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Hemolytic anemia (Intrinsic red cell defect (Membrane (Paroxysmal…

- - - - Dx - PS elliptocytes
      - Milder hemolysis than HS
    - - Mutation of PIG-A need to make GPI --> loss of GPI-linked proteins CD55 and CD59 --> cell without GPI, cells more easily targeted by complements --> RBC lysis
      - Symptoms
        
        Chronic intravascular hemolysis
        
        Hemosiderinuria - dark urine int he morning
        
        Thrombosis
        
        Pancytopenia (not always)
        
        BM failure
      - A/w aplastic anemia, MDS
      - Dx
        
        Flow cytometry - loss of GPI-linked proteins CD55, CD59
        
        PNH screen - screen for granulocyte, monocyte clones, PNH type I/II/III cell
        
        Type I - normal RBC, has CD59
        
        Type II - RBC with slight sensitivity to complement system, missing some CD55, CD59
        
        Type III - RBC v sensitive to complement system, no CD55, CD59
      - Tx
        
        Eculizumab - humanized monoclonal antibody against C5, inhibit terminal complement activation
        
        Immunosuppression, SCT
        
        Anticoagulation
      - Intravascular hemolytic anemia
      - Complications - IVC, portal vein thrombosis during pregnancy
      - Classifications
        
        Classic PNH - HA, thrombosis, BM failure
        
        PNH clones - detected by PNH screen, a/w AA, MDS
        
        Subclinical PNH
    - - Autosomal dominant
      - Dx
        
        PS - spherocytes
        
        Negative direct Coombs test
        
        CBC - Increased MCHC
        
        Flow cytometry - eosin-maleimide binding to red cells
      - Intrinsic red cell membrane defect - increased fragility and shortened life span
      - Neonatal jaundice, gallstones, splenomegaly
      - Tx
        
        Folate replacement
        
        Monitor growth and development
        
        Symptoms - splenectomy, cholecystectomy
    - - Dx - PS ovalocytes, somatocytes, coffee beans
      - Asymptomatic
  - - - X-linked
      - Acute hemolytic anemia in response to oxidative stress, neonatal jaundice
      - Dx - PS irregular contract cells, bite cells, blister cells, Heinz bodies
      - Triggered by infection, acute illness, drugs, fava beans, antimalarials, analgesics, antibacterials, sulphonamides
- - - - Dx
        
        Positive Coombs test
      - Warm AIHA
        
        Caused by autoimmune IgG anitbody --> antibody coats red cells, eaten by macrophages
        
        Extravascular hemolytic anemia
        
        Direct positive Coombs test
        
        Tx
        
        Blood transfusion if severe symptomatic
        
        Monoclonal antibody - Rituximab
        
        Remove underlying cause
        
        Steroid, immunsuppression
        
        Folic acid
      - Cold AIHA
        
        Cause by autoimmune IgM antibody --> complement fixing
        
        Chronic hemolytic anemia aggravated by the cold
        
        Intravascular hemolytic anemia
        
        Cold red cell agglutination
        
        PCH - paroxysmal cold hemoglobinuria
        
        A/w malignancies, viral infections, syphillis
        
        Donath-Landsteiner test for anti-P IgG antibody that binds at cold temp and causes hemolysis at warm temp
        
        Tx
        
        Keep warm
        
        Treat underlying cause
        
        Rituximab, chlorambucil, purine nucleoside analogues
    - - Transfusion and transplantation
        
        Hemolytic transfusion reactions - red cell antigens
      - Hemolytic disease of newborn (HDN)
        
        ABO incompatability
        
        Rhd alloimmunisation
      - Drug induced
        
        Drug and red cell membrane forms complex
        
        Immune complex
  - - - Microangiopathic hemolytic anemia
        
        Pre-eclampsia
        
        TTP
        
        Autoantibody that blocks ADAMTS13 protease which cleaves large vWF, large vWF more thrombogenic
        
        In autoimmune diseases, malignancies, infections, pregnancy
        
        Symptoms
        
        Fever
        
        Microangiopathic hemolytic anemia - red cell fragmentation
        
        Thrombocytopenia
        
        Renal impairment
        
        Neurological signs
        
        DIC
        
        Non-localised coagulation, thrombosis and intravascular fibrin deposits
        
        Acquired, complicates other illnesses
        
        Symptoms
        
        Thrombosis
        
        Bleeding
        
        Causes
        
        Widespread tissue damage
        
        Infections
        
        Malignancy
        
        Obstetric complications
        
        HUS
        
        Symptoms
        
        Microangiopathic hemolyic anemia
        
        Thrombocytopenia
        
        Severe renal impairment
        
        Minimal neurological symptoms
        
        Not due to ADAMTS13
        
        Typical
        
        GE with E coli, Shigella
        
        Atypical
        
        No GE, triggered by infection
        
        Some people genetically predisposed
      - After cardiac surgery
      - Arteriovenous malformations