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Hemoglobinopathies II
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Questions in this quiz (12)
1With respect to sickle cell disease (SCD) genotypes, the HbSS/HbSB0 types are more severe than the HbSC/HbSB+ types.
- ATrue
- BFalse
2Globally, about how many people are born each year with some form of sickle cell?
- A100k
- B200k
- C400k
- D500k
3A newborn screening of hemoglobin phenotype reports findings in order of relative abundance. The newborn's top reported Hb is "FSA". Which of the following conditions matches this Hb?
- ASickle cell trait
- BSickle cell anemia (SS or SB0)
- CSickle B+ thalassemia
- DSickle cell disease (HbSC)
4One of very few outward manifestations (and the most common initial presentation) of SCD is
- AFunctional asplenia
- BAcute chest syndrome
- CDactylitis
- DStroke
5The mainstay of treatment in SCD is ___. Given the pharmacokinetic/dynamic properties of this drug, it is not indicated for ____ episodes.
- Ahydroxyurea; chronic
- Bhydroxyurea; acute
- Chydroxycholorquine; chronic
- Dhydroxychloroquine; acute
6Hyrdoxyurea, as a treatment for SCD, results in
- Aincreased HbF production
- Bdecreased HbF production
- Creduced RBC sickling
- Dlowered WBCs
711% of those with SCD have a stroke by the age of 45.
- ATrue
- BFalse
8A patient has been treated with hydroxyurea for SCD. Which of the following changes in lab parameters would you expect (compared to baseline)?
- AIncreased Hgb
- BIncreased MCV
- CDecreased ANC
- DIncreased LDH, Bili
- EDecreased WBC
- FDecreased HbF
9The primary diagnostic modality for sickle cell trait is
- APBS
- BHgb electrophoresis
- CPlasmapheresis
- DOsmotic fragility test
10Which of the following would you (usually) expect to note on PBS from a patient with sickle cell trait?
- ADecreased Hgb
- BIncreased hematocrit
- CReticulocytopenia
- DNone of the above
11To prevent sepsis in children w/ sickle cell, prophylactic PCN should be started at 3 months and can be discontinued around age 5 once pneumococcal/meningococcal vaccines have been completed.
- ATrue
- BFalse
12A patient has a hemoglobin phenotype of: HbA 60%, HbS 40%, and normal HbA2, HbF. This is consistent with which condition/gene mutation?
- ANormal (AA); B with B
- BSickle cell trait (AS); B with Bs
- CSickle cell anemia (SS); Bs with Bs
- DSickle cell disease (SC); Bs with Bc
Information
- Description
- Quiz on Hemoglobinopathies II, created by Trey W on 19/09/2020.
- Author
- Trey W
- Language
- English
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- 49
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