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17 Cards in this Set

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How do hematopoietic stem cells divide?
1 daughter returns to SC pool

1 daughter begins to differentiate
What are important factors in Non-hematopoietic regulation of hematopoiesis?
1. Environment such as fibroblasts, endothelial cells,etc

2. Activated T cells, monocytes amplify basal pathways during infection

3. Hematopoietic Growth Factors - CSF's, IL's
Where is Erythropoietin (EPO) made?
Kidney
What is EPO's function?
Stimulates erythropoiesis
What are the functions of G-CSF and GM-CSF
Colony Stimulating Factors that stimulate proliferation of myeloid progenitor cells

- Speed differentiation to neutrophils

- helps with recovery after bone marrow transplantation
What are the function of TPO (thrombopoietin) mimetic (Romiplostim & Eltrombopag)?
- Stimulate megakaryocyte proliferation and differentiation

- Give rise in platelet counts
What is aplastic anemia?
- Condition where bone marrow does not produce enough new cells to replenish blood cells

- Bone Marrow become Hypocellular
What are 4 important Pediatric or Inherited causes of bone marrow failure?
1. Dyskeratosis Congenita

2. Schwachman Diamond Syndrome

3. Diamond Blackfan Anemia (DBA)

4. Familial Aplastic Anemia
What is the most important Acquired or adult cause of bone marrow failure?
Paroxysmal Nocturnal Hemoglobinuria (PNH)

Recall: Important cause of Hemolytic Anemia
What are the 3 categories for clinical classification of Aplastic Anemia?
1. Onset: Acute, Chronic, Congenital

2. Blood cells affected
- Pancytopenia
- Single Lineage
- Bi-lineage

3. Site of BMF
- Central
- Peripheral (marrow normal but can't exit)
- Maturational arrest
What is seen in Diamond Blackfan Anemia?
Lack of RBC differentiation
Dyskeratosis Congenita (DC) causes what?
Pancytopenia
What is the genetic cause of Dyskeratosis Congenita (DC)?
Defect in telomere maintenance
Fanconi Anemia (FA) causes what?
Pancytopenia
Schwachman Diamond Syndrome (SDS) causes what?
Myeloid Cell cytopenia
What does Severe Congenital Neutropenia cause?
- Neutropenia

- Promyelocytic maturation arrest
What do all Inherited Bone Marrow Failure Syndromes' Pathways have in common?
All pathways share activation of p53

- causes cell cycle arrest and/or senescence