Aslan Deniz
Section of Hematology, Department of Pediatrics, Faculty of Medicine, Gazi University, Ankara, Turkey.
J Pediatr Hematol Oncol. 2016 Nov;38(8):e326-e328. doi: 10.1097/MPH.0000000000000602.
Inherited giant platelet disorders are a subgroup of congenital thrombocytopenias characterized by decreased platelet counts along with macroplatelets and variable bleeding symptoms. Harris platelet syndrome, a newly described rare entity, is a subtype of inherited giant platelet disorders and is characterized by mild-to-severe thrombocytopenia, macroplatelets, and no bleeding manifestations. This entity was observed incidentally in healthy blood donors from India in the early 2000s, and the reported cases to date have without exception originated from the same region of the Indian subcontinent. We herein report the occurrence of Harris platelet syndrome in patients from a different ethnogeographic origin.
遗传性巨大血小板疾病是先天性血小板减少症的一个亚组,其特征是血小板计数减少,伴有大血小板和不同程度的出血症状。哈里斯血小板综合征是一种新描述的罕见病症,是遗传性巨大血小板疾病的一种亚型,其特征为轻度至重度血小板减少、大血小板,且无出血表现。21世纪初在印度健康献血者中偶然发现了这一病症,迄今为止报告的病例无一例外都来自印度次大陆的同一地区。我们在此报告了不同种族地理来源的患者中出现哈里斯血小板综合征的情况。