The Korean Journal of Medicine

The Korean Association of Internal Medicine (대한내과학회)
권호 표지

Central Nervous System Relapse in Patients with Acute Promyelocytic Leukemia Treated with All-trans Retinoic Acid

All-trans retinoic acid 관해 유도 치료 후 중추신경계에서 재발한 급성 전골수성 백혈병 5예

  • Kim, Eun-Ok (Department of Internal Medicine, Catholic BMT Center, Seoul St. Mary's Hospital, The Catholic University of Korea College of Medicine) ;
  • Gil, Sang-Hyun (Department of Internal Medicine, Catholic BMT Center, Seoul St. Mary's Hospital, The Catholic University of Korea College of Medicine) ;
  • Choi, Jae-Ki (Department of Internal Medicine, Catholic BMT Center, Seoul St. Mary's Hospital, The Catholic University of Korea College of Medicine) ;
  • Lee, Hyun-Jeong (Department of Internal Medicine, Catholic BMT Center, Seoul St. Mary's Hospital, The Catholic University of Korea College of Medicine) ;
  • Kim, Hee-Je (Department of Internal Medicine, Catholic BMT Center, Seoul St. Mary's Hospital, The Catholic University of Korea College of Medicine) ;
  • Lee, Jong-Wook (Department of Internal Medicine, Catholic BMT Center, Seoul St. Mary's Hospital, The Catholic University of Korea College of Medicine) ;
  • Min, Woo-Sung (Department of Internal Medicine, Catholic BMT Center, Seoul St. Mary's Hospital, The Catholic University of Korea College of Medicine)
  • 김은옥 (가톨릭대학교 의과대학 내과학교실, 서울성모병원 BMT 센터) ;
  • 김상현 (가톨릭대학교 의과대학 내과학교실, 서울성모병원 BMT 센터) ;
  • 최재기 (가톨릭대학교 의과대학 내과학교실, 서울성모병원 BMT 센터) ;
  • 이현정 (가톨릭대학교 의과대학 내과학교실, 서울성모병원 BMT 센터) ;
  • 김희제 (가톨릭대학교 의과대학 내과학교실, 서울성모병원 BMT 센터) ;
  • 이종욱 (가톨릭대학교 의과대학 내과학교실, 서울성모병원 BMT 센터) ;
  • 민우성 (가톨릭대학교 의과대학 내과학교실, 서울성모병원 BMT 센터)
  • Published : 2011.02.01
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Abstract

Acute promyelocytic leukemia (APL) is distinguished from other subtypes of acute myeloid leukemia (AML) by its distinctive morphology, specific chromosomal abnormality, coagulopathy, and unique response to treatment with all-trans retinoic acid (ATRA). Central nervous system (CNS) involvement is rare in APL. Most CNS relapses occur in patients with hyperleukocytosis at initial presentation, and the optimal management of such patients is still controversial. We report five patients with APL who had CNS relapse with or without evidence of cytological and molecular disease of the bone marrow after ATRA treatment. Brain magnetic resonance imaging revealed leptomeningeal infiltration and cerebrospinal fluid examination showed the presence of promyelocytes. Patients were treated with a combination of systemic chemotherapy and radiotherapy with or without intrathecal chemotherapy, and most were subsequently treated with arsenic trioxide (ATO) as maintenance therapy. Among these patients, one received allogeneic stem cell transplantation in second complete remission.

급성 전골수성 백혈병은 특별한 세포의 형태, 전형적인 염색체 이상, 응고 장애 및 ATRA 치료에 반응이 좋은 것을 특징으로 하는 질환으로 일반적으로 중추신경계로의 재발은 흔하지 않다. 진단 당시 백혈구 증다증이 있는 경우에 중추신경계 재발의 위험이 증가하는 것으로 알려져 있으며 현재까지 중추신경계 재발에 대한 적절한 치료원칙은 정립되지 않은 상태이다. 저자들은 급성 전골수성 백혈병 환자 중 ATRA 치료 이후 세포학적 혹은 분자생물학적 재발을 동반하거나 동반하지 않은 중추신경계 재발 환자를 5예 경험하였다. 두부 방사선학적 검사에서 연수막 전이 소견을 보였으며 뇌척수액 검사에서도 전골수성 백혈병 세포의 침윤이 확인되었다. 환자들은 전신적 항암요법과 방사선 치료, 척수 항암치료로 치료를 받았으며 대부분은 삼산화비소로 유지치료를 받았다. 5예 중 1예에서는 2차 관해 이후 동종 조혈모세포이식을 시행 받고 안정된 경과를 유지하였다.

Keywords

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