Extracorpuscular Hemolytic Anemias Flashcards
Classifications of hemolysis
- acute vs chronic
- inherited vs acquired
- intravascular vs extravascular
- intracorpuscular vs extracorpuscular (intrinsic vs extrinsic)
acute
rapid onset, shorter time course
chronic hemolysis
slow, with compensation from bone marrow
premature destruction of structurally and functionally normal RBCs due to mechanisms outside of the RBCs
extracorpuscular hemolytic anemia
Causes of Intracorpuscular Hemolysis
- membrane defects
- enzyme defects
- hemoglobinopathies
- PNH
Causes of Extracorpuscular Hemolysis
- non-immune causes: microangiopathic hemolytic anemias, infectious agents, mechanical forces
- immune causes: alloimmune hemolytic anemias, autoimmune hemolytic anemias
caused by a condition that leads to physical or mechanical RBC damage
non-immune hemolytic anemias
- microangiopathic, macroangiopathic
abnormalities in the microvasculature
microangiopathic
abnormalities in the heart or large blood vessels
macroangiopathic
two other causes on non-immune hemolytic anemias
- infectious agents
- chemical and physical agents: chemicals, drugs, venom, thermal injury
microangiopathic hemolytic anemia
- intravascular hemolysis = RBC fragmentation
- thrombocytopenia
thrombotic microangiopathies (TMAs)
- damage occurs when RBCs quickly pass through turbulent areas of small blood vessels partially blocked by microthrombi
- combination of MAHA and thrombocytopenia in the appropriate clinical setting
PBS Morphologies of MAHAs and TMAs
- keratocytes (horn cells)
- helmet cells
- triangulocytes
- microspherocytes
Top 4 scary microangiopathic hemolytic anemias
- thrombotic thrombocytopenic purpura
- hemolytic uremic syndrome
- disseminated intravascular coagulation (DIC)
- HELLP syndrome
What causes thrombotic thrombocytopenic purpura?
a deficiency of ADAMTS13
- protease responsible for cleaving ‘ultra-large’ multimers of von Willebrand Factor, which are involved in coagulation