| Acute panmyelosis with myelofibrosis | |
|---|---|
| Specialty | Hematology, oncology |
Acute panmyelosis with myelofibrosis (APMF) is a poorly defined disorder that arises as either a clonal disorder, or following toxic exposure to the bone marrow. [1]
Signs and symptoms
Bone 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), very few blasts in the peripheral blood, and no or little spleen enlargement ( splenomegaly).
Cells are usually CD34 positive. [2]
Prognosis and treatment
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.
- ^ 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. S2CID 20626984.
- ^ 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. S2CID 2422812.