SICKLE CELL ANEMIA

Question 1

sickle cell anemia or HBS results from this mutation? on this gene?

Answer 1

point mutation

beta chain gene

Question 2

HbS polymerizes under what conditions?

Answer 2

ischemic conditions

Question 3

crystals of HbS cause RBC to assume what shape?

Answer 3

sickle shape

Question 4

sickling of RBCs is influenced by concentration of _____?

Answer 4

HbS

Question 5

this increases sickling?

Answer 5

hypoxia

dehydration

Question 6

what are the two clinical presentations?

Answer 6

sickle cell trait

sickle cell disease

Question 7

name this presentation?

vaso occlusion may occur during periods of severe hypoxia/stress

Answer 7

sickle cell trait

Question 8

name this presentation?

chronic hemolytic anemia

chronic ischemic disease and episodic vaso occlusive crises

manifests after 6 months

Answer 8

sickle cell disease

Question 9

hyper plastic bone marrow

chronic ischemia

vasoocclusive crisis

these are clinical manifestations of?

Answer 9

sickle cell disease

Question 10

what are the therapies?

Answer 10

rehydration

exchange transfusion to lower HbS burden

chronic RBC transfusion for anemia

hydroxyyrea

Question 11

benefits of longterm treatment with hydroxyurea?

Answer 11

increase circulating HbF

decrease rate of sickling