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词条 Acute panmyelosis with myelofibrosis
释义

  1. Signs and symptoms

  2. Prognosis and treatment

  3. Terminology

  4. See also

  5. References

  6. See also

  7. External links

{{Infobox medical condition (new)
| name = Acute panmyelosis with myelofibrosis
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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.[1]

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.[2]

Prognosis and treatment

Median survival is about 9 months.{{Citation needed|date=December 2008}}

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

  • List of hematologic conditions

References

1. ^{{cite book |vauthors =Hoffman R, Benz E, Shattil S, Furie B, Cohen H |title=Hematology: Basic Principles and Practice |publisher=Churchill Livingstone |year=2004 |edition=4th}}
2. ^{{cite journal |vauthors =Orazi A, O'Malley DP, Jiang J |title=Acute panmyelosis with myelofibrosis: an entity distinct from acute megakaryoblastic leukemia |journal=Mod. Pathol. |volume=18 |issue=5 |pages=603–14 |date=May 2005 |pmid=15578075 |doi=10.1038/modpathol.3800348|display-authors=etal}}
3. ^{{cite journal |vauthors =Ngirabacu MC, Ravoet C, Dargent JL |title=Long-term follow-up of autologous peripheral blood stem cell transplantation in the treatment of a patient with acute panmyelosis with myelofibrosis |journal=Haematologica |volume=91 |issue=12 Suppl |pages=ECR53 |date=December 2006 |pmid=17194659 |url=http://www.haematologica.org/cgi/pmidlookup?view=long&pmid=17194659|display-authors=etal}}
4. ^{{cite journal |vauthors =Thiele J, Kvasnicka HM, Schmitt-Graeff A |title=Acute panmyelosis with myelofibrosis |journal=Leuk. Lymphoma |volume=45 |issue=4 |pages=681–7 |date=April 2004 |pmid=15160939 |doi=10.1080/10428190310001625692 |url=http://informahealthcare.com/doi/abs/10.1080/10428190310001625692}}
5. ^{{cite journal |vauthors =Thiele J, Kvasnicka HM, Zerhusen G |title=Acute panmyelosis with myelofibrosis: a clinicopathological study on 46 patients including histochemistry of bone marrow biopsies and follow-up |journal=Ann. Hematol. |volume=83 |issue=8 |pages=513–21 |date=August 2004 |pmid=15173958 |doi=10.1007/s00277-004-0881-8 |display-authors=etal}}

See also

  • Acute myeloid leukemia
  • Panmyelosis
  • Myelofibrosis

External links

{{Medical resources
| DiseasesDB =
| ICD10 = {{ICD10|C|94|4|c|81}}
| ICD9 = {{ICD9|238.79}}
| ICDO = 9931/3
| OMIM =
| MedlinePlus =
| eMedicineSubj =
| eMedicineTopic =
| MeshID =
}}{{Myeloid malignancy}}

1 : Acute myeloid leukemia

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