hemolysis Destruction or lysis of RBCs
Hemolysis
Intracorpuscular hemolysis
• Membrane defects, eg hereditary elliptocytosis, spherocytosis, stomatocytosis and paroxysmal nocturnal hemoglobinuria
• Metabolic defects, eg G6PD, pyruvate kinase deficiency
Extracorpuscular hemolysis
1º immune reactions, eg autoimmune hemolytic anemia
2º immune reactions, due to
• Infections, eg Bartonella, Clostridia, malaria, sepsis
• Neoplasia, eg lymphoma, leukemias
• Drug reactions due to the 'Innocent bystander' phenomenon (drug-antibody complex activates complement, causing intravascular hemolysis, eg quinidine), hapten-mediated —a protein-bound drug attaches to the red cell membrane, eliciting an immune response when the hapten-protein complex is recognized as foreign, evoking an immune response, eg penicillin acting as a hapten
• Induction of autoimmunity by RBC antigen alterations, eg Rh antigen
Physical, eg thermal, concentrated glycerol due to inadequate washing of frozen blood, bladder irrigation, cardiac valves
Extravascular Less severe, IgG-mediated and does not activate complement, eg Rh, Kell, Duffy Laboratory ↓ haptoglobin, ↓ T1/2 of circulating RBCs, ↑ indirect BR as liver capacity to conjugate BR–ergo direct BR is overwhelmed by massive hemolysis, ↑ LDH, Hb in blood and urine, hemosiderinuria, MetHb and metalbumin, ↑ urobilinogen in urine and feces, ↑ in acid phosphatase, K+, and prostatic acid phosphatase Clin Chem 1992; 38:575; peripheral smears demonstrate anisocytosis, polychromatophilia, nucleated RBCs, basophilic stippling; immune hemolysis is suggested by spherocytes NEJM 2000; 342:722cpc
Intravascular More severe, IgM-mediated and requires complement activation, eg ABO blood groups Laboratory ↑ free Hb Note: Clinically significant hemolysis is usually detected by hemagglutination, less commonly by hemolysis per se, which detects anti-P,
-P1, -PP1Pk, -Jka, -Lea, occasionally also anti-Leb and -Vel