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'''Myeloproliferative neoplasms''', also '''myeloproliferative disorders''', are a group of indolent hematologic neoplasms characterized by clonal expansion of pluripotent hematopoietic progenitor. | '''Myeloproliferative neoplasms''', also '''myeloproliferative disorders''', are a group of indolent hematologic neoplasms characterized by clonal expansion of pluripotent hematopoietic progenitor. | ||
These should '''not''' be confused with ''[[myelodysplastic syndromes]]'' (MDS). | These should '''not''' be confused with ''[[myelodysplastic syndromes]]'' (MDS), the main difference is that MPNs produce functional haematological cells detectable on a complete blood count, whereas in MDS, hematopoisis is ineffective and is characterised by cytopenias. | ||
==Grouping== | ==Grouping== | ||
They include the following:<ref name=pmid15781101>{{cite journal |author=Baxter EJ, Scott LM, Campbell PJ, ''et al.'' |title=Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders |journal=Lancet |volume=365 |issue=9464 |pages=1054–61 |year=2005 |pmid=15781101 |doi=10.1016/S0140-6736(05)71142-9 |url=}}</ref> | They include the following:<ref name=pmid15781101>{{cite journal |author=Baxter EJ, Scott LM, Campbell PJ, ''et al.'' |title=Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders |journal=Lancet |volume=365 |issue=9464 |pages=1054–61 |year=2005 |pmid=15781101 |doi=10.1016/S0140-6736(05)71142-9 |url=}}</ref> | ||
*Polycythemia vera | *[[Chronic myeloid leukemia]] | ||
*Essential thrombocythemia. | *[[Polycythemia vera]] | ||
*Idiopathic myelofibrosis. | *[[Essential thrombocythemia]]. | ||
*[[Idiopathic myelofibrosis]]. | |||
==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== | |||
*ICD-O: 9950/3 | |||
*Median age: 60 years. | |||
*Median survival: >10years. | |||
*Typical course: | |||
**Prodromal stadium: Mild erythrocytosis. | |||
**Polycythemic phase: Increased RBC count. | |||
**Post-polycythemic ("spent") phase: Ineffective hematopoiesis, bone marrow fibrosis. | |||
*Transformation to [[AML]] possible (2-3%). | |||
===Morphology=== | |||
*Panmyelosis. | |||
*Many megakaryocytes in bone marrow section. | |||
*Increased cellularity of bone marrow (35-100%). | |||
*Teardrop-RBC cells (dacrocytes) in late stage. | |||
*Fibrosis (reticulin stain). | |||
==Essential thrombocythaemia== | |||
*ICD-O: 9962/3 | |||
*Median age: 55 years. | |||
*Mostly asymptomatic. | |||
*Indolent course, long-term survival. Life-threatening thromboembolic epsisodes. | |||
===Morphology=== | |||
*Thrombocytosis. | |||
*Bone marrow normo- to slightly hypercellular. | |||
*Large hypersegmented megakaryocytes in bone-marrow. | |||
*Usually no BM fibrosis. | |||
==Images== | |||
<gallery> | |||
File:Thrombocytosis.jpg | Thrombocythemia. Peripheral blood (WC/Erhabor Osario) | |||
File:Polycythemia_vera,_blood_smear.jpg | Polycythemia vera. Peripheral blood (WC/Orlandi Mourao) | |||
File:Teardrop Cells smear 2009-09-22.JPG | Teardrop RBC cells in polycythemia vera. Peripheral blood (WC/AFIP) | |||
File:Essential Thrombocythemia, Peripheral Blood (10189570483).jpg | Essential thrombocythemia. Peripheral blood (WC/Ed Uthman) | |||
File:Essential thrombocythemia (2).jpg | Essential thrombocythemia. Bone marrow (WC/KGH) | |||
</gallery> | |||
==Molecular== | ==Molecular== | ||