Acute panmyelosis with myelofibrosis

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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]

Contents

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

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References

  1. Hoffman R, Benz E, Shattil S, Furie B, Cohen H (2004). Hematology: Basic Principles and Practice (4th ed.). Churchill Livingstone.
  2. 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.
  3. 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.
  4. 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.
  5. 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.

See also