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19 Cards in this Set

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What is MDS?
MDS - Myelodysplastic Syndrome

A stem cell problem where RBC, WBC, and or Platelet production is affected. There is bone marrow, but it doesn't make cells.
What's different between MDS and aplastic anemia?
MDS has bone marrow, but it doesn't make cells. In AA, the marrow changes to fat.
What causes the low peripheral cell count in MDS?
Excessive progenitor apoptosis, possibly due to high TNFalpha sensitivity, or T-Cell attack.
What is the typical incidence age for MDS?
60-70 years
What are two common presenting symptoms?
Macrocytic anemia, possibly pancytopenia.
What blood disease might MDS evolve into?
Acute myelogenous leukemia.
What type of disease is MDS?
MDS is a NEOPLASTIC disease, and may be cured by bone marrow transplant.
What are the treatments for MDS?
1) Transplant (risky with age)
2) Transfusion support
3) Growth factors (GMCSF, EPO)
Why might cyclosporin work for MDS?
Some cases of MDS are T-Cell mediated. Cyclosporin shows effects in approx. 60% of cases.
What is "5q Syndrome"?
Deletion of the 5q chromosome is associated with the loss of hematopoetic stem cell factors.
What is "polycythemia"?
A.K.A Erythrocytosis - too many RBCs
What are the causes of polycythemia?
1) Spurious - decreased plasma volume
2) Hypoxia - appropriate rise in EPO
3) High EPO - due to renal carcinoma
4) Polycythemia vera - neoplastic hematopoetic cell disorder
What are some of the causes of tissue hypoxia?
1) Eisenmongers / VSD
2) COPD
3) OSA
4) Chronic CO poisoning
What are the presenting symptoms of PV?
1) Peak incidence over 60
2) Erythromelalgia - episodic severe burning pain in the fingers or toes
3) HA
4) Dizziness
What signs are associated with PV?
1) Facial plethora
2) Hepato and splenomegaly
What lab level is typically LOW in PV?
EPO is typically undetectable in PV.
What are the two main complications with PV?
1) Greatly increased risk of thrombosis
2) Increases risk of bleeding.
(3) Increased risk for AML
(4) Increased risk for bone marrow fibrosis
What are the treatment options for PV?
1) Phlebotomy
2) Hydroxyurea
What are the important elements of a polycythemia H&P?
1) Smoking
2) Lung disease
3) Congenital Heart Disease
4) Family hx of same
5) Size of spleen