10 Intrinsic Hemolysis

Cause

  • normally complement does not lyse RBC

  • acquired: genetic mutation some point in life
  • GPI: anchors that attach DAF/CD59 to membrane
  • stem cell: RBC, platelets, WBC also involved

Pathogenesis, Symptoms

  • Free plasma Hgb thrombogenic, thrombosis
  • platelets lysed: spill granules
  • Priapism: sustained prolonged erection

  • paroxysmal: sudden
  • episodes at night when urine changes color

  • hypercoagulable

Diagnosis, treatment

  • antibodies not part of problem

Pyruvate Kinase Deficiency

  • when levels low, RBC most affected (require pyruvate kinase and glycolysis for anerobic)
  • no ATP, membrane fails
  • when problem with membrane, eaten in spleen

  • disease severity based on how low enzyme activity is

G6PD Deficiency

  • especially fava beans

  • antimalarials can trigger hemolysis

  • know the countries

  • Can't make ATP, RBC become rigid, cannot maintain membrane
  • most extravascular
  • some become so damaged/rigid: intravascular

  • Bite and seeds: bite cells and Heinz bodies seen in RBCs
  • Broken G6PD-free fruit: hemolytic anemia in G6PD deficiency

  • Bite and seeds: bite cells and Heinz bodies seen in RBCs
  • Broken G6PD-free fruit: dapsone can cause hemolytic anemia in G6PD deficiency

  • "Color queen" - chloroquine, beads join together as polymer, blocking plasmodium heme polymerase. High resistance
  • "Primal queen" - primaquine, can cause anemia in G6PD deficiency

  • fluorescent spot: screening test
  • pts unable to generate NADPH
  • normal: generate NADPH, fluoresce
  • must test when not having acute attack

Hereditary Spherocytosis

  • proteins give RBC biconcave shape and flexibility
  • abnormal: rigid membrane
  • spectrin for spherocytosis

  • problem lies in chronic extravascular hemolysis, not O2 carrying capacity

Diagnosis

  • 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

  • material density goes up
  • MCHC: mean corpuscular hemoglobin concentration
  • MCV: mean corpuscular volume: average size of RBCs
  • chronic hemolysis: more reticulocytes, high MCV

  • viscosity of blood go up with rigid cells

  • no spherocytes in G6PD with infection