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
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
- antimalarials can trigger hemolysis
- 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
Backlinks