Nargund Ashwini R, Patil Geeta V, Raghuram C P, Venkataswamy Eswarachary
Department of Molecular Biology, R & D, Triesta Reference Laboratory, HCG, Bangalore, Karnataka, India.
Indian J Pathol Microbiol. 2012 Oct-Dec;55(4):574-7. doi: 10.4103/0377-4929.107828.
Acute promyelocytic leukemia (APL) is a subtype of acute myeloid leukemia (AML) in which abnormal promyelocytes predominate. APL is rare in children (approximately 10% of childhood AML) and is characterized by a higher incidence of hyperleukocytosis, an increased incidence of microgranular morphology, the presence of balanced t(15;17)(q22;q11.2-12) translocation, and more frequent occurrence of the PML-RARα isoforms bcr 2 and bcr 3 compared to adults. The cytomorphology of microgranular variant blasts is obviously different from AML M3 blasts; these cells have a nongranular or hypogranular cytoplasm or contain fine dust-like cytoplasmic azurophil granules that may not be apparent by light microscopy. This case report emphasizes the importance of a high index of suspicion for the diagnosis of APL, the hypogranular variant in particular. They are responsive to differentiation therapy with all trans-retinoic acid and complete remission in seen in >80% cases.
急性早幼粒细胞白血病(APL)是急性髓系白血病(AML)的一种亚型,其中异常早幼粒细胞占主导。APL在儿童中较为罕见(约占儿童AML的10%),其特征是白细胞增多症的发生率较高、微颗粒形态的发生率增加、存在平衡的t(15;17)(q22;q11.2 - 12)易位,并且与成人相比,PML - RARα异构体bcr 2和bcr 3的出现频率更高。微颗粒变异型原始细胞的细胞形态学与AML M3原始细胞明显不同;这些细胞具有无颗粒或颗粒减少的细胞质,或含有细小的尘样细胞质嗜天青颗粒,光学显微镜下可能不明显。本病例报告强调了对APL诊断保持高度怀疑的重要性,尤其是对颗粒减少变异型。它们对全反式维甲酸分化治疗有反应,超过80%的病例可实现完全缓解。