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WorksheetsBlood Disorders
Total questions: 46
Worksheet time: 23mins
What common trigger can produce transient cold agglutinins?
Bacterial infection
Viral or other infections
Autoimmune disease
Drug reaction
Why should elective cardiac surgery be delayed in patients with cold agglutinins if hypothermia is planned?
Hypothermia increases myocardial oxygen demand
Hypothermia or cold cardioplegia can provoke agglutination
Hypothermia increases platelet consumption
Hypothermia reduces oxygen saturation
What is the basic perfusion strategy for patients with significant cold agglutinins?
Deep hypothermia with cold prime
Normothermic bypass with warm prime and warming devices
Moderate hypothermia with ice slush
Cold cardioplegia infusion
What procedure is required if the patient must be cooled below the thermal amplitude?
Exchange transfusion
Plasmapheresis
Administration of DDAVP
Hemodilution
Which is NOT a complication if a severe cold-agglutinin patient is cooled?
Myocardial infarction
Renal failure
Systemic thrombosis
Pulmonary embolism
How might the dilutional effect of the pump prime help with cold agglutinins?
It increases antibody concentration
It lowers antibody concentration, reducing risk
It removes IgM entirely
It prevents hemolysis by warming blood
What intra-operative warming measures are recommended for cold-agglutinin patients?
Use of ice packs
Use of warm prime and heating blanket
Avoid heating to prevent vasodilation
None are necessary
What cardioplegia strategy must be used if blood cardioplegia is chosen for cold-agglutinin patients?
Cold blood cardioplegia
Warm blood cardioplegia
Crystalloid only
Retrograde cardioplegia
How can cold cardioplegia be administered safely when cold agglutinins are present?
Use cold blood cardioplegia with rapid rewarming
Use crystalloid cardioplegia with warm flushing
Avoid cardioplegia entirely
Administer cold cardioplegia directly
What temperatures of typical cold blood cardioplegia would do to cold-agglutinin blood?
Prevent clotting
Cause almost certain agglutination
Stabilize antibodies
Reduce hemolysis
What key team action is essential when managing a patient with cold agglutinins?
Use of steroids preoperatively
All team members must create a warming plan
Avoid CPB entirely
Preoperative cooling
What causes sickle-shaped red blood cells in sickle cell disease?
Hemoglobin F
Hemoglobin S
Hemoglobin A2
Lack of iron
How do sickled red blood cells behave differently than normal RBCs?
They live longer than normal cells
They move slowly, clump together, and break down rapidly
They do not carry oxygen
They are unaffected by hypoxia
What is the predominant form of hemoglobin in a normal adult?
Hemoglobin F
Hemoglobin A
Hemoglobin S
Hemoglobin A2
What form of hemoglobin predominates in individuals with sickle cell disease?
Hemoglobin A
Hemoglobin F
Hemoglobin S
Hemoglobin C
What is the difference between sickle cell trait and sickle cell disease?
Trait is homozygous; disease is heterozygous
Trait involves <45% Hgb-S; disease involves majority Hgb-S
Trait only occurs in infants
Disease has no symptoms
What are typical symptoms of a sickle cell crisis?
Chest pain and fever
Extremity pain, shortness of breath, convulsions
Petechiae and bruising
Severe anemia only
What serious complications are sickle cell patients at risk for during CPB?
Blood clots, chronic anemia, and increased sickling
Only pulmonary embolism
Only thrombocytopenia
No additional risks
What is the only FDA-approved treatment for sickle cell disease?
Hydroxyurea
Bone marrow or stem cell transplant
Iron supplements
Exchange transfusion
What should the perfusionist’s primary strategy be when managing a sickle cell patient?
Induce hypothermia
Prevent sickling by avoiding hypoxia, acidosis, and hypothermia
Use high-dose heparin
Avoid oxygenation
At what oxygen saturation do patients with sickle cell disease typically sickle?
Below 95%
Below 85%
Below 70%
Below 40%
At what oxygen saturation do patients with sickle cell trait typically sickle?
Below 40%
Below 60%
Below 80%
Below 90%
What factors should be avoided in sickle cell patients to prevent sickling?
Hypoxia, acidosis, hypothermia
Hyperoxia, alkalosis
Hypothermia only
Normothermia
What type of cardioplegia regimen is best for sickle cell patients?
