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Autoimmune hemolytic anemia is caused by what?
Autoantibodies
If you have autoantibodies, do you have autoimmune hemolytic anemia?
Not necessarily. They have to show immune mediated RBC destruction
If a person has AIHA, what would their test results look like for blood bank and other departments?
blood bank: + DAT, + auto control, + antibody screen with panagglutination, panreactive eluate
Other departments: low Hgb/Hct, low haptoglobin, high bilirubin, high retic count, high LDH
What causes autoantibodies?
Loss of suppressor T-cell function
What are some reasons for having a positive DAT?
-autoantibody
-alloantibody
-maternal antibody
-drug related process
Difference between benign and pathologic CAA
benign: thermal range below 20 - 24 C, Titer < 64, marginally enhanced with albumin, anti-I or anti- H
pathologic: may be reactive >30 C, titer > 1000, strongly enhanced by albumin, Anti-I
If you have an ABO discrepancy with a CAA, how do you fix it?
-37 degree backtype
-Prewarm technique
If you have panreactivity from a CAA on an ABID, how do you fix the problem?
(Hint* answer changes if the patient has been transfused)
If the patient has not been transfused, you do a prewarm. If they have been transfused, use RESt.
What do you do if your autocontrol comes out positive?
Perform a DAT
When do you use a alloadsorption vs an autoadsorption?
auto = patient has NOT been transfused and you have enough cells
allo = patient has been transfused or you do NOT have enough cells
What are two pathological CAAs?
CHD and PCH
defining characteristics of CHD
-found in old people
-associated with pneumonia or mono
-anti-I
-chronic
-extravascular/ intravascular hemolysis
-IgM
-titer > 1000
-fix by avoiding the cold
Defining characteristics of PCH
-seen in children
-follows viral infections
-acute
-intravascular hemolysis
-IgG (biphasic)
-anti- P
- < 64 titer
- + donath-landsteiner test
- treat by fixing underlying cause
What is the most common autoantibody?
Warm autoantibodies (70%)
What are characteristics of WAAs?
-IgG
-react best at AHG
-enhanced by enzymes
-antibody panel looks positive in the AHG column for everything including auto control
-eluate will be positive for everything
What are some transfusion consideration when working with WAAs?
-only transfuse if clinically indicated
-transfuse blood that is ABO, Rh and Kell compatible
Drug Adsorption:
drug involved
process
DAT results
eluate results
hemolysis types
-penicillin, cephalosporins
-drug binds RBC and is recognized by antigen
-IgG +++, C3 ++
-ABSC neg, eluate neg
-extravascular
Immune Complex:
drug involved
process
DAT results
eluate results
hemolysis types
-Quinine, phenacetin
-AB/drug complex binds to RBC
-IgG +, C3 +++
-ABSC neg, eluate neg
-intravascular
Induction of autoantibody (methyldopa):
drug involved
process
DAT results
eluate results
hemolysis types
-methyldopa
-alters membrane or down regulates normal immune suppression
-IgG +++, C3 +
- ABSC pos, eluate pos
-extravascular
Membrane Modification:
drug involved
process
DAT results
eluate results
hemolysis types
-cephalosporin
-drug coats RBC
-IgG+, C3+, IgM+
-ABSC neg, eluate neg
What vitals must be measured pre, during, and post transfusion?
-temp
-blood pressure
-pulse
Difference between acute and delayed reactions?
acute - less than 24 hours
delayed - greater than 24 hours
What are the three febrile acute transfusion reactions?
-AHTR
-TAS
-FNHTR
What are the two allergic acute transfusion reactions?
mild and severe
What are the two pulmonary transfusion reactions?
-TACO
-TRALI
What is the transfusion reaction we are most worried about?
Acute hemolytic transfusion reactions
What is the only drug induced hemolytic anemia that causes intravascular hemolysis?
Immune complex
If someone is on methyldopa, what will their test results look similar to?
it will look like a WAA
How do you tell the difference between a mimicking anti-e autoantibody, and an actual anti-e antibody?
phenotype the patient. If it is an actual anti-e they will be e negative for the antigen
What is the work flow for a WAA?
-Type and screen
-screen is positive
-ABID
-panreactive ABID including auto ctrl
-DAT
-IgG positive
-eluate
-panreactive eluate
-adsorption
What is the work up for a CAA?
-Type and screen
-type discrepancy
-short cold panel
-panreactivity
-do 37 degree backtype
-if that doesnt work, prewarm
-If ABSC positive, do ABID
-ABID panreactive
-DAT
-IgG neg
-prewarm
Can you do a blood type using RESt plasma?
No. It reduces the titer of anti-B
What cells are need to perform a donath-landsteiner test?
-O type
-P+
-50% cell suspension
If a donath landsteiner test needs to be done, how is the sample collected?
