PDF(Pubmed)
Abstract：
Factor VII (FVII) deficiency is a rare bleeding disorder that can be classified as congenital or acquired, and the majority of acquired cases are due to vitamin K deficiency or liver disease. Isolated acquired FVII deficiency is a rare occurrence and has been associated with inhibitors or auto-antibodies. Here, we describe a patient with polycythemia vera who developed systemic mastocytosis and FVII deficiency simultaneously. FVII deficiency was not caused by inhibitors and improved with antineoplastic treatment. Acquired FVII deficiency has been reported in cases of sepsis, possibly due to proteolytic degradation induced by the activation of monocytes or endothelial cells. Malignancies have been shown to cause a depletion in circulating FVII through the direct binding of cancer cells. This case report suggests a potential association between SM associated with a hematological neoplasm (SM-AHN) and acquired FVII deficiency. Further evaluations are recommended in patients with systemic mastocytosis to gain a better understanding of the relationship between pathological mast cells and clotting factor concentrations.
摘要：
凝血因子VII（FVII）缺乏症是一种罕见的出血性疾病，可分为先天性或获得性。大多数获得性病例是由于维生素K缺乏或肝脏疾病。孤立的获得性FVII缺乏症很少发生，并且与抑制剂或自身抗体有关。这里,我们描述了一例真性红细胞增多症患者同时出现系统性肥大细胞增多症和FVII缺乏症.FVII缺乏不是由抑制剂引起的，并且通过抗肿瘤治疗得到改善。在脓毒症病例中已经报道了获得性FVII缺乏症，可能是由于单核细胞或内皮细胞活化诱导的蛋白水解降解。已显示恶性肿瘤通过癌细胞的直接结合引起循环FVII的消耗。该病例报告表明，与血液学肿瘤（SM-AHN）相关的SM与获得性FVII缺乏症之间存在潜在关联。建议对全身性肥大细胞增多症患者进行进一步评估，以更好地了解病理性肥大细胞与凝血因子浓度之间的关系。
