Department of Biological Hematology, Aziza Othmana Hospital, Tunis, Tunisia.
UR14ES1 University of Tunis El Manar, Tunisia.
Lab Med. 2022 Sep 1;53(5):e120-e122. doi: 10.1093/labmed/lmab120.
Factor VII (FVII) deficiency is the most common among all rare inherited bleeding disorders. However, acquired FVII deficiency (aFVIID) is uncommon. Only few cases in the literature have been reported. Herein, we present a case of an aFVIID associated with acute myeloid leukemia (AML), along with a literature review regarding this condition. A 50 year old Arab male patient was diagnosed with AML at the hematology department of our institution. At admission, coagulation tests showed a prolonged prothrombin time (PT) with a normal activated partial thromboplastin time (aPTT) and a slightly elevated fibrinogen level. Prothrombin complex coagulation factors dosing (PCCFD) revealed a decrease only in FVII levels. The patient, however, did not experience any bleeding. The evolution of the health of the patient was marked by a normalization of PT and FVII levels and complete remission.
VII 因子(FVII)缺乏症是所有罕见遗传性出血性疾病中最常见的。然而,获得性 FVII 缺乏症(aFVIID)并不常见。仅有少数文献报道过此类病例。本文报道了一例与急性髓系白血病(AML)相关的 aFVIID,并对该疾病进行了文献复习。一名 50 岁的阿拉伯男性患者在我院血液科被诊断为 AML。入院时,凝血试验显示凝血酶原时间(PT)延长,活化部分凝血活酶时间(aPTT)正常,纤维蛋白原水平略有升高。凝血酶原复合物凝血因子剂量测定(PCCFD)显示仅 FVII 水平降低。然而,患者并未出现任何出血症状。患者的病情演变以 PT 和 FVII 水平的正常化以及完全缓解为标志。