Hemolytic Anemia Flashcards
What is hemolysis
Premature distraction of erythrocytes
What causes hemolytic anemias
Increased rates of red cell destruction
Lifespan of red cells
90 to 120 days
What happens to the bone marrow when there is hemolytic anemia
There is a Eryhtroid hyperplasia
What cells are responsible for the removal of red cells that are destructed
Extra vascular macrophages of the reticuloendothelial system
In which organ does the removal of destructed red cell occur
Deliver the spleen and mostly the bone marrow
Breakdown pathway of normal red cell
Hemoglobin broken down to haem and globin
Globin broken down into amino acids
Haem broken down into iron and Protoporphyrin
Iron -> transferrin -> erythroblast
Protoporphyrrin-> free bilirubin -> bilirubin glucuronides in liver -> stercobilirubin -> faeces or urine ( urobilinogen)
Main organ that removes old and damaged red blood cells from circulation
Spleen
First way the body compensate when there is higher rates of red blood cell breakdown
By increasing production of red blood cell
Why is there jaundice in hemolytic anemia
Free BiliRubin from red blood cell destruction
Why do red blood cells are more susceptible to oxidative damage and a short abnormal life cycle
Because they lack mitochondria so rely mostly on glycolysis for materials needed to reduce oxidative damage
What is Intravascular hemolysis
Release of red blood cell contents into the bloodstream
Classification of hemolytic anemia based on site of hemolysis
Extravascular hemolytic disorder
Intravascular hemolytic disorders
Combination of both extra and Intravascular hemolysis
What happens in extravascular hemolysis
Red cell destruction occurs in the cells of the reticuloendothelial system
Laboratory signs of extravascular hemolysis
Indirect hyperbilirubinemia
Increased excretion of bilirubin by bile
Erythroid hyperplasia
hemosiderosis
Clinical states associated with extravascular hemolysis
Auto immune hemolysis delayed hemolytic transfusion reactions hemoglobinopathies hereditary spherocytosis Hypersplenism
What happens inIntravascular hemolysis
Destruction of red blood cell in the blood vessels
What are the clinical states associated with Intravascular hemolysis
Severe and extensive burns acute hemolytic transfusion reactions paroxysmal nocturnal hemoglobinuria severe microanngiopathic hemolysis physical trauma bacterial infections and parasitic infections
Classification of Hemolytic anemia based on site of defects
Intrinsic defect
Extrinsic defect
Classification of hemolytic anemia based on inherited or acquired States
Inherited hemolytic anemia mostly caused by intrinsic defects
Acquired hemolytic anemia mostly caused by extrinsic defect
What disease is a an exception to the classification of inherited or acquired hemolytic anemia
Paroxysmal nocturnal hemoglobinuria is an exception because it’s acquired but has intrinsic defect
disease linked with hereditary HA
Membrane defects - hereditary spherocytosis
Metabolic defects - G6PD
Hemoglobin defects - sickle cell disease
Acquired HA disease conditions
Autoimmune Alloimune Red cell fragmentation syndrome March Hemoglobinemia Infections Chemical and physical agents PNH
Diagnosis of hemolytic anemia
Family history
Ethnic origin (Méditerranean and Chinese with G6PD )
Past history like neonatal jaundice
Triggering events - drugs history, infections
Clinical features of HA
Pallor of mucus membrane Jaundice Splenomegaly Dark urine Pigment gall stones Ulcers around ankle Aplastic crisis Growth retardation Hypertrophic skeletal changes
Laboratory findings of HA which shows increased red cell breakdown
High serum bilirubin Unconjugated Increased urine urobilinogen Increased fecal stercobilinogen Reduced serum haptoglobin Reduced serum hemopexin Increased LDH
Peripheral blood film findings in HA
Normocytic and Macrocytic red cells Polychromasia Nucleated red blood cells Schistocyte Microspherocytes Elliptocytes Helmets cells Reticulocytes
HA Findings in bone marrow
Erythroid hyperplasia
Test that shows shortening of red cell survival
51 Cr labelling
Reticulocytosis is a sign of …
Increased red cell production
Reticulocyte stain
New methylene blue & brilliant Crésyl blue
Lab feature of Intravascular hemolysis
Hemoglobinaemia
Hemoglobinuria
hemosiderinuria
Methaemalbumin
Color appearance of urine in hemoglobinuria
Dark
