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Flashcards in Hematology Deck (111)
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1

What triggers RBCs to sickle in affected individuals?

Low O2 states

2

Why are sickle cells bad - how do they cause harm?

Vasoocclusion of organs/tissues

3

How does acute chest syndrome present?

Pulmonary infiltrates on CXRFever, hypoxia, acidosisNo infection

4

What is the typical electrophoresis in sickle cell disease and trait?

SS: Mostly Hgb S, some A2 and FTrait: Mostly Hgb A noted, with some S, A2, and F

5

Who should be offered sickle cell carrier screening?

Those of African and mediterranean ancestry

6

How should you screen for sickle cell in those with African or other heritage?

African-American: CBC + electrophoresisOther: CBC with diff, and electrophoresis only if microcytic (<80)

7

What are the risks associated with sickle cell disease in pregnancy?

PTL, PPROM, infection post-partum, IUGR, IUFD

8

How much folic acid should patients with sickle cell take daily?

4mg/day

9

How do you counsel patients with sickle cell on crisis avoidance?

Avoid triggers (cold, physical exertion, dehydration, and stress)

10

Can hydroxyurea be used in pregnancy to avoid a sickle crisis?

No - hydroxyurea is teratogenic

11

How do we treat sickle crises?

O2, opiodsEvaluation/treatment of cause (infection, dehydration)Management of compliations (acute chest)

12

When exchange transfusions are utilized in pregnancy for sickle cell, what is the typical indication?

Reduce frequency of pain crises

13

With an exchange transfusion, what is the treatment goal for % HgbS?

Hgb S <40%

14

How should we monitor fetuses in pregnancies complicated by SS?

Serial growth ultrasound q 4-6 weeksAntepartum testing beginning at 32 weeks

15

What electrophoresis pattern is consistent with beta-thalassemia?

Increased Hgb A2 > 3.5%, increased Hgb F, no Hgb S

16

What causes B-thal?

Bad beta-chain -> loss of type A Hgb (alpha/beta)

17

What are the two general types of gene mutations associated with B-thal?

B (0) - absent beta chain productionB (+) - decreased amount of beta chain production

18

What are the two grades of B-thal severity?

B-thal MINOR: mild anemiaB-thal MAJOR: severe anemia, complications

19

What complications are associated with B-thal major over a lifetime (outside of pregnancy)

Extramedullary hematopoeisisDelayed sexual development (due to hemosiderin deposition in hypothalamus)Poor growthDeath by age 10 if blood transfusions are not initiated

20

What patients with B-thal major are candidates for pregnancy?

Normal heart functionNo end organ damage from chronic transfusion (or h/o transfusion, coupled with deferoxamine for chelation)

21

Which ethnicities are most commonly associated with B-thal mutations?

Mediterranean, Asian, middle eastern, Hispanic, W.Indian

22

What is the outcome of B-thal combined with HgbS?

Severe anemia due to absent functional beta-chain production -> no Hgb A production

23

While pregnancies with B-thal minor are usually favorable, what outcomes should be monitored for?

IUGR, oligohydramnios

24

Instead of iron, what supplement should be given to women with B-thal?

Folic acid 1mg/day

25

If you diagnose a patient with B-thal, what supplement should be initiated, and who else should be tested?

Initiated folic acid 1mg/dayTest father with CBC -> electrophoresis if microcyticGenetic counseling if dad also a carrier

26

When should you refer a patient for alpha-thal testing?

Microcytic anemiaNormal electrophoresis and iron testingSE Asian, African, W Indian, and mediterranean

27

What causese alpha-thalassemia?

Defunct alpha-chain production -> decreased Hgb A (alpha/beta), A2 (alpha/delta), and F (alpha/gamma)

28

Alpha thall involves mutations in how many alpha genes? What are the possible mutation combinations called?

TRANS: a_/a_CIS: __/aa

29

What ethnicities are most likely to be affected by sickle cell?

SE Asian, African, W. Indian, Mediterranean

30

Why are E.Asian individuals with a-thall more likely to have a child with Hgb Barts than African individuals?

CIS mutation (__/aa) seen more commonly in SE Asia -> more likely to have fetus with (__/__)