关键词: Anomalies des fonctions plaquettaires Cerebral hemorrhage Chronic neutrophilic leukemia Hemorrhagic syndrome Hémorragie intracérébrale Leucémie chronique à polynucléaires neutrophiles Platelet function defect Syndrome hémorragique

Mesh : Aged Cerebral Hemorrhage / diagnosis etiology pathology Fatal Outcome Humans Leukemia, Neutrophilic, Chronic / complications diagnosis pathology Male Platelet Aggregation

来  源:   DOI:10.1016/j.revmed.2020.03.012   PDF(Sci-hub)

Abstract:
BACKGROUND: Chronic neutrophilic leukemia (CNL) is a rare myeloproliferative syndrome characterized by a significant increase in mature neutrophils. One of the most serious complications is the occurrence of bleeding events, which may sometimes lead to death.
METHODS: A 75-year-old patient presented with CNL, complicated by a severe bleeding phenotype. Biological investigations revealed platelet function defect and increase in neutrophil elastase. The follow-up was marked by an intracranial hemorrhage leading to the patient\'s death 7 months after diagnosis.
CONCLUSIONS: This bleeding phenotype has been reported several times in patients with CNL. However, the pathophysiological mechanisms that cause bleeding are not yet fully understood.
摘要:
背景:慢性中性粒细胞白血病(CNL)是一种罕见的骨髓增生综合征,其特征是成熟中性粒细胞的显着增加。最严重的并发症之一是出血事件的发生,有时会导致死亡。
方法:一名75岁患者出现CNL,并发严重出血表型。生物学研究显示血小板功能缺陷和中性粒细胞弹性蛋白酶增加。随访的特点是颅内出血导致患者在诊断后7个月死亡。
结论:在CNL患者中已经多次报道了这种出血表型。然而,引起出血的病理生理机制尚不完全清楚.
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