Autoimmune Hemolytic Anemia is a group of disorders characterized by - Answer 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 ...
CPHON EXAM questions with correct answers
Autoimmune Hemolytic Anemia is a group of disorders characterized by - Answer 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. - 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 is - 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 AIHA - - 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
Pathophysiology of Warm Body AIHA IgG - Answer 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) - Answer True
Prognosis of AIHA - - Answer Usually transient - less than 3 months - usually resolve spontaneously Cold Antibody AIHA is most common in children when - . - Answer 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? - Answer Liver
Primary cold agglutinin disease is : - Answer chronic or transient chronic
Most common infection causing secondary cold antibody AIHA is: - Answer Mycoplasma pneumoniae but also viral (measles, mumps, flu, EBV, Adeno, VZV, CMV) and bacterial (syphilis and HIB)
T/F Blood products should be washed and warmed before transfusion for persons with Cold Antibody AIHA - Answer True
6 nursing assessment and interventions for AIHA - Answer 1. Read labs for decreased hemoglobin 2. Monitor for anemia 3. Monitor for transfusion complications (rxn and fluid overload) 4. Maximize child's physical tolerance 5. Teach family about AIHA 6. Teach aout post slenectomy care prn
Definition of Sickle Cell Disease - - Answer a hemogloinopathy -DNA mutation for hbg production - normal hemoglobin or hgb A is absent, but hbg S is present (alone or in combination with another form of abnormal hemoglobin)
Who first described SCD? - Answer James Herrick (1904
SCD pathophysiology - - Answer Hbg two pairs of polypeptide chains (alpha and beta) > on 6th position on the Beta chain in hbg A - glutamic acid is replaced by
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