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HUCM Sickle cell anemia
Key points from the loonnnggg lecture
15
Medical
Graduate
08/06/2011

Additional Medical Flashcards

 


 

Cards

Term
What 4 factors enhance polymerization in patients with sickle cell?
Definition
1. dehydration
2. high concentration of deoxy-HbS
3.acidosis
4.increase in 2,3-BPG
Term
What 2 factors inhibit polymerization of HbS?
Definition
the presence of HbA and/or HbF
Term
what is an HbS mutation?
Definition
A single amino acid replacement in the beta chain (substituion of normal glutamic acid with valine).
Term
What are the three major clinical manifestations of sickle cell?
Definition
1. Anemia
2. Crisis
3. Complication
Term
Vaso-occlusive crisis cause?
Definition
Caused by sickle-shaped red blood cells that block capillaries and restrict blood flow to an organ resulting in ischemia.
Term
Vaso-occlusive crisis prevention?
Definition
regular blood transfusion
Term
Splenic sequestration crisis
Definition
Sickling within the spleen causing painful enlargements of the spleen, hypovolemia, and worsening of anemia. The abdomen becomes bloated and very hard. Splenic sequestration crisis is considered an emergency. The patient may die within 1 to 2 hours due to circulatory failure.
Term
Aplastic crisis
Definition
Acute exacerbation of anemia, transient arrest of erythropoiesis, and reticulocytopenia (decrease in immature red blood cells) caused by parvovirus B19 infection.
Term
Hemolytic crisis
Definition
Severe acute anemia caused by rapid destruction of red blood cells. Usually accompanies Vaso-occlusion.
Term
first symptom of sickle cell
Definition
dactylitis and fever (6 months to 2 years old)
Term
Examples of compound heterozygous states of sickle cell
Definition
HbSC HbS/β+ HbS/β0
Term

Expected test results for HbSC

 

Blood Smear____

HPLC_____

Sickling Solubility Test______

Definition

Expected test results for HbSC

 

Blood Smear - Many "Target" Cells

HPLC - equal amounts of HbS and HbC 

Sickling Solubility Test - positive

Term

Expected test results for HbSS

 

HPLC_____

Sickling Solubility Test______

Definition

Expected test results for HbSS

 

HPLC - Majority HbS, no HbA present, and increased HbF 

Sickling Solubility Test - positive

Term

Expected test results for HbS/β+

 

Blood Smear____

HPLC_____

 

Definition

 

Expected test results for HbS/β+

 

Blood Smear -sickled and "target" cells

HPLC - Marjority HbS, 5-30% HbA, Increase HbF and HbA2

 

 

Term

 

Expected test results for HbS/β0

 

Blood Smear____

HPLC_____

 

 

Definition

 

Expected test results for HbS/β0

 

Blood Smear -"target" and sickled cells

HPLC - NO HbA, Majority HbS, increased HbF and HbA2

 

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