Approach to HemolysisKuang PuDivision of Hematology, West China Hospital *

Pathogenesis in HemolysisLifespan of RBC

Clinical manifestation of Hemolysis

HemolysisExtravascularHereditaryMembrane DefectsSpherocytosis Elliptocytosis Hemoglobinopathies Sickle Cells AnemiaUnstable HemoglobinsAcquiredAutoimmune Hemolytic Anemias()Associated with warm antibodiesAssociated with cold antibodiesHypersplenism()IntravascularHereditaryEnzyme DeficiencyG6PD DeficiencyPyruvate Kinase DeficiencyAcquiredParoxysmal Nocturnal Hemoglobinuria Drug induced Hemolytic AnemiasInfectionsPlasmodiamU(), Clostridium ()Isoimmune Hemolytic AnemiasABO mismatched transfusionMicroangiopathic Hemolytic AnemiasDIC HUS-TTP

Extra vascular hemolysisDestruction of RBC occurs extra-vascularly. Most commonly seen.Macrophages in the reticuloendothelial system, particularly the spleen are responsible for clearance of RBC fragments

Intra vascular HemolysisDestruction of RBC occurs intra-vascularlyNot commonly seen, acute onsetHemoglobin released into the bloodMetabolize throughHaptoglobin() LiverProximal tubule of kidneyHemosiderin Prussian Blue() or Hemoglobin in urine

Any Questions

Hemolytic are a group of disorders characterised by shortened survival of RBCs in circulation .A. Isoimmune Hemolytic disease of the newborn incompatible blood transfusion

In most hemolytic conditions red cell destruction is extra vascular. The red Cells are removed from the circulation by macrophages in the reticuloendothelial system, particularly the spleen.

When red cells are rapidly destroyed within the circulation Hb is liberated. This is initially bound to plasma haptoglobin but these soon become saturated. Excess free plasma Hb is filtered by the glomerulus and enters the urine, although small amounts are reabsorbed by the renal tubules. In the renal tubular cells Hb is broken down and becomes deposited in the cells as haemosiderin.