CPHON EXAM –Questions With Solutions
Autoimmune Hemolytic Anemia is a group of disorders characterized by
✔️Ans - malfunction of the immune system where antibodies are
produced against antigens on the surface of RBSs, resulting in hemolysis
T/F RBC survival is proportional to the amount of antibody on the RBC
surface; therefore the greater the amount of antibody the more rapidly the
RBC is destroyed. ✔️Ans - True
Intravascular Hemolysis is - ✔️Ans - RBC lysis in circulation - Antibodies
bind to the RBC membrane therefore activating the complement cascade -
damaged membrane causes increased osmotic pressure within cell and the
cell bursts
Extravascular hemolysis is ✔️Ans - occurs when complement fixation to
RBC fails to activate complement cascade - the complement on the RBC
surface interacts with receptors in macrophages in the lungs, liver, and
spleen -> RBC phagocytosis
Causes of AIHA (4) ✔️Ans - 1. Autoimmune disorders (LUPUS) 2.
Infections (hepatitis, EBV, myco pneu) 3. Drugs (peni and quinine) 4.
Hematologic disorders(Evan's syndrome and paroxysmal nocturnal
hemoglobinuria)
Incidence of AIHA ✔️Ans - 1 case per 80,000 persons
Clinical Symptoms of Severe AIHA - ✔️Ans - - pallor - jaundice - fatigue -
tachycardia -hypoxia --> organ damage - splenomagaly
What labs should be ordered if AIHA is suspected? ✔️Ans - cbc d/p,
retic, peripheral smear, Coombs test, bilirubin, LDH, and haptoglobin
Labs findings that suggest AIHA is possible: ✔️Ans - 1. Coombs direct
(DAT) : + which indicates antibodies against the RBC 2. Low Hemoglobin 3.
Increased Retic 4. Spherocytes, schistocytes, or erythrocyte agglutination
on blood smear 5. Increased LDH 6. Decreased haptoglobin 7.
Hemoglobinuria 8. Increased Unconj bilirubin
, Possible parts of the Treatment Plan of AIHA ✔️Ans - 1. Stop medication
if suspected as cause 2. Prednisone 2-4 mg/kg/day 3. High Dose IVIG
4.Splenectomy 5. pRBC transfusion 6. Folic Acid supplementation 7.
Plasmapheresis (b/c IgM is confind to the intravascular space) 8. Cytotoxic
agents 9. Immunosuppressive agents (Cyclosporine) 10. Hormonal therapy
(danazol)
What are the three types of cytoxic agents that can be used for AIHA?
✔️Ans - 1. Antimetabolites (6-mecaptupurine, azathioprine) 2. alkylating
agents (cyclophosphamide) 3. Mitotic agents (vincristine, vinblastine)
T/F Cold Antibody AIHA is most common AIHA, in whcih the
autoantibodies become most active and attack RBCs usually at
temperatures well below normal. . ✔️Ans - False. 75% of cases are warm
body
Pathophysiology of Warm Body AIHA IgG ✔️Ans - is the most common
antibody > attaches to RBC > recognized by monocytes and macrophages in
the spleen > destroy RBC membrane> RBC changes shape and singled out
for destruction
T/F 50% of cases if warm anitbody AIHA are idiopathic (primary) ✔️Ans
- True
Prognosis of AIHA - ✔️Ans - Usually transient - less than 3 months -
usually resolve spontaneously
Cold Antibody AIHA is most common in children when - . ✔️Ans -
secondary to infection - IgM or IgG cold reacting antibodies that cross react
with the ABO antigens on the surface of RBCs are produced
What organ is the main site of hemolysis in cold antibody AIHA? ✔️Ans -
Liver
Primary cold agglutinin disease is : ✔️Ans - chronic or transient chronic
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