Hemolytic Anemias

Hemolytic Anemias

1. Intravascular hemolysis

   • Paroxysmal nocturnal hemoglobinuria
     • Missing CD55 and CD59
     • Positive Ham's test
   • Autoimmune hemolytic anemia
   • Mechanical destruction
     • MAHA
       • DIC
       • HUS
       • TTP
       • Macroangiopathic
         • Prosthetic valves
         • Aortic stenosis

2. Extravascular hemolysis

   • Hereditary spherocytosis
     • Ankyrin
     • Spectrin
     • Increased MCHC and RDW
     • Splenomegaly
     • Aplastic crises due to parvovirus B19
     • Osmotic fragility test
     • Splenectomy
   • G6PD deficiency
     • Sensitive to oxidative stress
       • Sulfa drugs
       • Fava beans
       • Infections
     • Heinz bodies
       • Bite cells
     • X-recessive
   • Pyruvate kinase deficiency
     • RBCs cannot generate ATP
     • Swelling and hemolysis of the red cells
     • ARecessive
   • Sickle cell disease
     • Low oxygen tension
     • Dehydration
     • Complications
       • Sequestration crisis
       • Autosplenectomy
       • Salmonella osteomyelitis
       • Aplastic criss due to parvovirus B19
       • Pain crises due to vasoocclusion
       • Renal papillary necrosis due to hypoxemia
     • Treatment
       • Hydroxyurea
       • Bone marrow transplantation
   • Hemoglobin C disease
     • Heterozygous: asymptomatic
     • Homozygous: mild hemolysis and splenomegaly
     • SC disease
     • HBC crystals
       • Increased MCHC
   • Alpha Thal
     • Silent carrier
       • Deletion of one gene
     • Alpha thal trait
       • Deletion of 2 genes on same chr. or one on either loci
         • Cis- common in asian and dangerous
         • Trans in African
     • HbH
       • Deletion of 3 genes
     • Hb Bart
       • Deletion of 4 genes
       • Erythroblastosis fetalis
       • Transfusion dep
       • Hydrops fetalis
   • Beta Thal
     • Beta Thal major: Total abscence of b globin
       • Clinical features
         • Chipmunk facies
         • Crew cut or hair on end on x-ray
         • Hepatosplenomegaly
           • Extramedullary hematopoeisis
           • Risk
             • HCC
               • Hemochromastosis
       • Hb electrophoresis
         • Marked raise in HbF, LOW HbA
     • Beta thal minor: Some beta chains
       • Microcytic hypochromic
         • Mentzer index<13
         • Hb electrophoresis : Raise in HbA2

3. Warm agglutinins (IgG antibodies)

   • Lupus
   • CLL
   • Drugs

4. Cold agglutinins (IgM antibodies)

   • CLL
   • Mycoplasma infections
   • Infectious mononucleosis: EBV

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