22 Myeloproliferative
- proliferation of cells of myeloid lineage
- myelofibrosis: overproduction of bone marrow fibrous tissues, low peripheral cell counts
Mutations
Polycythemia Vera
Pathogenesis
- increased resistance to flow, stasis, thrombosis
- histamine and PGE release after shower
- Budd Chiari: blood clot in hepatic vein
- excessive proliferation: bone marrow burned out
- progress to myeloid leukemia
Diagnosis
- diagnosis must exclude other causes
- hydroxyurea: converts RNA nucleotide to DNA nucleotide
Essential Thrombocytosis
- must exclude polycythemia and CML
- iron deficiency anemia: protective mechanism against blood loss
- increased platelets but not functioning normally
- overactivity of platelets
Myelofibrosis
- primary: fibrous tissues overtaking bone marrow
- secondary: spent phase in polycythemia
Primary
- collagen overtakes bone marrow, pancytopenia
- cytokines from macrophages
- bone marrow fails, other organs take over jobs
- increased metabolism from extramedullary hematopoiesis and severe anemia
- immature precursor cells pushed out of bone marrow
Langerhan Histiocytosis
- langerhan: type of histiocyte
- malignant cells precursor myeloid cells, not really langerhan, just look like it
- most severe: letterer-siwe
- least severe: eosinophilic granuloma, presenting with bone fracture and langerhan cells/eosinophils
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