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Hemolytic Anemias (Inherited and Acquired)

Terms in this set (56)

4 Basic Concepts of Hemolytic Anemias
-RBC Destruction is INCREASED
-Bone Marrow Compensation Occurs (INCREASED RBC Production)
-Bone Marrow RBC Production can INCREASE around 7 Fold
-Some patients have Hemolysis WITHOUT Anemia
3 Components Determining RBC Lifespan
-Membrane Cytoskeleton
-ATP-Generating Pathway
-Hemoglobin
Each day, ̴ 1% of the red cell mass dies and is replaced by...
-Reticulocytes
Haptoglobin Function
-binds free Hemoglobin that is released into the blood stream as a result of Hemolysis.
-thus, if Hemolysis is occuring, free Haptoglobin will be DECREASED on Lab Results
Laboratory Features of Hemolysis (2 Factors)
-Signs of RBC Destruction
-Signs of Accelerated Erythropoiesis
Signs of RBC Destruction (5 Signs)
↑ indirect bilirubin
↑ LDH (Lactate Dehydrogenase)
↓ haptoglobin
↑ plasma hemoglobin
↑ urine hemoglobin
Signs of Accelerated Erythropoiesis (3 Signs)
↑ reticulocytes
blood smear
bone marrow erythroid hyperplasia
Hemolysis Phenotypes
-Jaundice (due to Bilirubin)
-Red Plasma
Inherited Hemolysis Disorders
-RBC membrane disorders
-hereditary spherocytosis
-hereditary elliptocytosis
-RBC enzyme disorders
-G6PD deficiency
-PK deficiency
-RBC hemoglobin disorders
-sickle cell disease
-thalassemia
Acquired Hemolysis Disorders
-Immune-related
-auto-immune hemolysis
-hemolytic disease of the newborn
-transfusion reaction
-Non-immune-related
-infection (malaria, clostridia)
-red cell fragmentation
-TTP
-HUS
-DIC
-prosthetic heart valve
-Other disorders
-PNH
-severe liver disease
-hypophosphatemia
Two Anemias due to Cytoskeletal Defects
-Hereditary Spherocytosis
-Hereditary Elliptocytosis
Hereditary Spherocytosis Genetic Inheritance
-Autosomal Dominant (so positive family history)
-WHITE PEOPLE!
Hereditary Spherocytosis Clinical Features (3)
-Splenomegaly
-Jaundice
-Aplastic Episodes (Parvovirus B19)
Hereditary Spherocytosis Labs
-Osmotic Fragility Test! HS Cells will Hemolyze faster when incubated with Saline Dilutions
Hereditary Spherocytosis Treatment
-Splenectomy
-Folate
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