Collinge Elodie, Tigaud Isabelle, Balme Brigitte, Gerland Luc-Marie, Sujobert Pierre, Carlioz Violette, Salles Gilles, Thomas Xavier, Paubelle Etienne
Department of Hematology, CHU UCL Namur, Belgium Department of Hematology Laboratory of Hematology Anatomopathology Department of Dermatology, Hospices Civils de Lyon, Centre Hospitalier Lyon Sud LBMC, ENS, CNRS UMR5239, Faculté de Médecine Lyon Sud, Pierre-Bénite, France.
Medicine (Baltimore). 2018 Feb;97(8):e9657. doi: 10.1097/MD.0000000000009657.
Acute promyelocytic leukemia (APL) is a curable subtype of acute myeloid leukemia. APL is currently treated with combination of all-trans retinoic acid (ATRA) and arsenic trioxide (ATO) resulting in the induction of apoptosis and differentiation of the leukemic cells. Differentiation syndrome (so-called ATRA syndrome) is the main life-threatening complication of induction therapy with these differentiating agents.
Herein, we report the case of a 49-year-old woman diagnosed with APL with, concomitantly, a bulky cutaneous lesion of 10 cm diameter with a red-to-purple background and a necrotic center, localized on her abdomen.
After 10 days of treatment, the cutaneous lesion became purulent. Fluorescence in situ hybridization (FISH) analysis performed on this pus confirmed the presence of malignant features in the involved granulocytes proving their origin from the differentiation of leukemic APL cells, as all the analyzed nuclei showed 2 promyelocytic leukemia (PML)-retinoic acid receptor-a (RARA) fusions signals.
The association by ATRA and ATO was continued.
Eventually, the evolution was favorable with healing in three weeks.
This case report therefore highlights the differentiation phenomenon of promyelocytic blasts within promyelocytic sarcoma with the ATRA-ATO combination and the efficacy of this drug association in resolving both the malignant sarcoma and a secondary local infection.
急性早幼粒细胞白血病(APL)是急性髓系白血病的一种可治愈亚型。目前,APL采用全反式维甲酸(ATRA)和三氧化二砷(ATO)联合治疗,可诱导白血病细胞凋亡和分化。分化综合征(所谓的ATRA综合征)是使用这些分化剂进行诱导治疗的主要危及生命的并发症。
在此,我们报告一例49岁女性,诊断为APL,同时腹部有一个直径10厘米的巨大皮肤病变,背景为红紫色,中心坏死。
治疗10天后,皮肤病变化脓。对该脓液进行荧光原位杂交(FISH)分析,证实受累粒细胞存在恶性特征,证明其起源于白血病APL细胞的分化,因为所有分析的细胞核均显示2个早幼粒细胞白血病(PML)-维甲酸受体-α(RARA)融合信号。
继续使用ATRA和ATO联合治疗。
最终病情发展良好,三周内痊愈。
因此,本病例报告突出了ATRA-ATO联合治疗在早幼粒细胞肉瘤中早幼粒细胞母细胞的分化现象,以及这种药物联合治疗在解决恶性肉瘤和继发性局部感染方面的疗效。