HLA-DR과 CD34 음성인 급성골수성백혈병의 빈도 및 특징: 전형적 급성골수성백혈병과 급성전골수구백혈병 사이의 중간적 특질
Frequency and Distinct Characteristics of Acute Myeloid Leukemia Lacking HLA-DR and CD34 Expression: Features Intermediate between Typical Acute Myeloid Leukemia and Acute Promyelocytic Leukemia
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배경: 본 연구의 목적은 HLA-DR-/CD34- AML (APL-유사 AML)의 빈도와 특성을 분석하고자 하였다. 방법: 새로 진단받은 683명의 AML 환자를 대상으로 하였다. 211명의 반복유전자이상 AML, 1명의 골수섬유증동반 급성범골수증, 2명의 다운증후군관련골수성백혈병, 그리고 중기분열세포를 획득하지 못한 2명의 환자를 제외한 나머지 467명을 다음과 같이 분류하였다: 제1군, HLA-DR+/CD34+ (전형적 AML); 제2군, HLA-DR+/CD34- 또는 HLA-DR-/CD34+; 제3군, APL-유사 AML. 결과: 제1군은 294명, 제2군은 133명, 제3군은 40명으로 분류되었다. 따라서 선별되지 않은 683명의 AML 환자군에서의 APL-유사 AML의 빈도는 5.9%이었다. APL-유사 AML은 제1군에 비해 백혈구 수와 골수의 모세포 분율, 정상핵형과 NPM1 돌연변이의 비율, CD33 양성세포의 분율, 섬유소(원)분해산물 및 D-이합체의 농도가 유의하게 높았으며, 복잡핵형, 단일염색체핵형, 세포유전학적 예후불량군의 빈도, CD13 양성세포의 분율, 그리고 섬유소원의 농도가 유의하게 낮았다. 골수 모세포 분율, CD33 양성세포 분율, 파종혈관내응고 점수의 경우 APL-유사 AML에서 전형적 AML과 APL 사이의 중간값을 보였다. 결론: 본 연구는 APL-유사 AML이 드물지 않고, 전형적 AML과 구별되는 특성을 보임을 제시하였다. APL-유사 AML과 APL 사이에는 병태생리적 공유점이 있을 것으로 추측되지만 심화 연구가 필요할 것으로 생각한다.
Abstract
Background: The objective of this study was to investigate the frequency and characteristics of HLA-DR-/CD34- acute myeloid leukemia (AML) also known as acute promyelocytic leukemia (APL)-like AML. Methods: This study included 683 newly diagnosed patients with AML. After exclusion of 211 patients with recurrent genetic abnormalities, one with acute panmyelosis with myelofibrosis, two with myeloid leukemia associated with Down syndrome, and two devoid of metaphase cells, we classified the remaining 467 patients as follows: group 1, HLA-DR+/CD34+ (typical AML); group 2, HLA-DR+/CD34- or HLA-DR-/CD34+; group 3, APL-like AML. Results: Group 1 comprised 294 patients, group 2 comprised 133, and group 3 comprised 40. Therefore, the frequency of APL-like AML among 683 unselected patients with AML was 5.9%. Group 3 patients had significantly higher leukocyte counts and bone marrow (BM) blast percentages, higher frequencies of normal karyotypes and NPM1 mutation, higher fractions of CD33-positive cells, higher concentrations of fibrin degradation products and D-dimers, lower frequencies of complex karyotypes, monosomal karyotypes and poor cytogenetic risk, lower fractions of CD13-positive cells, and lower fibrinogen concentrations, compared with group 1 patients. The values of the BM blast percentage, number of CD33-positive cells, and DIC score of the patients with APL-like AML were intermediate between those of the patients with typical AML and APL. Conclusions: This study demonstrates that APL-like AML is not uncommon, and it has characteristics distinguishable from those of typical AML. APL-like AML may have some pathophysiological relationships with APL, which need further investigation.
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