10 Intrinsic Hemolysis
Cause
![](https://photos.thisispiggy.com/file/wikiFiles/OCzULN1.jpg)
- normally complement does not lyse RBC
![](https://photos.thisispiggy.com/file/wikiFiles/T1iUIla.jpg)
- acquired: genetic mutation some point in life
- GPI: anchors that attach DAF/CD59 to membrane
- stem cell: RBC, platelets, WBC also involved
Pathogenesis, Symptoms
![](https://photos.thisispiggy.com/file/wikiFiles/TXT68QS.jpg)
- Free plasma Hgb thrombogenic, thrombosis
- platelets lysed: spill granules
- Priapism: sustained prolonged erection
![](https://photos.thisispiggy.com/file/wikiFiles/SrPQcij.jpg)
- paroxysmal: sudden
- episodes at night when urine changes color
![](https://photos.thisispiggy.com/file/wikiFiles/0Ry8E4L.jpg)
Diagnosis, treatment
![](https://photos.thisispiggy.com/file/wikiFiles/wKk9urA.jpg)
- antibodies not part of problem
![](https://photos.thisispiggy.com/file/wikiFiles/IBXf9MV.jpg)
Pyruvate Kinase Deficiency
![](https://photos.thisispiggy.com/file/wikiFiles/fFLUg14.jpg)
- when levels low, RBC most affected (require pyruvate kinase and glycolysis for anerobic)
- no ATP, membrane fails
- when problem with membrane, eaten in spleen
![](https://photos.thisispiggy.com/file/wikiFiles/8uxEaQA.jpg)
- disease severity based on how low enzyme activity is
G6PD Deficiency
![](https://photos.thisispiggy.com/file/wikiFiles/W4PJidk.jpg)
![](https://photos.thisispiggy.com/file/wikiFiles/r5HCUIJ.jpg)
![](https://photos.thisispiggy.com/file/wikiFiles/KmgRdpY.jpg)
- antimalarials can trigger hemolysis
![](https://photos.thisispiggy.com/file/wikiFiles/z71q28L.jpg)
![](https://photos.thisispiggy.com/file/wikiFiles/ecqBRrb.jpg)
- Can't make ATP, RBC become rigid, cannot maintain membrane
- most extravascular
- some become so damaged/rigid: intravascular
![](https://photos.thisispiggy.com/file/wikiFiles/RxJfuEz.jpg)
![](https://photos.thisispiggy.com/file/wikiFiles/5XGsQLW.jpg)
- Bite and seeds: bite cells and Heinz bodies seen in RBCs
- Broken G6PD-free fruit: hemolytic anemia in G6PD deficiency
![](https://photos.thisispiggy.com/file/wikiFiles/HkOUx2l.jpg)
- Bite and seeds: bite cells and Heinz bodies seen in RBCs
- Broken G6PD-free fruit: dapsone can cause hemolytic anemia in G6PD deficiency
![](https://photos.thisispiggy.com/file/wikiFiles/jEybmas.jpg)
- "Color queen" - chloroquine, beads join together as polymer, blocking plasmodium heme polymerase. High resistance
- "Primal queen" - primaquine, can cause anemia in G6PD deficiency
![](https://photos.thisispiggy.com/file/wikiFiles/bdtDD5G.jpg)
- fluorescent spot: screening test
- pts unable to generate NADPH
- normal: generate NADPH, fluoresce
- must test when not having acute attack
![](https://photos.thisispiggy.com/file/wikiFiles/c67Wx0T.jpg)
Hereditary Spherocytosis
![](https://photos.thisispiggy.com/file/wikiFiles/Vi1xZeA.jpg)
- proteins give RBC biconcave shape and flexibility
- abnormal: rigid membrane
- spectrin for spherocytosis
![](https://photos.thisispiggy.com/file/wikiFiles/pcXcGvt.jpg)
- problem lies in chronic extravascular hemolysis, not O2 carrying capacity
Diagnosis
![](https://photos.thisispiggy.com/file/wikiFiles/HoL4UuB.jpg)
![](https://photos.thisispiggy.com/file/wikiFiles/6QvNBLo.jpg)
- progressive loss of cell membrane as RBC circulate in vasculature
- RDW: red cell distribution width
- normally: narrow, some small/large cells. Most are in middle
- Spherocytosis: large spectrum of RBC sizes
![](https://photos.thisispiggy.com/file/wikiFiles/nNxQgPS.jpg)
- material density goes up
- MCHC: mean corpuscular hemoglobin concentration
- MCV: mean corpuscular volume: average size of RBCs
- chronic hemolysis: more reticulocytes, high MCV
![](https://photos.thisispiggy.com/file/wikiFiles/B6ffANM.jpg)
![](https://photos.thisispiggy.com/file/wikiFiles/iNN2pHD.jpg)
- viscosity of blood go up with rigid cells
![](https://photos.thisispiggy.com/file/wikiFiles/TBHaWlj.jpg)
- no spherocytes in G6PD with infection
![](https://photos.thisispiggy.com/file/wikiFiles/AubSNjf.jpg)
![](https://photos.thisispiggy.com/file/wikiFiles/j4ovCQI.jpg)
Backlinks