Glossary of Hematology - Myeloproliferative Diseases
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- What is the pathophysiological cause of myeloproliferative diseases?
- Clonal proliferation of a myeloid stem cell --> excessive production of mature, differentiated myeloid cell lines
- Name 4 subtypes of myeloproliferative diseases
- Polycythemia vera
Chronic Myeloid Leukemia
- Epidemiology of Polycythemia Vera (age, gender, incidence)
- 50-60 yr, male, rare
- Si/Sx of PV
- HA, vertigo, diplopia, retinal hemorrhages
strokes, angina, claudication
**pruritus after showering**
- Tx and Px of PV
- Tx: phlebotomy
Px: good (**5% progress to leukemia, 20% to myelofibrosis**)
- What is DDx for elevated hematocrit? (2)
- primary polycythemia
- What is the pathophysiology of Essential Thrombocythemia (secondary thrombocytosis)?
How do you diagnose ET?
- Clonal proliferation of megakaryocytes --> elevated platelet counts
**Diagnosis of exclusion!**
- Name 3 general causes of ET?
- Iron deficiency
Inflammatory Dz (infection, IBD, etc)
- Si/Sx of ET
- plt > 5 x 10^5 cells/ul, **ecchymoses/bleeding but not typically thrombosis**
- Tx and Px of ET
- Only treat if symptomatic
- apheresis (plt exchange)
- hydroxyurea & INF-alpha
- Anagrelide is first line (unknown mechanism which decreases plt synthesis from megas)
- What is the population of idiopathic myelofibrosis?
- >50 yo
- Pathophysiology of idiopathic myelofibrosis
- Clonal proliferation of unknown cell type leading to fibrosis of bone marros --> extramedullary hematopoiesis
- Si/Sx of idiopathic myelofibrosis
What do you see on blood smear?
- Si/Sx: massive hepatomegaly and splenomegaly, gout
Blood: teardrop cells, nucleated rbc, immature wbc, anemia with high wbc and plt
- Tx and Px of idiopathic myelofibrosis
- Tx: symptomatic (splenectomy, ABx, allopurinol)
Px: very poor (5yr until total marrow failure)
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