真性红细胞增多症病程中发生的急性淋巴细胞白血病：一例报告并文献复习
Acute Lymphoblastic Leukemia in the Course of Polycythemia Vera: A Case Report and Review of Literature.
作者信息
Wu Dijiong, Ye Baodong, Shen Jianping, Peng Laijun, Zhong Zhiqiang, Zhou Yuhong
机构信息
Department of Hematology, Zhejiang Provincial Hospital of Traditional Chinese Medicine (National Clinical Research Base of TCM), Youdian Road No. 54, Hangzhou, 310006 China.
Department of Clinical Laboratory, Zhejiang Provincial Hospital of Traditional Chinese Medicine (National Clinical Research Base of TCM), Hangzhou, China.
出版信息
Indian J Hematol Blood Transfus. 2016 Jun;32(Suppl 1):50-5. doi: 10.1007/s12288-015-0598-y. Epub 2015 Sep 21.
INTRODUCTION
Polycythemia vera (PV) is one of the most common forms of myeloproliferative neoplasms. Acute myeloid leukemia secondary to PV is well reported, and the mechanism has been clarified to some extent. Only a limited number of cases have been reported about the development of acute lymphoblastic leukemia (ALL) in the course of PV, and the possible underlying mechanism has not been explored well.
CASE PRESENTATION
A 75-year-old patient who developed ALL 3 years after he was diagnosed with PV. The presence of remarkable splenomegaly, typical immunophenotyping of the peripheral blood and increased expression of serum fibrosis markers indicated the existence of extramedullary hematopoiesis which may ascribe to myelofibrosis. After the treatment of dosage-modulated chemotherapy, the patient got complete remission.
CONCLUSION
The JAK2 mutation may the underlying factor that contributes to the development of ALL, and the existence of MF may indicate the progression to post- polycythemic MF, which may be a risk factor for the accelerated transformation.
引言
真性红细胞增多症（PV）是骨髓增殖性肿瘤最常见的形式之一。继发于PV的急性髓系白血病已有大量报道，其机制在一定程度上已得到阐明。关于PV病程中发生急性淋巴细胞白血病（ALL）的病例报道有限，其潜在机制尚未得到充分探讨。
病例报告
一名75岁患者在诊断为PV 3年后发生ALL。显著脾肿大、外周血典型免疫表型以及血清纤维化标志物表达增加提示存在可能归因于骨髓纤维化的髓外造血。经剂量调整化疗治疗后，患者完全缓解。
结论
JAK2突变可能是导致ALL发生的潜在因素，MF的存在可能表明已进展为真性红细胞增多症后骨髓纤维化，这可能是加速转化的危险因素。
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