原发性骨髓纤维化误诊为脾功能亢进行脾切除1例并文献复习.PDFVIP

临床与病理杂志 2017, 37(4) 872 J Clin Path ol R e s doi: 10.3978/j.issn.2095-6959.2017.04.038 ·临床病例讨论· View this article at: /10.3978/j.issn.2095-6959.2017.04.038 原发性骨髓纤维化误诊为脾功能亢进行脾切除1例并文献复习 田洋，李燕，郝洪岭，杨洁，王素云 (河北省人民医院血液内科，石家庄 050051) [摘　要] 目的：学习原发性骨髓纤维化 ( pr imar y myelof ibrosi s ，PMF) 和脾功能亢进的诊断及鉴别诊断。 方法：对 1例PMF 患者进行临床资料、骨髓病理及基因检测总结，并结合文献对其诊断及鉴别诊断 进行学习。 结果：PMF 时骨髓造血功能差，肝、脾增大是身体代偿性的髓外造血，并非脾功能亢 进，不可盲目行脾切除术。 结论：对有原因不明的脾大患者，诊断依据要充分，需进一步行骨髓 涂片、骨髓活检以及基因学检查，排除PMF 。 [关键词] 骨髓纤维化；脾功能亢进；脾切除 A case report of splenectomy for a patient with primary myelofibrosis misdiagnosed as hypersplenism TIAN Yang, LI Yan, HAO Hongling, YANG Jie, WANG Suyun (Department of Hematology, Hebei General Hospital, Shijiazhuang 050051, China) Abstract Objective: To study the diagnosis and differential diagnosis of primary myelofibrosis (PMF). Methods: The clinical data, bone marrow pathological and gene studies of one case of PMF were organized. And then we learned its pathological diagnosis and differential diagnosis combined with literature. Results: The hematopoietic function is poor in PMF patients. Liver and spleen enlargement is the body compensatory reaction, but not because of hypersplenism. We cannot do splenectomy when the diagnosis is not clear. Conclusion: For those patients with unexplained splenomegaly, diagnosis evidence should be adequate. The clinical and histological features combined with bone marrow smear, biopsy and gene detection can contribute to its diagnosis