急性全髓细胞增生症伴骨髓纤维化

急性全髓细胞增生症伴骨髓纤维化
类型急性骨髓性白血病
分类和外部资源
醫學專科血液学、肿瘤学
ICD-112A60.38
ICD-10C94.4
ICD-9-CM289.89
Orphanet86843
[编辑此条目的维基数据]

急性全髓细胞增生症伴骨髓纤维化（APMF）是一种非常罕见的髓系恶性肿瘤，是由克隆性疾病或骨髓接触毒性物质而引起的[1]。

体征和症状

骨活检显示巨核细胞异常、大细胞性红细胞生成、中性粒细胞生成缺陷和骨髓纤维化；临床上，患者表现为全血细胞计数减少，外周血中原始细胞很少，脾脏无肿大或很小[2][3]。细胞通常为CD34阳性[4]。

预后和治疗

自体干细胞移植已用于治疗[5]。

分类争议

关于这种疾病是急性髓系白血病还是骨髓增生异常综合征的亚型，至今仍存在争议；不过，根据世界卫生组织血液淋巴肿瘤分类标准，它目前被归类为急性骨髓性白血病下[6][7]，约占急性骨髓性白血病发病率的1.2%[8]。

参考资料

  1. ^ Hoffman R, Benz E, Shattil S, Furie B, Cohen H. Hematology: Basic Principles and Practice 4th. Churchill Livingstone. 2004. 
  2. ^ Bae, E.; Park, C.‐J.; Cho, Y.‐U.; Seo, E.‐J.; Chi, H.‐S.; Jang, S.; Lee, K.‐H.; Lee, J.‐H.; Lee, J.‐H.; Suh, J.‐J.; IM, H.‐J. Differential diagnosis of myelofibrosis based on <scp>WHO</scp> 2008 criteria: Acute panmyelosis with myelofibrosis, acute megakaryoblastic leukemia with myelofibrosis, primary myelofibrosis and myelodysplastic syndrome with myelofibrosis. International Journal of Laboratory Hematology. 2013-05-22, 35 (6). ISSN 1751-5521. doi:10.1111/ijlh.12101. 
  3. ^ Suvajdzic, N; Marisavljevic, D; Kraguljac, N; Pantic, M; Djordjevic, V; Jankovic, G; Cemerikic-Martinovic, V; Colovic, M. Acute Panmyelosis with Myelofibrosis: Clinical, Immunophenotypic and Cytogenetic Study of Twelve Cases. Leukemia & Lymphoma. 2004-09, 45 (9). ISSN 1042-8194. doi:10.1080/10428190410001683688 （英语）. 
  4. ^ Orazi A, O'Malley DP, Jiang J, et al. Acute panmyelosis with myelofibrosis: an entity distinct from acute megakaryoblastic leukemia. Mod. Pathol. May 2005, 18 (5): 603–14. PMID 15578075. doi:10.1038/modpathol.3800348 可免费查阅. 
  5. ^ Ngirabacu MC, Ravoet C, Dargent JL, et al. Long-term follow-up of autologous peripheral blood stem cell transplantation in the treatment of a patient with acute panmyelosis with myelofibrosis. Haematologica. December 2006, 91 (12 Suppl): ECR53. PMID 17194659. 
  6. ^ Thiele J, Kvasnicka HM, Schmitt-Graeff A. Acute panmyelosis with myelofibrosis. Leuk. Lymphoma. April 2004, 45 (4): 681–7. PMID 15160939. S2CID 20626984. doi:10.1080/10428190310001625692. 
  7. ^ Thiele J, Kvasnicka HM, Zerhusen G, et al. Acute panmyelosis with myelofibrosis: a clinicopathological study on 46 patients including histochemistry of bone marrow biopsies and follow-up. Ann. Hematol. August 2004, 83 (8): 513–21. PMID 15173958. S2CID 2422812. doi:10.1007/s00277-004-0881-8. 
  8. ^ Suvajdzic, N; Marisavljevic, D; Kraguljac, N; Pantic, M; Djordjevic, V; Jankovic, G; Cemerikic-Martinovic, V; Colovic, M. Acute Panmyelosis with Myelofibrosis: Clinical, Immunophenotypic and Cytogenetic Study of Twelve Cases. Leukemia & Lymphoma. 2004-09, 45 (9). ISSN 1042-8194. doi:10.1080/10428190410001683688 （英语）. 

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