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m (MPN vs MDS) |
m |
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==Clinic== | ==Clinic== | ||
* | * 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 | ||
* 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: | **Polycythemic phase: Increased RBC count. | ||
**Post-polycythemic ("spent") phase: Ineffective hematopoiesis, bone marrow fibrosis. | **Post-polycythemic ("spent") phase: Ineffective hematopoiesis, bone marrow fibrosis. | ||
*Transformation | *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 ( | *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 | *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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