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JAK2 阳性的非费城染色体阳性骨髓增殖性肿瘤

JAK2-positive Philadelphia-negative myeloproliferative neoplasms.

作者信息

Sharma Anjali, Buxi Gurdeep, Marwah Sadhna, Yadav Rajbala

机构信息

Department of Pathology, Dr. Ram Manohar Lohia Hospital, PGIMER, New Delhi, India.

出版信息

Indian J Pathol Microbiol. 2011 Jan-Mar;54(1):117-20. doi: 10.4103/0377-4929.77355.

Abstract

The recent discovery of the JAK2 mutations has rekindled interest in the approach to classic BCR/ABL-negative myeloproliferative neoplasms (MPNs) in terms of both diagnostic evaluation and treatment. However, additional clinical, laboratory and histological parameters play a key role to allow diagnosis and subclassification, regardless of whether JAK2 V617F mutation is present or not. Here are two cases which incidentally presented with splenomegaly and moderate leukocytosis, and were diagnosed as MPN-primary myelofibrosis (PMF) in prefibrotic phase and polycythemia vera (PV), respectively, using revised World Health Organization (WHO) 2008 criteria.

摘要

JAK2突变的近期发现,在经典的BCR/ABL阴性骨髓增殖性肿瘤(MPN)的诊断评估和治疗方面,重新激起了人们的兴趣。然而,无论是否存在JAK2 V617F突变,额外的临床、实验室和组织学参数对于诊断和亚型分类都起着关键作用。以下是两例偶然出现脾肿大和中度白细胞增多的病例,分别根据2008年修订的世界卫生组织(WHO)标准,被诊断为MPN-原发性骨髓纤维化(PMF)纤维化前期和真性红细胞增多症(PV)。

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