Term
| What 4 factors enhance polymerization in patients with sickle cell? |
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Definition
1. dehydration
2. high concentration of deoxy-HbS
3.acidosis
4.increase in 2,3-BPG |
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Term
| What 2 factors inhibit polymerization of HbS? |
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Definition
| the presence of HbA and/or HbF |
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Term
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Definition
| A single amino acid replacement in the beta chain (substituion of normal glutamic acid with valine). |
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Term
| What are the three major clinical manifestations of sickle cell? |
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Definition
1. Anemia
2. Crisis
3. Complication |
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Term
| Vaso-occlusive crisis cause? |
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Definition
| Caused by sickle-shaped red blood cells that block capillaries and restrict blood flow to an organ resulting in ischemia. |
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Term
| Vaso-occlusive crisis prevention? |
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Definition
| regular blood transfusion |
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Term
| Splenic sequestration crisis |
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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. |
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Term
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Definition
| Acute exacerbation of anemia, transient arrest of erythropoiesis, and reticulocytopenia (decrease in immature red blood cells) caused by parvovirus B19 infection. |
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Term
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Definition
| Severe acute anemia caused by rapid destruction of red blood cells. Usually accompanies Vaso-occlusion. |
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Term
| first symptom of sickle cell |
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Definition
| dactylitis and fever (6 months to 2 years old) |
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Term
| Examples of compound heterozygous states of sickle cell |
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Definition
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Term
Expected test results for HbSC
Blood Smear____
HPLC_____
Sickling Solubility Test______ |
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Definition
Expected test results for HbSC
Blood Smear - Many "Target" Cells
HPLC - equal amounts of HbS and HbC
Sickling Solubility Test - positive
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Term
Expected test results for HbSS
HPLC_____
Sickling Solubility Test______
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Definition
Expected test results for HbSS
HPLC - Majority HbS, no HbA present, and increased HbF
Sickling Solubility Test - positive
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Term
Expected test results for HbS/β+
Blood Smear____
HPLC_____
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Definition
Expected test results for HbS/β+
Blood Smear -sickled and "target" cells
HPLC - Marjority HbS, 5-30% HbA, Increase HbF and HbA2
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Term
Expected test results for HbS/β0
Blood Smear____
HPLC_____
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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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