原发性骨髓纤维化转化为急性巨核细胞白血病1例及文献复习

目的报道1例由原发性骨髓纤维化转化的急性巨核细胞白血病（M7），并讨论转化前后骨髓MRI表现的改变。方法对我院1例由原发性骨髓纤维化转化为急性巨核细胞白血病的患者在转变前后进行骨髓细胞形态学、细胞遗传学检测，并同步进行髂骨骨髓的MRI检查；使用标准IDA、HA、MA、ACLA等方案序贯治疗，并随访至今。结果在患者处于原发性骨髓纤维化阶段时，髂骨骨髓在MRI上表现为T1WI及T2WI上的片状低信号；而在转化后相同部位骨髓在MRI上表现为T1WI片状低信号，T2WI上散在高信号。该患者（染色体检测提示核型正常）经序贯化疗后达到完全缓解至今。结论原发性骨髓纤维化与急性巨核细胞性白血病在MRI上骨髓表现有明显的改变；核型正常的由骨髓纤维化转变的M7经合理序贯化疗后仍能达到完全缓解。

A case report of acute megakaryocytic leukemia transformed from chronic idiopathic myelofibrosis with a review in literature

Objective One patient with acute megakaryocytic leukemia(M7)transformed from chronic idiopathic myelofibrosis(CIMF)was reported and the changes of MRI image were demonstrated before and after disease transformation.Methods Bone marrow cytology,cytogenetic and MRI image were performed before and after transformation.Standard chemotherapeutic protocols of IDA,HA,MA,ACLA were consecutively implied on this patient.Follow-up had been done till getting complete remission.Results There was only low MR signal on both T1WI and T2WI of iliac bone marrow when the patient suffered chronic idiopathic myelofibrosis.After disease transformation,MR image showed high signal on T2WI except low signal on T1WI in iliac bone marrow.Karyotype analysis had been normal in whatever disease status.Conclusion Distinct change was found between these two diseases on MR image of iliac bone marrow.The M7 transformed from CIMF with normal karyotype can also achieve complete remission after rationale consecutive chemotherapy.