De novo t(11:19) 염색체 전위를 보인 급성전골수성백혈병 1예
A Case of Acute Promyelocytic Leukemia with a De novo t(11;19) Chromosomal Translocation
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초록
저자들은 APL 환자에서 흔히 발견되는 t(15;17) 전위가 아닌 t(11;19)를 처음 진단 시점부터 보이는 증례를 경험하였다. 이 환자는 idarubicin과 cytarabine으로 관해유도치료를 시행하여 완전관해에 도달하였으나, 그 기간이 전형적인 APL보다 짧았고 ATRA에 반응을 보이지 않았다. 이는 전 세계적으로도 매우 드문 것으로 국내에서는 최초로 보고되는 증례이다.
Abstract
Acute promyelocytic leukemia (APL), which is usually defined by the morphological features of the leukemic cells, is characterized by the t(15;17) (q22;q21) chromosomal translocation and disseminated intravascular coagulation. This specific translocation results in a new fusion transcript between the promyelocytic leukemia (PML) gene and the retinoic acid receptor-alpha (RARα) gene. Although the presence of this fusion gene can predict a favorable clinical response to all-trans-retinoic-acid (ATRA) treatment, APL with chromosomal translocations other than t(15;17) (q22;q21) is extremely rare and is associated with a poor prognosis. We experienced a case of APL with de novo t(11;19). (Korean J Med 2011;80:S268-S272)
참고문헌 (10)
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