# 13 Sickle Cell

![](https://f001.backblazeb2.com/file/wikiFiles/1KwdMym.jpg)

![](https://f001.backblazeb2.com/file/wikiFiles/sTQDaFb.jpg)

![](https://f001.backblazeb2.com/file/wikiFiles/kojSLma.jpg)

* 2 genes, 1 from each parent

![](https://f001.backblazeb2.com/file/wikiFiles/eLaSuYV.jpg)

* A to T leads to aa substition

![](https://f001.backblazeb2.com/file/wikiFiles/qctRror.jpg)

* dehydration/acidosis: acute exacerbation
* polymerize and make RBC form crescents
* decrease ESR instead of up

## Symptoms

![](https://f001.backblazeb2.com/file/wikiFiles/gKNJfd8.jpg)

* hemolytic anemia similar to beta thalassemia major
* vaso occlusion: more dangerous and unique symptoms

### Hemolysis

![](https://f001.backblazeb2.com/file/wikiFiles/TN7yvDn.jpg)

* sometimes simply rupture in vasculature

![](https://f001.backblazeb2.com/file/wikiFiles/IFt8tWc.jpg)

![](https://f001.backblazeb2.com/file/wikiFiles/Hman2gL.jpg)

![](https://f001.backblazeb2.com/file/wikiFiles/zdqCwKP.jpg)

![](https://f001.backblazeb2.com/file/wikiFiles/B4xj9LH.jpg)

### Vasoocclusion

![](https://f001.backblazeb2.com/file/wikiFiles/t7LGDaH.jpg)

* heart, brain

![](https://f001.backblazeb2.com/file/wikiFiles/JSG9GT6.jpg)

* first thing that happen

![](https://f001.backblazeb2.com/file/wikiFiles/sXjJvL7.jpg)

![](https://f001.backblazeb2.com/file/wikiFiles/qvBN8Eb.jpg)

* jagged femoral head

![](https://f001.backblazeb2.com/file/wikiFiles/dXZwUye.jpg)

* dehydration lead to sickling
* can become dependent on narcotics

![](https://f001.backblazeb2.com/file/wikiFiles/X65LJMF.jpg)

* have spleen but not working

![](https://f001.backblazeb2.com/file/wikiFiles/37i4WYj.jpg)

* can die from infection
* salmonella encapsulated, staph not
* The spleen may demonstrate brownish discoloration (hemosiderosis) due to extensive ingestion of sickled RBCs by splenic macrophages (extravascular hemolysis).

![](https://f001.backblazeb2.com/file/wikiFiles/gGF7rOk.jpg)

* fish bone: Osteomyelitis
* sickle: In Sickle Cell disease

![](https://f001.backblazeb2.com/file/wikiFiles/Syw2PQ6.jpg)

* sickle: Increased risk of infection in sickle cell or asplenic patients 

![](https://f001.backblazeb2.com/file/wikiFiles/v38lIy4.jpg)

* pooling of RBC in spleen from obstruction
* children: spleen not yet fibrosis

![](https://f001.backblazeb2.com/file/wikiFiles/9DaQPLl.jpg)

![](https://f001.backblazeb2.com/file/wikiFiles/w2sxEa6.jpg)

* treated with antibiotics because looks just like pneumonia. Don't know if microbe present or not

![](https://f001.backblazeb2.com/file/wikiFiles/2q4efrj.jpg)

* papillary necrosis

![](https://f001.backblazeb2.com/file/wikiFiles/OMnxBKG.jpg)

* high concentration in medulla abolished

![](https://f001.backblazeb2.com/file/wikiFiles/c0ag5fn.jpg)

## Treatment

![](https://f001.backblazeb2.com/file/wikiFiles/ShqNTua.jpg)

## Trait

![](https://f001.backblazeb2.com/file/wikiFiles/LUT3mLB.jpg)

## Diagnosis

![](https://f001.backblazeb2.com/file/wikiFiles/SYKC7XL.jpg)

![](https://f001.backblazeb2.com/file/wikiFiles/QsilJYm.jpg)

## Malaria

![](https://f001.backblazeb2.com/file/wikiFiles/hIGCoo5.jpg)

* most severe form
* just as likely to be infected, less likely to die

## Thalassemia

![](https://f001.backblazeb2.com/file/wikiFiles/pGtBwQd.jpg)

* less beta production depending on how severe infection is

## HbC

![](https://f001.backblazeb2.com/file/wikiFiles/8B16mXR.jpg)

* lysine more polar than valine: not as severe of a problem
* lysing more positive than glutamate, less travel than HbS and Hb normal on electrophoresis
* very rare to be homozygous

![](https://f001.backblazeb2.com/file/wikiFiles/TRBsxXr.jpg)

## Hb SC

![](https://f001.backblazeb2.com/file/wikiFiles/ThQPqm3.jpg)

* heterozygous: usually mild anemia without sickling
* except HbS plus HbC