disease that develops first stimulates secondary disease. After development of secondary disease, they stimulate each other. A few cases of ITP associated with sarcoidosis are well documented in English; however, the disease has rarely been reported in Korea. Here, we report on a case of ITP with sarcoidosis in a 29-year-old man. He suffered from easy bruising. The chest X-ray and the contrast-enhanced computed tomography scan showed bihilar lymphadenopathy and reticulonodular infiltrates. Bone marrow study and fluoroscopy-guided percutaneous needle biopsy were performed and the patient was diagnosed with sarcoidosis and ITP. He was put on 400 mg/kg of intravenous immunoglobulin for 5 days and administered oral steroids and further follow-up will be carried out. He has shown a good response without significant bleeding event. However, administration of more oral steroid and additional follow-up is required than for single disease, whether sarcoidosis or ITP.

참고문헌 (10)

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