Acute panmyelosis with myelofibrosis

Acute panmyelosis with myelofibrosis
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Field	Hematology, oncology
Symptoms
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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.<ref>,

 Hematology: Basic Principles and Practice, 
 4th edition, 
 Churchill Livingstone, 
 2004,</ref>

Signs and symptoms

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.<ref name="pmid15578075">,

 Acute panmyelosis with myelofibrosis: an entity distinct from acute megakaryoblastic leukemia, 
 Mod. Pathol., 
 
 Vol. 18(Issue: 5),
 pp. 603–14,
 DOI: 10.1038/modpathol.3800348,
 PMID: 15578075,</ref>

Prognosis and treatment

Median survival is about 9 months. ^{(December 2008)}

Autologous stem cell transplantation has been used in treatment.<ref name="pmid17194659">,

 Long-term follow-up of autologous peripheral blood stem cell transplantation in the treatment of a patient with acute panmyelosis with myelofibrosis, 
 Haematologica, 
 
 Vol. 91(Issue: 12 Suppl),
 pp. ECR53,
 
 PMID: 17194659,
 
 
 Full text,</ref>

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.<ref>,

 Acute panmyelosis with myelofibrosis, 
 Leuk. Lymphoma, 
 
 Vol. 45(Issue: 4),
 pp. 681–7,
 DOI: 10.1080/10428190310001625692,
 PMID: 15160939,
 
 
 Full text,</ref><ref>, 
 Acute panmyelosis with myelofibrosis: a clinicopathological study on 46 patients including histochemistry of bone marrow biopsies and follow-up, 
 Ann. Hematol., 
 
 Vol. 83(Issue: 8),
 pp. 513–21,
 DOI: 10.1007/s00277-004-0881-8,
 PMID: 15173958,</ref>

See also

• List of hematologic conditions

See also

• Acute myeloid leukemia
• Panmyelosis
• Myelofibrosis

External links

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