22 Myeloproliferative
![](https://photos.thisispiggy.com/file/wikiFiles/Fi9uIFY.jpg)
- proliferation of cells of myeloid lineage
![](https://photos.thisispiggy.com/file/wikiFiles/89T2WmL.jpg)
- myelofibrosis: overproduction of bone marrow fibrous tissues, low peripheral cell counts
Mutations
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![](https://photos.thisispiggy.com/file/wikiFiles/CIFP5kJ.jpg)
Polycythemia Vera
Pathogenesis
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- increased resistance to flow, stasis, thrombosis
![](https://photos.thisispiggy.com/file/wikiFiles/GKO9SrP.jpg)
- histamine and PGE release after shower
- Budd Chiari: blood clot in hepatic vein
![](https://photos.thisispiggy.com/file/wikiFiles/U8MFBla.jpg)
- excessive proliferation: bone marrow burned out
- progress to myeloid leukemia
Diagnosis
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![](https://photos.thisispiggy.com/file/wikiFiles/tH829b3.jpg)
- diagnosis must exclude other causes
![](https://photos.thisispiggy.com/file/wikiFiles/rzo8bb6.jpg)
- hydroxyurea: converts RNA nucleotide to DNA nucleotide
Essential Thrombocytosis
![](https://photos.thisispiggy.com/file/wikiFiles/DaMBZgj.jpg)
![](https://photos.thisispiggy.com/file/wikiFiles/9MpxHdt.jpg)
- must exclude polycythemia and CML
- iron deficiency anemia: protective mechanism against blood loss
![](https://photos.thisispiggy.com/file/wikiFiles/nxctIQM.jpg)
- increased platelets but not functioning normally
- overactivity of platelets
![](https://photos.thisispiggy.com/file/wikiFiles/oUzzZsC.jpg)
Myelofibrosis
![](https://photos.thisispiggy.com/file/wikiFiles/iP45XYF.jpg)
- primary: fibrous tissues overtaking bone marrow
- secondary: spent phase in polycythemia
Primary
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- collagen overtakes bone marrow, pancytopenia
- cytokines from macrophages
![](https://photos.thisispiggy.com/file/wikiFiles/Id5IxwO.jpg)
- bone marrow fails, other organs take over jobs
![](https://photos.thisispiggy.com/file/wikiFiles/G2uY0Bv.jpg)
- increased metabolism from extramedullary hematopoiesis and severe anemia
![](https://photos.thisispiggy.com/file/wikiFiles/XauijLB.jpg)
- immature precursor cells pushed out of bone marrow
![](https://photos.thisispiggy.com/file/wikiFiles/ciTlVpr.jpg)
![](https://photos.thisispiggy.com/file/wikiFiles/rPF2EKw.jpg)
Langerhan Histiocytosis
![](https://photos.thisispiggy.com/file/wikiFiles/Bj9KArF.jpg)
- langerhan: type of histiocyte
![](https://photos.thisispiggy.com/file/wikiFiles/YqJB87s.jpg)
- malignant cells precursor myeloid cells, not really langerhan, just look like it
![](https://photos.thisispiggy.com/file/wikiFiles/BjS8gts.jpg)
![](https://photos.thisispiggy.com/file/wikiFiles/U9vAE2R.jpg)
![](https://photos.thisispiggy.com/file/wikiFiles/N0L7q0f.jpg)
- most severe: letterer-siwe
- least severe: eosinophilic granuloma, presenting with bone fracture and langerhan cells/eosinophils
![](https://photos.thisispiggy.com/file/wikiFiles/s5KlnYc.jpg)
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