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23 Cards in this Set

  • Front
  • Back
Myeloid disorders are what type of disorders
Neoplastic disorders of stem cells
What are the classifications of Neoplastic Myeloid disoders
1. Chronic Myeloproliferative diorders
2. Myelodisplastic Syndrome
3. Acute Myloblastic Syndrome
Classification of Chronic Myleoproliferative Disorders
1.Polycythemia Vera
2. Chronic Myelogenous leukemia
3. Myeloid Metaplasia with myelofibrosis
4. Essential Thrombocythemia
Polycythemia is defined as
increase in hemoglobin, hematocrit, and RBC
Polycythemia: plasma volume
changes based on the type
RBC Count vs RBC MASS
RBC = # of RBC's/uL
RBC Mass= total # of RBC in Body
RBC count is the ratio of RBC mass to Plasma volume
If you have polycythemia what question should you ask first?
Is it relative or absolute
What is relative polycythemia?
Increased RBC count due to a decrease in PV Plasma Volume
--Ex: Volume depletion from sweating
What is absolute polycythemia?
Increase in bone marrow production of RBC
What would the clinical findings of relative polycythemia look like?
Increase in RBC count, Decrease in PV, Normal O2 sat, and EPO
After you have determined that it is absolute polycythemia, what should ask yourself next?
Is it appropriate or inappropriate
What is appropriate polycythemia?
Appropriate absolute poly= occurs when there is tissue hypoxia
---EX. primary lung disease, cyanotic heart disease, high altitude living,
What does appropriate polycythemia look like? RBC mass, EPO, O2 sat, Plasma volume
Increase RBC mass
Increase EPO
Normal PV
Decrease Sao2
What does inappropriate polycythemia look like? RBC mass, EPO, O2 sat, Plasma volume
Increase RBC mass
Increase EPO
Increase PV
Normal Sa02
What is the definition of inappropriate polycythemia?
No hypoxic stimulus for EPO release
What are the causes of inappropriate polycythemia?
1. Polycythemia Vera
2. Ectopic Secretion of EPO ( Renal cell damage, cyst or cancer)
Pathogenesis of Polycythemia Vera
1. Clonal expansion of multipotent myeloid stem cell
2. Increase in RBC's, granulocytes, mast cells, and platelets
What are the clinical findings of Polycythemia Vera?
1.Splenomegaly
2.Thrombotic Events due to hyperviscosity
3. Signs of histamine release
4. Gout
What are the signs of histamine release in Polycythemia Vera
1. Ruddy Race - Red Face
2. Pruritus when bathing *****
3. PUD- histamine stimulates production of acid release
Why do you have gout in polycythemia vera?
Due to increased breakdown of nucleated cells with release of purines
What is do you give patients on chemotherapy and why?
Allopurinol
B/c of huge cell death from chemo releases purines which are broken down to hypoxathine
What would expect the Lab Findings of Poly to look like?
RBC
WBC
Platelets
EPO
Bone Marrow
1.Increase RBC mass and PV
***Only polycythemia with increase in PV
2. Absolute leukocytosis >12,000
3.Thrombocytosis > 400,000
4. Decreased EPO
5. Normal Sa02
6. Hypercellular bone marrow with fibrosis in later stages
Why is EPO decreased in Polycythemia Vera?
1. Increased O2 content inhibits EPO release
2. Only poly with decreased EPO