AML1-ETO 양성인 양표현형 급성 백혈병의 1예
A case of biphenotypic acute leukemia with expression of the AML1-ETO gene rearrangement
0
조회
0
다운로드
0
인용
0
참고문헌
키워드
초록
BAL은 일반적으로 기타 급성 백혈병보다 예후가 나쁘다고 알려져 있으나, 그 치료방침이 아직 확립되어 있지 않다. AML1-ETO 유전자 재배열은 BAL에서도 좋은 예후인자 중 하나로 제시된 적이 있으며 본 증례에서도 1차례의 관해 유도요법 후 완전관해를 보였고, t(8:21) 즉, AML1-ETO 정량 검사상 전사 정도가 현저히 감소함을 경험하였다. 특히, AML에 준한 치료만으로 성공적 관해가 유도되었으며, 이는 BAL에 있어서도 AML1-ETO 종양단백이 병태생리에 주 역할을 할 것임을 짐작할 수 있다. AML에서와 마찬가지로 AML1- ETO 유전자 재배열은 향후 BAL 환자의 치료 효과와 예후를 알 수 있는 지표로 사용될 수 있을 것이다.
Abstract
Biphenotypic acute leukemia (BAL) is a subtype of acute leukemia that expresses two different immunophenotypic lineages, most commonly myeloid and either B- or T-lymphoid lineages. This entity has been defined by a scoring system proposed by the European Group for the Immunological Characterization of Leukemias (EGIL). The prognosis of BAL is regarded as being worse than either acute lymphoid or myeloid leukemia that does not show lineage ambiguity. However, a treatment strategy for BAL has not yet been established. We experienced a case of BAL with the t(8;21) translocation, a favorable cytogenetic rearrangement in acute myeloid leukemia (AML). The patient was successfully treated with cytarabine and anthracycline for induction and consolidation. The quantitative value of the AML1-ETO gene decreased after achieving complete hematologic remission. Thus, the AML1-ETO gene rearrangement in BAL may be associated with an acceptable response to the treatment strategy for AML.
참고문헌 (12)
- Frater JL, "Biphenotypic acute leukemia with coexpression of CD79a and markers of myeloid lineage", Arch Pathol Lab Med, 127, pp.356~359 (2003)
- Bene MC, "The reliability and specificity of c-kit for the diagnosis of acute myeloid leukemias and undifferentiated leukemias", Blood, 92, pp.596~599 (1998)
- Matutes E, "Definition of acute biphenotypic leukemia", Haematologica, 82, pp.64~66 (1997)
- Killick S, "Outcome of biphenotypic acute leukemia", Haematologica, 84, pp.699~706 (1999)
- He G, "B-lymphoid and myeloid lineages biphenotypic acute leukemia with t(8;21)(q22;q22)", Int J Hematol, 87, pp.132~136 (2008)
- Kozlov I, "CD79a expression in acute myeloid leukemia t(8;21) and the importance of cytogenetics in the diagnosis of leukemia with immunophenotypic ambiguity", Cancer Genet Cytogenet, 163, pp.62~67 (2005)
- Aribi A, "Biphenotypic acute leukemia: a case series", Br J Haematol, 138, pp.213~216 (2007)
- Jaffe ES, "Acute myeloid leukemias. In Pathology and genetics of tumours of haematopoietic and lymphoid tissues. World Health Organization Classification of Tumours", pp.75~107 (2001), IARC Press
- Mulloy JC, "The AML1", ETO fusion protein promotes the expansion of human hematopoietic stem cell, 2002, pp.99, 15~23
- Klampfer L, "The AML1", ETO fusion protein activates transcription of BCL-2, 1996, pp.93, 14059~14064
- Krawter F, "Prognostic value of minimal residual disease quantification by real-time reverse transcriptase polymerase chain reaction in patient with core binding factor leukemia", J Clin Oncol, 21, pp.4413~4422 (2003)
- 박경분, "AML1", ETO-양성 급성골수성백혈병에서 역전사중합효소연쇄반응을 이용한 미세잔존질환 연속측정의 의의, 2003, pp.38(1), 3~23[DOI]