将稳态镰状细胞病成人患者的常规止血情况与实验室值、不同表型之间以及妊娠期间的常规止血情况进行比较:回顾性、描述性研究

EJHaem Pub Date : 2024-08-20 DOI:10.1002/jha2.876
Comlan Affo, Carine Schmidt, Antoine Bosquet, Bénédicte Dumont, Isabelle Mahé
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引用次数: 0

摘要

镰状细胞病是一种因血红蛋白异常而导致的罕见遗传病。稳态止血是指≥2个月无血管闭塞性危象,但文献中对稳态止血的描述很少。我们报告了处于稳定状态的镰状细胞病(SCD)患者的常规止血情况,包括妊娠期和表型。这项回顾性研究收集了 2010 年至 2021 年期间的数据。我们从病历中收集了常规止血参数(凝血酶原时间 [PT]、活化部分凝血活酶时间 [aPTT] 和血小板)的数据,并将其与实验室标准(包括孕期)进行了比较;将 HbSS 表型与 HbSC、HbSB°地中海贫血和 HbSB+ 地中海贫血表型进行了比较。其中排除了七名服用抗凝药物的患者(15 家日间医院)。11名患者(17家日间医院)为孕妇。无论是否怀孕,常规止血参数的平均值都在正常值范围内。妊娠期平均 PT 值较低(12.3 ± 0.6 秒 vs. 13.2 ± 1.0 秒;P = .01)。与 HbSC 组相比,HbSS 组男性和非孕妇的 PT 和血小板计数更高(P = .01),aPTT 更低(P = .03)。孕妇和非孕妇的 PT 值有明显差异,HbSS、HbSC 和 HbSB+ 地中海贫血表型的 PT、aPTT 和血小板计数也有差异。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Routine hemostasis profile in steady state sickle cell disease adult patients compared to laboratory values, between phenotypes and during pregnancy: A retrospective, descriptive study

Routine hemostasis profile in steady state sickle cell disease adult patients compared to laboratory values, between phenotypes and during pregnancy: A retrospective, descriptive study

Sickle cell disease is a rare genetic disease resulting from an abnormality in hemoglobin. Hemostasis in the steady state, defined as ≥2 months without vaso-occlusive crises, is poorly described in the literature. We report the routine hemostasis profile in steady state patients with sickle cell disease (SCD), including during pregnancy and according to phenotype. This retrospective study collected data over the period 2010 to 2021. Data on routine hemostasis parameters (prothrombin time [PT] activated partial thromboplastin time [aPTT], and platelets) were collected from medical records and were compared with laboratory norms including during pregnancy; the HbSS phenotype was compared with the HbSC, HbSB°thalassemia, and HbSB+thalassemia phenotypes. we included 119 adults (representing 190 day-hospitals) with SCD who had attended at least one checkup in the steady state. Seven patients (15 day-hospitals) on anticoagulants were excluded. Eleven (17 day-hospitals) were pregnant. Mean routine hemostasis parameters were within normal values regardless of pregnancy. Mean PT was lower during pregnancy (12.3 ± 0.6 s vs. 13.2 ± 1.0 s; P = .01). PT and platelet counts were higher (P = .01) and aPTT was lower (P = .03) in men and nonpregnant women in the HbSS group compared with those in the HbSC group. routinely collected hemostasis parameters in steady state patients were within normal laboratory values, including in pregnant women. PT values differed significantly between pregnant women and nonpregnant women, and PT, aPTT, and platelet counts differed between HbSS, HbSC, and HbSB+thalassemia phenotypes.

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