Acute panmyelosis with myelofibrosis: Difference between revisions
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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>Hoffman R, Benz E, Shattil S, Furie B, Cohen H |
'''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>{{cite book |author=Hoffman R, Benz E, Shattil S, Furie B, Cohen H |title=Hematology: Basic Principles and Practice |publisher=Churchill Livingstone |year=2004 |edition=4th}}</ref> |
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==Presentation== |
==Presentation== |
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Median survival is about 9 months.{{Fact|date=December 2008}} |
Median survival is about 9 months.{{Fact|date=December 2008}} |
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Autologous stem cell transplantation has been used in treatment.<ref name="pmid17194659">{{cite journal |author=Ngirabacu MC, Ravoet C, Dargent JL, ''et al.'' |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 |year=2006 |month=December |pmid=17194659 |
Autologous stem cell transplantation has been used in treatment.<ref name="pmid17194659">{{cite journal |author=Ngirabacu MC, Ravoet C, Dargent JL, ''et al.'' |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 |year=2006 |month=December |pmid=17194659 |url=http://www.haematologica.org/cgi/pmidlookup?view=long&pmid=17194659}}</ref> |
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==Terminology== |
==Terminology== |
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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>Thiele J, Kvasnicka HM, Schmitt-Graeff A |
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>{{cite journal |author=Thiele J, Kvasnicka HM, Schmitt-Graeff A |title=Acute panmyelosis with myelofibrosis |journal=Leuk. Lymphoma |volume=45 |issue=4 |pages=681–7 |year=2004 |month=April |pmid=15160939 |doi=10.1080/10428190310001625692 |url=http://informahealthcare.com/doi/abs/10.1080/10428190310001625692}}</ref><ref>{{cite journal |author=Thiele J, Kvasnicka HM, Zerhusen G, ''et al.'' |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 |year=2004 |month=August |pmid=15173958 |doi=10.1007/s00277-004-0881-8 }}</ref> |
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==See also== |
==See also== |
Revision as of 02:48, 2 July 2011
Acute panmyelosis with myelofibrosis | |
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Specialty | 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. [1]
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.[2]
Prognosis and treatment
Median survival is about 9 months.[citation needed]
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
References
- ^ Hoffman R, Benz E, Shattil S, Furie B, Cohen H (2004). Hematology: Basic Principles and Practice (4th ed.). Churchill Livingstone.
{{cite book}}
: CS1 maint: multiple names: authors list (link) - ^ Orazi A, O'Malley DP, Jiang J; et al. (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.
{{cite journal}}
: Explicit use of et al. in:|author=
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ignored (help)CS1 maint: multiple names: authors list (link) - ^ Ngirabacu MC, Ravoet C, Dargent JL; et al. (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.
{{cite journal}}
: Explicit use of et al. in:|author=
(help); Unknown parameter|month=
ignored (help)CS1 maint: multiple names: authors list (link) - ^ Thiele J, Kvasnicka HM, Schmitt-Graeff A (2004). "Acute panmyelosis with myelofibrosis". Leuk. Lymphoma. 45 (4): 681–7. doi:10.1080/10428190310001625692. PMID 15160939.
{{cite journal}}
: Unknown parameter|month=
ignored (help)CS1 maint: multiple names: authors list (link) - ^ Thiele J, Kvasnicka HM, Zerhusen G; et al. (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.
{{cite journal}}
: Explicit use of et al. in:|author=
(help); Unknown parameter|month=
ignored (help)CS1 maint: multiple names: authors list (link)