Острый панмиелоз с миелофиброзом: различия между версиями
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Версия от 21:14, 16 сентября 2014
| Acute panmyelosis with myelofibrosis | |
|---|---|
| МКБ-11 | 2A60.38 |
| МКБ-10 | C94.4 |
| МКБ-10-КМ | C94.4 |
| МКБ-9 | 238.79 |
| МКБ-9-КМ | 289.89[1] |
| МКБ-О | 9931/3 |
Acute panmyelosis with myelofibrosis (APMF) it is a poorly defined disorder that arises as either a clonal disorder, or following toxic exposure to the bone marrow.[2]
Клиническая симптоматика
Bone marrow biopsy shows abnormal megakaryocytes, macrocytic erythropoiesis, and defects in neutrophil production and fibrosis of the marrow (myelofibrosis).
Clinically patients present with reduction in the count of all blood cells (pancytopenia), a very few blasts in the peripheral blood and no or little spleen enlargement (splenomegaly).
Cells are usually CD34 positive.[3]
Прогноз и лечение
Median survival is about 9 months.
Autologous stem cell transplantation has been used in treatment.[4]
Терминологические разногласия
Controversy remains today whether this disorder is a subtype of acute myeloid leukemia or myelodysplastic syndromes; however, it is currently classified as a form of AML.[5][6]
Примечания
- ↑ Monarch Disease Ontology release 2018-06-29 — 2018-06-29 — 2018.
- ↑ Hoffman R, Benz E, Shattil S, Furie B, Cohen H. Hematology: Basic Principles and Practice. — 4th. — Churchill Livingstone, 2004.
- ↑ Orazi A, O'Malley DP, Jiang J; et al. (May 2005). "Acute panmyelosis with myelofibrosis: an entity distinct from acute megakaryoblastic leukemia". Mod. Pathol. 18 (5): 603—14. doi:10.1038/modpathol.3800348. PMID 15578075.
{{cite journal}}: Явное указание et al. в:|author=(справка)Википедия:Обслуживание CS1 (множественные имена: authors list) (ссылка) - ↑ Ngirabacu MC, Ravoet C, Dargent JL; et al. (December 2006). "Long-term follow-up of autologous peripheral blood stem cell transplantation in the treatment of a patient with acute panmyelosis with myelofibrosis". Haematologica. 91 (12 Suppl): ECR53. PMID 17194659.
{{cite journal}}: Явное указание et al. в:|author=(справка)Википедия:Обслуживание CS1 (множественные имена: authors list) (ссылка) - ↑ Thiele J, Kvasnicka HM, Schmitt-Graeff A (April 2004). "Acute panmyelosis with myelofibrosis". Leuk. Lymphoma. 45 (4): 681—7. doi:10.1080/10428190310001625692. PMID 15160939.
{{cite journal}}: Википедия:Обслуживание CS1 (множественные имена: authors list) (ссылка) - ↑ Thiele J, Kvasnicka HM, Zerhusen G; et al. (August 2004). "Acute panmyelosis with myelofibrosis: a clinicopathological study on 46 patients including histochemistry of bone marrow biopsies and follow-up". Ann. Hematol. 83 (8): 513—21. doi:10.1007/s00277-004-0881-8. PMID 15173958.
{{cite journal}}: Явное указание et al. в:|author=(справка)Википедия:Обслуживание CS1 (множественные имена: authors list) (ссылка)