심근 및 전신의 연조직을 침범한 Richter 증후군의 1예 (Rare Presentation of Richter’s Transformation to Diffuse Large B Cell Lymphoma: a Case Report)

서지정보

· 발행기관 : 대한내과학회
· 수록지 정보 : 대한내과학회지 / 90권 / 2호 / 163 ~ 168페이지
· 저자명 : 구은주, 김민경, 공은정, 고성애, 구미진, 정지윤, 현명수

초록

Richter 증후군(Richter’s syndrome or Richter’s transformation)은 만성 림프구백혈병이 공격적인 악성종양으로 전환되는 것을 의미하는 임상병리학적 용어이다[1-3]. 1928년에 Maurice Richter에 의해 만성 림프구백혈병이 림프종으로 전환된 예가 처음으로 보고되었으며, 1966년 “Richter 증후군”이라는 용어가 처음으로 사용되었다[1-3]. 만성림프구백혈병 환자의 2-10%에서 Richter 증후군이 발생하는 것으로 알려져 있다[1].

영어초록

Richter’s syndrome refers to the development of aggressive lymphoma in a patient with chronic lymphocytic leukemia (CLL). It occurs in about 2% to 10% of patients with CLL. The most frequent manifestation of Richter’s syndrome is diffuse large B cell lymphoma (DLBCL). Extranodal involvement is rare but can occur. The prognosis of Richter’s syndrome is very poor. We herein report a case of a rare presentation of Richter’s syndrome. A 42-year-old man diagnosed with CLL 2 years previously developed nodules on the bilateral thighs and buttocks. A positron emission tomography (PET)-CT scan revealed high fluorodeoxyglucose uptake in multiple lymph nodes, skeletal muscles, and the myocardium. An ultrasonography-guided biopsy confirmed Richter’s syndrome from CLL to DLBCL. The patient was treated with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone chemotherapy. After six cycles of chemotherapy, we performed a PET-CT scan that revealed a complete response. However, 3 months later, the syndrome recurred. The patient was undergoing salvage chemotherapy at the time of this writing.

참고자료

· 없음