2 - Stem Cell Hematopoiesis and Bone Marrow Failure Flashcards

1
Q

sites of early hematopoiesis

A
yolk sac blood islands (16 d)
aortogonadomesonephros (20-30 d)
liver (6 w)
bone marrow (5 mo - postnatal)
thymus (T cell development)
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2
Q

lymphoid derived cells

A

T, B, NK, dendritic

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3
Q

myeloid derived cells

A

RBCs, megakaryocytes/platelets, monocytes/macrophages, granulocytes

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4
Q

3 important characteristics of hematopoietic stem cells

A

multipotent
self renewing
quiescent (only divide when needed)

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5
Q

what class of signaling molecules are responsible for directing stem cell trafficking?

A

cytokines

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6
Q

severe aplastic anemia (SAA) definition

A

bone marrow hypocellularity (<20k)

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7
Q

5 categories of causes for pancytopenia

A
HSC injury
clonal hematopoietic cell mutation
myelophthisis (BM infiltration)
defective maturation
enhanced peripheral destruction
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8
Q

causes of bone marrow failure

A

congenital (Fanconi anemia, dyskeratosis congenita, Shwachman Diamond syndrome, Diamond-Blackfan anemia, congenital amegakaryocytic thromboctyopenia)
acquired (toxic, drugs, viral infxn)
idiopathic (thought to be immune mediated)

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9
Q

Fanconi anemia

A

AR inheritance, variable phenotype
DNA repair disorder

cafe au lait spots, microophthalmia, abnormal thumbs, predisposed to AML/MDS and other cancers

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10
Q

Diamond Blackfan anemia

A

congenital red cell aplasia
present in first year of life - macrocytic, hypoproductive anemia +/- congenital abnormalities

bone marrow has few erythroid precursors, otherwise normal. Also increased eosinophils

most respond to steroids

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11
Q

Acquired pure red cell aplasia

A

rare, can occur w/ thymoma, lymphoma, autoimmune disorders, or after treatment w/ recombinant EPO (formation of Abs against EPO), or after parvo B19 infxn esp in pts w/ chronic hemolytic anemia

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12
Q

tx of idiopathic SAA

A

supportive care - transfusion, judicious use of abx and granulocyte colony stimulating factor (GCSF)

definitive therapy -HSCT or immunosuppressive therapy

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