Acute panmyelosis with myelofibrosis

Infobox_Disease
Name = Acute panmyelosis with myelofibrosis


Caption =
DiseasesDB =
ICD10 = ICD10|C|94|4|c|81
ICD9 = ICD9|238.79
ICDO = 9931/3
OMIM =
MedlinePlus =
eMedicineSubj =
eMedicineTopic =
MeshID =

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. [Hoffman R, Benz E, Shattil S, Furie B, Cohen H, "Hematology: Basic Principles and Practice", Churchill Livingstone, 4th ed, 2004.] 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). Median survival is about 9 months. Controversy remains today whether this disorder is a subtype of acute myeloid leukemia or myelodysplastic syndromes; however, it is currently classified as a form AML. [Thiele J, Kvasnicka HM, Schmitt-Graeff A., Acute panmyelosis with myelofibrosis., Leuk Lymphoma. 2004 Apr;45(4):681-7.] [Thiele J, Kvasnicka HM, Zerhusen G, Vardiman J, Diehl V, Luebbert M, Schmitt-Graeff A., Acute panmyelosis with myelofibrosis: a clinicopathological study on 46 patients including histochemistry of bone marrow biopsies and follow-up. Ann Hematol. 2004 Aug;83(8):513-21]

References

ee also

*Acute myeloid leukemia

Further reading

*