CPHON EXAMS KEY CONCEPTS AND
PRACTICE QUESTIONS
◉ 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..
Answer: True
◉ Intravascular Hemolysis is -.
Answer: 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.
Answer: 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).
Answer: 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.
Answer: 1 case per 80,000 persons
◉ Clinical Symptoms of Severe
Autoimmune Hemolytic Anemia.
Answer: - pallor
- jaundice
- fatigue
- tachycardia -
hypoxia --> organ damage - splenomagaly
◉ What labs should be ordered if AIHA is suspected?.
Answer: cbc d/p, retic, peripheral smear, Coombs test, bilirubin,
LDH, and haptoglobin
◉ Labs findings that suggest AIHA is possible:.
Answer: 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.
Answer: 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?.
Answer: 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. ..
Answer: False. 75% of cases are warm body
PRACTICE QUESTIONS
◉ 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..
Answer: True
◉ Intravascular Hemolysis is -.
Answer: 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.
Answer: 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).
Answer: 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.
Answer: 1 case per 80,000 persons
◉ Clinical Symptoms of Severe
Autoimmune Hemolytic Anemia.
Answer: - pallor
- jaundice
- fatigue
- tachycardia -
hypoxia --> organ damage - splenomagaly
◉ What labs should be ordered if AIHA is suspected?.
Answer: cbc d/p, retic, peripheral smear, Coombs test, bilirubin,
LDH, and haptoglobin
◉ Labs findings that suggest AIHA is possible:.
Answer: 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.
Answer: 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?.
Answer: 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. ..
Answer: False. 75% of cases are warm body
