Acute panmyelosis with myelofibrosis explained
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
See also
Notes and References
- Book: Hoffman R, Benz E, Shattil S, Furie B, Cohen H . Hematology: Basic Principles and Practice . Churchill Livingstone . 2004 . 4th.
- Orazi A, O'Malley DP, Jiang J . Acute panmyelosis with myelofibrosis: an entity distinct from acute megakaryoblastic leukemia . Mod. Pathol. . 18 . 5 . 603–14 . May 2005 . 15578075 . 10.1038/modpathol.3800348. etal. free .
- Ngirabacu MC, Ravoet C, Dargent JL . 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 . 17194659 . etal.
- Thiele J, Kvasnicka HM, Schmitt-Graeff A . Acute panmyelosis with myelofibrosis . Leuk. Lymphoma . 45 . 4 . 681–7 . April 2004 . 15160939 . 10.1080/10428190310001625692 . 20626984 .
- Thiele J, Kvasnicka HM, Zerhusen G . 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 . 15173958 . 10.1007/s00277-004-0881-8 . 2422812 . etal.
