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14 Cards in this Set

  • Front
  • Back
4 types of CMPDs.
CML - Chronic Myeloblastic Leukemia
PV - Polycythemia Vera
ET - Essential Thrombocythemia
IMF - Idiopathic Myelofibrosis
General characteristics of CMPDs
Hypercellular b.m.
Increased quantities of some or more cell lines
60-90% pts have splenomegaly
Clonal
Clinical and hematological features of CMPDs
middle age and older groups
extra-meduallary hematopoiesis -> spleen and liver
may terminate in Acute Leukemia
Frequent transition between CMPDs
Cytogenic abN
Molecular abN
IMF characteristics
fibrosis of the marrow
extra-meduallary hematopoiesis or myeloid metaplasia
leukoerythroblastosis
dacryocytes present
No cure
IMF pathogenesis & current theory
increased acculmulation of marrow collagen
plts are implicated
calcitrol
calcitrol
inhibits collagen synthesis by suppressing megakaryocyte proliferation
PV laboratory findings
^^ rbcs in pb and bm + excessive proliferation of myeloid & plt elements
PV pathogenesis
- origin of PV is an abN pluripotent stem cell
PV lab features
1. ^rbc/^hct/^hb
2. EPO N or decrease
3. rbcs are N/N
4. ^plts (50%)/^granulocytes(68%)
5. falsely elevated PT and APTT tests
6. ^LAP (70%)
7. splenomegaly is present
PV risks
1. in vivo clotting
2. transformation into IMF or leukemia
PV treatment
to reduce rbc mass
- phlebotomy
- myelosuppressive drugs
ET characteristics
1. marked thrombocytosis
2. plts have abN plt function
3. quite rare
4. 2/3 pt no sym, 1/3 bleeding sym
ET lab features
1. plts >600 x 10^9/L
2. PDW ^ (usually <20)
3. abN plt aggregation pattern
4. prolonged bleeding times
5. megakaryocytic hyperplasia in bm
6. ph chromosome is absent
ET treatment
1. plateletpheresis
2. chemo