Cold blood cardioplegia
Warm cardioplegia regimen
Retrograde only
Crystalloid only
How can dilution help reduce the severity of sickle cell disease during CPB?
It increases hemoglobin S levels
It lessens concentration of sickled cells
It eliminates platelets
It reduces clotting factors
What is the purpose of preoperative exchange transfusions in anemic sickle cell patients?
Increase hemoglobin S
Replace sickled RBCs with healthy donor RBCs
Remove platelets
Reduce oxygenation
How do exchange transfusions reduce sickling risk?
By lowering oxygen saturation
By increasing hemoglobin A levels
By decreasing hemoglobin A levels
By eliminating all RBCs
How is intraoperative exchange transfusion performed in nonanemic patients?
Direct transfusion without cell salvage
Sequester sickled blood with cell saver and replace with donor blood
Exchange using only crystalloids
Remove plasma only
What happens to the separated components from the cell salvage process?
Discarded entirely
Normal plasma and platelets are saved for reinfusion
Only RBCs are reinfused
Only plasma is discarded
How should volume be maintained during intraoperative exchange transfusion?
By allowing hypovolemia
By keeping the patient euvolemic and infusing volume as needed
By avoiding arterial cannula use
By overloading with crystalloids
What hemoglobin A fraction is targeted after exchange transfusion in sickle cell patients?
30%
40%
60–70%
80–90%
What is the main concern when perfusing patients with fragile red blood cell disorders?
Minimizing oxygen delivery
Preserving red cell integrity with high oxygen saturation and normothermia
Using cold cardioplegia
Lowering hematocrit
What cardioplegia strategy is recommended for fragile red blood cell disorders if blood cardioplegia is used?
Cold blood cardioplegia only
Warm blood cardioplegia, or crystalloid with warm flushing if cold is needed
Avoid cardioplegia
Any cardioplegia without modification
What is polycythemia and who may exhibit it?
Low RBC count; infants only
High RBC count; patients with pulmonary or heart disease, or high-altitude dwellers
Low RBC count; only smokers
High platelet count; adults only
What perfusion adjustment is needed for polycythemia patients?
Lower-than-normal hematocrit after CPB
No specific action, but maintain higher-than-normal hematocrit after CPB
Use hypothermia
Avoid transfusions
What is thalassemia and its two main forms?
RBC overproduction; Major and Minor
Insufficient hemoglobin synthesis; Thalassemia Major and Minor
Platelet disorder; Cooley’s and Minor
Hemolytic anemia; Sickle and Non-sickle forms
What are the characteristics of red blood cells in thalassemia?
Macrocytic and hyperchromic
Microcytic, hypochromic, short life span
Normal size, normal lifespan
Large and fragile
What are key features of Thalassemia Major (Cooley's Anemia)?
Mild anemia, splenomegaly, minor RBC changes
Severe anemia, splenomegaly, failure to thrive, iron overload
Increased clotting, normal hemoglobin
Fragile RBC membranes
How is anemia treated in thalassemia patients on bypass?
Exchange transfusion
Packed red blood cells if necessary
Platelets
Cryoprecipitate
Do thalassemia patients have fragile red cell membranes during bypass?
Yes, with increased hemolysis
No, they are not especially fragile
Only if they have anemia
Always with CPB
What is thalassemia minor and how does it present?
Severe anemia, iron overload
Mild anemia, minor RBC changes, well-tolerated
Normal RBC count
Only splenomegaly
What is elliptocytosis?
Platelet disorder
Blood disorder with increased abnormal oval RBCs
Autoimmune anemia
Vitamin deficiency anemia
What percentage of red cells are usually elliptocytes in elliptocytosis?
Less than 15%
20–30%
50%
80%
What is spherocytosis and how does it affect red blood cells?
RBCs become oval and fragile
RBCs become spherical, fragile, and have shortened lifespan
RBCs become elongated
RBC membranes become thicker
What symptoms are associated with spherocytosis?
Petechiae, gum bleeding
Stomach pain, fever, splenomegaly
Chest pain, cyanosis
Thrombosis, dyspnea
How is spherocytosis treated?
Exchange transfusion only
Transfusions and possibly splenectomy
Platelet transfusion
Iron chelation
