髓系肿瘤中获得性因子XIII缺乏:病例系列和文献回顾。

Alfadil Haroon, Mostafa F Mohammed Saleh, Ali Alahmari, Syed Osman, Ahmed Alotaibi, Hazzaa Alzahrani, Mahmoud Aljurf
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引用次数: 0

摘要

获得性因子XIII (FXIII)缺乏是一种罕见的疾病,可能与自身免疫性疾病和恶性疾病相关,出血风险高。在急性白血病中,获得性FXIII缺乏已被报道,FXIII的替代有助于控制显著出血。在这里,我们报告了四例髓系肿瘤获得性FXIII缺乏,并在两例患者的分子背景中伴随RUNX1突变。3例患者使用FXIII浓缩物替代FXIII, 1例患者使用冷冻沉淀替代FXIII,所有患者的出血并发症矫正均成功。研究FXIII缺乏与此类肿瘤分子异常的关系,有助于更好地理解和检测常见的病理生理途径。这可能有助于在诊断或治疗干预期间避免高危患者的严重出血。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Acquired factor XIII deficiency in myeloid neoplasms: case series and review of literature.

Acquired factor XIII (FXIII) deficiency is a rare disorder that could be associated with autoimmune and malignant disorders with a high risk of bleeding. In acute leukemias, acquired FXIII deficiency has been reported and replacement of FXIII helped to control significant bleeding. Here, we report four cases of myeloid neoplasms to have acquired FXIII deficiency with interesting concomitant RUNX1 mutation in the molecular background of two patients. Correction of bleeding complications was successful in all patients with FXIII substitution by FXIII concentrate in three patients and cryoprecipitate in one patient. Studying the association of FXIII deficiency with molecular abnormalities in such neoplasms is needed for better understanding and detection of common pathophysiologic pathways. This could help to avoid severe bleeding during diagnostic or therapeutic interventions in patients at risk.

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