Coagulopathy in pediatric acute promyelocytic leukemia in Bangladesh: A single-center, prospective study

Eshita Reza Khan , Ferdousi Begum , Afiqul Islam , K.M. Ahasan Ahmed
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Abstract

Background

Acute promyelocytic leukemia (APL) is one of the most curable subtypes of acute myeloid leukemia in childhood. It usually presents with a characteristic coagulopathy. The aim of the study was to determine the extent and outcome of this coagulation disorder.

Methods

This prospective observational study was conducted at Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, and included PML:RAR-α (Promyelocytic Leukemia-Retinoic Acid Receptor-α) positive APL cases. At presentation, all patients were assessed with coagulation parameters and followed up clinically and hematologically while treated with ATRA (all-trans-retinoic acid)-based chemotherapy. The presence of disseminated intravascular coagulation (DIC) was determined using the DIC scoring system of the International Society on Thrombosis and Hemostasis (ISTH).

Results

Among 20 APL cases, the mean DIC score was 5.75 ± 0.6. DIC was detected in 90% (n = 18/20) of the patients. The incidence of fatal thrombo-hemorrhagic complications was 15% (n = 3/20). Though hemorrhagic complications are common, thrombosis may also occur in pediatric APL.

孟加拉国小儿急性早幼粒细胞白血病患者的凝血功能障碍:单中心前瞻性研究
背景急性早幼粒细胞白血病(APL)是儿童急性髓细胞白血病中最容易治愈的亚型之一。它通常伴有特征性凝血病。这项前瞻性观察研究在达卡的班加班杜-谢赫-穆吉布医科大学(BSMMU)进行,纳入了 PML:RAR-α(早幼粒细胞白血病-Retinoic Acid Receptor-α)阳性 APL 病例。所有患者在发病时都接受了凝血指标评估,并在接受以 ATRA(全反式维甲酸)为基础的化疗时接受了临床和血液学随访。采用国际血栓与止血学会(ISTH)的 DIC 评分系统确定是否存在弥散性血管内凝血(DIC)。90%的患者(n = 18/20)发现了 DIC。致命性血栓-出血并发症的发生率为 15%(3/20)。虽然出血并发症很常见,但血栓形成也可能发生在小儿 APL 中。
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