Difference between revisions of "Myeloproliferative neoplasms"

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m (MPN vs MDS)
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==Clinic==
==Clinic==
* moderate to severe anemia (in cases where the erythrocytosis is not a feature)
* increase in blood count(s), dependent on type of MPN
* anemia may occur in late stage disease or due leukemic transformation
* pale skin
* pale skin
* fatigue
* fatigue
* prolonged bleeding
* enlarged spleen  
* enlarged spleen  
* increased clotting
* increased clotting (i.e. thrombosis)


==Polycythemia vera==
==Polycythemia vera==
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*Typical course:
*Typical course:
**Prodromal stadium: Mild erythrocytosis.
**Prodromal stadium: Mild erythrocytosis.
**Polycythemic phase: Incereased RBC count.
**Polycythemic phase: Increased RBC count.
**Post-polycythemic ("spent") phase: Ineffective hematopoiesis, bone marrow fibrosis.
**Post-polycythemic ("spent") phase: Ineffective hematopoiesis, bone marrow fibrosis.
*Transformation in [[AML]] possible (2-3%).
*Transformation to [[AML]] possible (2-3%).


===Morphology===
===Morphology===
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*Many megakaryocytes in bone marrow section.
*Many megakaryocytes in bone marrow section.
*Increased cellularity of bone marrow (35-100%).
*Increased cellularity of bone marrow (35-100%).
*Teardrop-RBC cells (dacryocytes) in late stage.
*Teardrop-RBC cells (dacrocytes) in late stage.
*Fibrosis (reticulin stain).
*Fibrosis (reticulin stain).


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*Median age: 55 years.
*Median age: 55 years.
*Mostly asymptomatic.
*Mostly asymptomatic.
*Indolent course, long-term survival. Life-threatening thromboembolitic epsisodes.
*Indolent course, long-term survival. Life-threatening thromboembolic epsisodes.


===Morphology===
===Morphology===
*Thrombocytosis.
*Thrombocytosis.
*Bone marrow normo- to slightly hypercellular.
*Bone marrow normo- to slightly hypercellular.
*Large megakaryocytes in bone-marrow.
*Large hypersegmented megakaryocytes in bone-marrow.
*Usually no BM fibrosis.
*Usually no BM fibrosis.


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