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目的:探讨急性全髓增殖症伴骨髓纤维化(APMF)的认识过程、临床特点及诊断进展。方法:对1例APMF的临床资料进行分析,并结合文献复习,为其鉴别诊断和治疗提供新的思路。结果:临床上起病急,以全血细胞减少所致的贫血、出血、感染为主要临床表现;脾脏不大,外周血可见原始细胞,而异形红细胞少见;骨髓粒系、红系、巨核系增生异常,可见病态造血,原始细胞在所有有核细胞中≥20%,伴有显著骨髓纤维化;治疗效果差。结论:APMF是一种少见的伴有骨髓纤维化的全髓增殖症,预后较差。
Objective: To investigate the cognitive process, clinical features and diagnosis of acute myelofibrosis with myelofibrosis (APMF). Methods: One case of APMF clinical data analysis, combined with the literature review, to provide a new idea for the differential diagnosis and treatment. Results: The clinical onset of acute, to pancytopenia caused by anemia, bleeding, infection as the main clinical manifestations; spleen is not large, visible in the peripheral blood of primitive cells, and rare shaped erythrocytes; bone marrow, erythroid, megakaryocyte hyperplasia Abnormalities, showing pathological hematopoietic, primitive cells in all nucleated cells ≥ 20%, with significant myelofibrosis; poor treatment. Conclusion: APMF is a rare myelofibrosis with myelofibrosis with poor prognosis.