Hemolytic Anemia Flashcards
what is released upon RBC hemolysis?
lactate dehydrogenase (LDH) & hemoglobin
haptoglobin
binds to free hemoglobin in circulation. decreased free levels in intravascular hemolysis.
bilirubin conjugated vs unconjugated
breakdown of heme. conjugated form when released from liver, **unconjugated when released by hemolysis
clinical manifestations specific to hemolysis
jaundice, hemoglobinuria (specifically intravascular hemolysis), pigmented gallstone disease
notable causes of intravascular hemolysis
microangiopathy (MAHA), paroxysmal nocturnal hemoglobinuria (PNH), infections
causes of extravascular hemolysis
herediatry spherocytosis, G6PD deficiency, autoimmune, drug induced, infections
proteins defective in hereditary spherocytosis
band 3, spectrin, or ankyrin
diagnostic test for HS?
osmotic fragility test (HS cells lyse earlier)
treatment for HS?
splenectomy
G6PD deficiency
renders RBCs more susceptible to free radical damage due to inability to synthesize glutathione
morphological consequence of G6PD deficiency?
Heinz bodies (denatured hemoglobin aggregates in these within RBCs) with bite cells (macrophages nibble on the cells)
G6PD A-
decreased G6PD activity in aged RBCs (African Americans)
G6PD B
decreased G6PD activity in all RBCs (Mediterranean & Asians)
clinical manifestations of G6PD deficiency
precipitated by oxidative stress (acute illness, drugs, fava beans)
diagnosis of G6PD deficiency
G6PD biochemical assay (months after event)