急性全髓細胞增生症伴骨髓纖維化
| 急性全髓細胞增生症伴骨髓纖維化 | |
|---|---|
| 類型 | 急性骨髓性白血病 |
| 分類和外部資源 | |
| 醫學專科 | 血液學、腫瘤學 |
| ICD-11 | 2A60.38 |
| ICD-10 | C94.4 |
| ICD-9-CM | 289.89 |
| Orphanet | 86843 |
急性全髓細胞增生症伴骨髓纖維化（APMF）是一種非常罕見的髓系惡性腫瘤，是由克隆性疾病或骨髓接觸毒性物質而引起的[1]。
體徵和症狀
[編輯]骨活檢顯示巨核細胞異常、大細胞性紅血球生成、中性粒細胞生成缺陷和骨髓纖維化；臨床上，患者表現為全血細胞計數減少，外周血中原始細胞很少，脾臟無腫大或很小[2][3]。細胞通常為CD34陽性[4]。
預後和治療
[編輯]自體幹細胞移植已用於治療[5]。
分類爭議
[編輯]關於這種疾病是急性髓系白血病還是骨髓增生異常症候群的亞型，至今仍存在爭議；不過，根據世界衛生組織血液淋巴腫瘤分類標準，它目前被歸類為急性骨髓性白血病下[6][7]，約占急性骨髓性白血病發病率的1.2%[8]。
參考資料
[編輯]- ^ Hoffman R, Benz E, Shattil S, Furie B, Cohen H. Hematology: Basic Principles and Practice 4th. Churchill Livingstone. 2004.
- ^ 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.
- ^ 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 （英語）.
- ^ 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
.
- ^ 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.
- ^ 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.
- ^ 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.
- ^ 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 （英語）.
