Lee Hee-Jeong, Park Hyung-Jin, Kim Hyun-Wook, Park Sang-Gon
Department of Internal Medicine, Hematology-Oncology, Chosun University Hospital, Gwangju, Korea.
Blood Res. 2013 Dec;48(4):250-3. doi: 10.5045/br.2013.48.4.250. Epub 2013 Dec 24.
Acute promyelocytic leukemia (APL) is an acute myeloid leukemia (AML) subtype with distinctive cell morphology, molecular presentation, clinical course, and treatment. About 90% of APL patients present with hemorrhagic complications due to disseminated intravascular coagulation (DIC). When APL is suspected, all-trans retinoic acid (ATRA) treatment is recommended even before confirmation by molecular tests. Specific criteria for differentiating unconfirmed APL from other AML subtypes with DIC are currently lacking. We aimed to achieve the early diagnosis of APL from other AML types with DIC by restricting the DIC criteria.
We retrospectively analyzed 29 patients newly diagnosed with AML accompanied by DIC from January 2005 to January 2013.
Fibrin degradation products (FDP) (77.7 µg/mL vs. 23.7 µg/mL, p=0.026), D-dimer (7,376.2 ng/mL vs. 1,315.2 ng/mL, p=0.018), and TIBC (264.4 µg/dL vs. 206.8 µg/dL, P=0.046) were higher, while fibrinogen (133.8 mg/dL vs. 373.2 mg/dL, p<0.001), WBC (14.988×10(9)/L vs. 70.755×10(9)/L, p=0.015), and ESR (7.1 mm/h vs. 50.0 mm/h, p <0.001) were lower in APL patients than in the patients with other AML subtypes. FDP ≥27 µg/mL, D-dimer ≥2,071 ng/mL, and fibrinogen ≤279 mg/dL were our threshold values. These markers may be characteristic to APL and helpful in presumptive diagnosis.
APL may be differentiated from other AML subtypes by core markers of DIC (FDP, D-dimer, and fibrinogen). We suggest that clinicians set new diagnostic thresholds by restricting the DIC criteria. These findings support the early initiation of ATRA, prior to confirmation by PML-RARA molecular testing.
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,具有独特的细胞形态、分子表现、临床病程及治疗方法。约90%的APL患者因弥散性血管内凝血(DIC)出现出血并发症。当怀疑为APL时,即使在分子检测确诊之前,也建议使用全反式维甲酸(ATRA)进行治疗。目前缺乏将未经确诊的APL与其他伴有DIC的AML亚型进行鉴别的具体标准。我们旨在通过限制DIC标准实现APL与其他伴有DIC的AML类型的早期诊断。
我们回顾性分析了2005年1月至2013年1月期间新诊断为伴有DIC的AML的29例患者。
APL患者的纤维蛋白降解产物(FDP)(77.7μg/mL对23.7μg/mL,p = 0.026)、D - 二聚体(7376.2 ng/mL对1315.2 ng/mL,p = 0.018)和总铁结合力(TIBC)(264.4μg/dL对206.8μg/dL,P = 0.046)较高,而纤维蛋白原(133.8 mg/dL对373.2 mg/dL,p <0.001)、白细胞(14.988×10⁹/L对70.755×10⁹/L,p = 0.015)和血沉(ESR)(7.1 mm/h对50.0 mm/h,p <0.001)低于其他AML亚型患者。FDP≥27μg/mL、D - 二聚体≥2071 ng/mL和纤维蛋白原≤279 mg/dL是我们的阈值。这些标志物可能是APL的特征性表现,有助于初步诊断。
APL可通过DIC的核心标志物(FDP、D - 二聚体和纤维蛋白原)与其他AML亚型相鉴别。我们建议临床医生通过限制DIC标准设定新的诊断阈值。这些发现支持在通过PML - RARA分子检测确诊之前尽早开始使用ATRA治疗。