Acute panmyelosis with myelofibrosis
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Acute panmyelosis with myelofibrosis
| Acute panmyelosis with myelofibrosis | |
|---|---|
| Classification and external resources | |
| ICD-10 | C94.4 |
| ICD-9 | 238.79 |
| ICD-O: | 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. [1]
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Contents
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Presentation
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.[2]
Prognosis and treatment
Median survival is about 9 months.[citation needed]
Autologous stem cell transplantation has been used in treatment.[3]
Terminology
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. [4][5]
See also
References
- ^ Hoffman R, Benz E, Shattil S, Furie B, Cohen H (2004). Hematology: Basic Principles and Practice (4th ed.). Churchill Livingstone.
- ^ 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.
- ^ 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. http://www.haematologica.org/cgi/pmidlookup?view=long&pmid=17194659.
- ^ 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. http://informahealthcare.com/doi/abs/10.1080/10428190310001625692.
- ^ 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.
See also
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