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Sickle Cell
Classified as hemolytic anemia
Autosomal recessive; mutation of beta globin chain of hgb resulting in defective hgb
Sickle-shaped cell
- Unstable and when exposed to acidosis or oxidative stress (hypoxia) the cell sickles
permanently
- Cells cannot change shape which prevents them from passing through narrow capillaries
resulting in occlusion of small blood vessels and tissue ischemia
- Sickled cells life span: 10-20 days
Sickle cell lab findings
- Hemoglobin electrophoresis confirms diagnosis
- Hct 20-30%
- Hgb 8-10
- Peripheral smear: sickled cells, target cells, howell-jolly bodies (spleen)
- Elevated reticulocytes
- Nucleated RBC's
- Elevated WBC's (12-15K)
Sickle Cell Signs and Symptoms
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,Painful episodic attacks brought on by infection, dehydration, hypoxia
▪ Episodes last hours to days
Vaso-occlusion
Jaundice caused by hemolysis
Pigment gallstones
Splenomegaly/infarcted spleen
- Splenic sequestration crisis
• Sudden pooling of blood in spleen - hypovolemia - distended abdomen - circulatory
collapse - surgical emergency
Poorly healing ulcers over lower tibia
Platelet functions
▪ Synthesize enzymes, ATP, ADP, prostaglandins, store calcium ions
▪ Help regulate blood flow into a damaged site by inducing vasoconstriction
▪ Initiate platelet-platelet interactions, resulting in the formation of a platelet plug
▪ Activate the coagulation cascade to stabilize the platelet plug
▪ Initiate repair processes including clot retraction and clot dissolution (fibrinolysis)
Platelet values
Normal: 150,000-400,000
Thrombocytopenia = Less than 100,000
- Less than 50,000 = s/s develop and risk of hemorrhage increases
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,- Less than 20,000 = spontaneous bleeding may occur - elevated spontaneous thrombosis
may occur
Calcium Role in clotting
- Required in multiple steps in the process of clotting cascade for activating clotting factors
- Prothrombin activator causing prothrombin to be converted to thrombin is completely
dependent on adequate _______
Make up of platelet cell membrane
▪ Have glycoproteins on surface that repulse adherence to normal endothelium and
promote adherence to injured endothelium
▪ Contains phospholipids that activate several stages in the blood clotting cascade
Destruction of old (senescent) erythrocytes
▪ Globin broken down into amino acids
▪ Iron is liberated from heme, oxidized and recycled (transferrin to ferritin)
▪ Porphyrin of heme is metabolized to bilirubin - transported to liver and conjugated -
excreted as bile into intestine - transformed to urobilinogen - excreted with feces, some
through ▪ Changes on outer surface of old erythrocyte attract macrophages
▪ Tissue macrophages digest erythrocyte - heme and globin dissociate
feces
Platelet life cycle
8-10 days
Irregularly-shaped cytoplasmic fragments formed by the fragmentation of megakaryocyte
progenitor cells
- produced in the bone marrow
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, - stored in the spleen where they are slowly released
Eliminated: tissue macrophage system within the spleen
Sequence of hemostasis
1. Vascular injury leads to vasoconstriction (local constriction)
2. Formation of a platelet plug (primary hemostasis)
3. Tissue factor activates coagulation cascade
4. Formation of a blood clot (secondary hemostasis)
5. Clot retraction and clot dissolution (fibrinolysis)
formation of platelet plug
▪ Adhesion: mediated by the binding of platelet surface receptor glycoprotein to vWF
("anchor")
▪ Activation: smooth platelet spheres change to spiny projections and degranulation
(platelet-release reaction) results in the release of various potent biochemicals
▪ Aggregation: facilitated by fibrinogen bridges between receptors on the platelets
▪ Clot retraction: fibrin strands shorten and become denser and stronger to approximate
the edges
Formation of blood clot (secondary hemostasis)
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