老年慢性ph阴性骨髓增生性肿瘤伴手术病理的综合诊治。临床观察

Yu. E. Ryabukhina, P. A. Zeynalova, O. I. Timofeeva, F. М. Abbasbeyli, T. V. Ponomarev, N. Kupryshina, A. G. Zhukov
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引用次数: 0

摘要

慢性骨髓增生性肿瘤(Chronic myeleloprolifative tumour, CMPN), ph阴性,是一种克隆性肿瘤,在造血干细胞水平上发生,以一种或多种造血途径的增殖为特征。目前,ph阴性CMPN组包括原发性血小板增多症、原发性骨髓纤维化、真性红细胞增多症、无法分类的骨髓增殖性肿瘤。Jak2 (V617F)、CALR和MPL基因突变的鉴定扩展了对ph阴性CMPN生物学特征的理解,并改善了髓系肿瘤的鉴别诊断。然而,临床实践中仍难以明确区分原发性髓纤维化、早期和原发性血小板增多症向高血小板增多性髓纤维化转化等疾病。血小板增多是血栓栓塞并发症的主要危险因素之一,尤其是在老年人中。本文报告了一例老年左股骨骨折患者的临床病例,该患者在ph阴性CMPN(骨髓纤维化)伴高水平血小板增多的情况下发生骨折,再加上强制长期固定和手术期间和术后出现血栓和出血的额外风险。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Combination approach to diagnosis and treatment of an elderly patient with chronic Ph-negative myeloproliferative neoplasm and concomitant surgical pathology. Clinical observation
Chronic myeloproliferative neoplasms (CMPN), Ph-negative, are of clonal nature, develop on the level of hematopoietic stem cell and are characterized by proliferation of one or more hematopoietic pathways. Currently, the group of Ph-negative CMPN includes essential thrombocythemia, primary myelofibrosis, polycythemia vera, myeloproliferative neoplasm unclassifiable.Identification of mutations in the Jak2 (V617F), CALR, and MPL genes extended understanding of biological features of Ph-negative CMPN and improved differential diagnosis of myeloid neoplasms. Nonetheless, clinical practice still encounters difficulties in clear separation between such disorders as primary myelofibrosis, early-stage and transformation of essential thrombocythemia into myelofibrosis with high thrombocytosis. Thrombocytosis is one of the main risk factors for thromboembolic complications, especially in elderly people.A clinical case of an elderly patient with fracture of the left femur developed in the context of Ph-negative CMPN (myelofibrosis) with high level of thrombocytosis is presented which in combination with enforced long-term immobilization and presence of additional risk created danger of thrombosis and hemorrhage during surgery and in the postoperative period.
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