Article
[A case of prefibrotic primary myelofibrosis in a child with type-Ⅰ CALR gene mutation].
Zhonghua er ke za zhi = Chinese journal of pediatrics - 2 Oct 2023
Li S Q, Zhao Y Q, Zhao X L, Wang X G, Li S B, Song L L, Zhou Y J, Zang W T, Hao T, Yao X J
Abstract excerpt
患儿 男,8岁,以“头痛、呕吐”就诊,治疗过程中血小板计数最高2 312×109/L,骨髓病理提示有核细胞增生过度,可见异形巨核细胞,部分呈“云朵样”,骨髓间质未见网状纤维明显增生,CALR基因Ⅰ型突变阳性,诊断为儿童纤维化前期原发性骨髓纤维化,给予重组人干扰素α、羟基脲及抗血小板药物治疗,半年后复查骨髓病理,未见纤维化进展及骨髓转化。随访2年半,无明显肝脾肿大、血栓及出血事件。.
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