Acute panmyelosis with myelofibrosis
 | It has been suggested that this article be merged with Panmyelosis. (Discuss) Proposed since September 2015. |
| Acute panmyelosis with myelofibrosis | |
|---|---|
| Specialty | Hematology and oncology |
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]
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
- ^ Hematology: Basic Principles and Practice (4th ed.). Churchill Livingstone. 2004.
{{cite book}}: Cite uses deprecated parameter|authors=(help) - ^ "Acute panmyelosis with myelofibrosis: an entity distinct from acute megakaryoblastic leukemia". Mod. Pathol. 18 (5): 603–14. May 2005. doi:10.1038/modpathol.3800348. PMID 15578075.
{{cite journal}}: Cite uses deprecated parameter|authors=(help) - ^ "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. December 2006. PMID 17194659.
{{cite journal}}: Cite uses deprecated parameter|authors=(help) - ^ "Acute panmyelosis with myelofibrosis". Leuk. Lymphoma. 45 (4): 681–7. April 2004. doi:10.1080/10428190310001625692. PMID 15160939.
{{cite journal}}: Cite uses deprecated parameter|authors=(help) - ^ "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. August 2004. doi:10.1007/s00277-004-0881-8. PMID 15173958.
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