ICD-10 C94.4 ICD-O 9931/3 | ICD-9-CM 238.79 | |
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.
Contents
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.
Prognosis and treatment
Median survival is about 9 months.
Autologous stem cell transplantation has been used in treatment.
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.