-drawn in red top
-transported at 37 C to blood bank immediately
Acute Hemolytic Transfusion Reaction:
cause
temp
blood pressure
pulse
symptoms
blood bank work up
hemolysis
-caused by ABO incompatibility
-temp increases
-pulse increases
-blood pressure decreases
-pain, hemoglobinuria
-DAT positive
-intravascular hemolysis
Transfusion Associated Sepsis:
cause
temp
blood pressure
pulse
blood bank work up
-contaminated products
-temp increases at 2 C
-pulse increases
-blood pressure decreases
- don't see anything, culture only of medical director indicates
Febrile nonhemolytic transfusion reaction:
temp
blood pressure
pulse
blood bank work up
-temp increases
-blood pressure and pulse can go either way
-nothing in the workup
Mild allergic reaction
Look for a rash, do a clerical work up, treat with benadryl then you are done
Severe allergic reaction:
SPO2
Respiration
temp
pulse
blood pressure
Blood bank workup
-spo2 decreased
-respiration increased
-temp maybe increase
-pulse increase
-blood pressure maybe increase
-nothing in workup
How do you prevent a severe allergic reaction during transfusion from happening?
-give washed cells
TACO
temp
pulse
blood pressure
spo2
respiration
caused by
symptoms
treatment
temp no change
pulse increase
blood pressure variable
spo2 decreases
respiration increases
caused by circulatory overload
symptoms jugular distention, wheezing, coughing, pulmonary edema
responds to diuretics
TRALI
temp
pulse
blood pressure
SPO2
respiration
symptoms
treatment
temp variabel
pulse increases
blood pressure increase
SPO2 decreases
respiration increases
symptoms coughing, wheezing
treatment oxygen therapy
What is the number one cause of transfusion fatalities in the US?
TRALI
What 3 transfusion reactions are considered to by immune responses?
DHTR
AHTR
Severe allergic reaction
What is a key indication you have graft vs host disease?
maculopapular rash (starts in the core and extends outwards toward extremities)
What is the only delayed reaction where you will see something on the blood bank bench in the workup?
Delayed hemolytic transfusion reaction
How do you prevent TA-GVHD?
use irradiated units
What is the most common antibody associated with Post Transfusion Purpura?
anti-HPA-1a
What patients tend to have problems with iron overload?
sickle cell, beta thalassemia, and blackfan diamond anemia
What are side effects of massive transfusions?
hypothermia
hyperkalemia
citrate toxicity
What antigens are warm autoantibodies directed toward?
High prevalence antigens
What antigens are cold autoantibodies directed toward?
I,i, H, I/H combo
What percent of AIHA are caused by warm autoantibodies?
70%
What antibody to warm autoantibodies usually mimic?
Anti-e
What reactivity would you expect in an antibody screen and panel for a cold auto patient?
Reactivity at IS that decreases at 37C and disappears at AHG phase. Reactivity stronger at RT and 4C
What reactivity would you expect in an antibody screen and panel for a warm auto patient?
Panagglutination at AHG phase, positive autocontrol
What reagents/tests can we use to remove a warm autoantibody from a patient's sample when it is interfering with testing?
Adsorption – Allo or Auto
With PCH (Paroxysmal Cold Hemoglobinuria), what antibody is involved and what are characteristics of it?
Anti-P, biphasic hemolysin.
Attaches to complement at lower temperatures (extremities), then causes hemolysis as it moves to warmer temperatures (The
heart, core).
Donath Landsteiner test to confirm presence.
Which drug adsorption method is associated with a drug induced positive DAT?
Cephalosporin – causes changes to RBC membrane
Quinidine - most associated with the immune complex mechanism of drug→ induced positive DAT
What are signs/symptoms of Cold Hemagglutinin Disease?
Anemia, Acrocyanosis of the hands and feet, Raynaud's phenomena, Agglutination of red cells in capillaries.
What is the most important step in transfusion?
Accurately identify the donor unit and recipient
What is an acute transfusion reaction?
A reaction with signs or symptoms that occurs within 24 hours of a transfusion.
What is a delayed transfusion reaction?
A reaction with signs or symptoms presenting after 24 hours of
a transfusion.
What are the acute transfusion reactions?
Acute hemolytic
Transfusion associated sepsis
Febrile non-hemolytic
mild allergic
severe allergic
TACO
TRALI
What are the delayed transfusion reactions?
Delayed hemolytic
Transfusion associated graft vs. host
Post transfusion purpura
Iron overload
Acute hemolytic syptoms
• Fever, shock, hypotension
• Abdominal, flank, or back pain
• Pain at the transfusion site
• Feeling of impending doom
• Hemoglobinemia, Hemoglobinuria
• Renal failure
• Diffuse intravascular coagulopathy
Delayed hemolytic symptoms
• Slight fever
• Decreasing hemoglobin and hematocrit
• Platelet refractoriness with bleeding
Febrile Nonhemolytic symptoms
• Chills, nausea or vomiting
• Tachycardia
• Increase in BP
• Tachypnea
• Occasionally shaking chills is initial symptom
Mild Allergic symptoms
Redness, itching, hives (urticaria), fever (may or may not happen).
Severe allergic
Anaphylaxis – coughing, dyspnea, nausea, vomiting, hypotension, skin flushing, abdominal cramps, diarrhea, shock, loss of consciousness
Transfusion Associated Circulatory Overload (TACO) symptoms
• Respiratory distress (wheezing, coughing)
• Headache
• Chest tightness
• Hypertension - Jugular vein distention
• Elevated central venous pressure, elevated pulmonary wedge pressure
Transfusion Related Lung Injury (TRALI)
• Respiratory distress (wheezing, coughing)
• Headache
• Fever
• Chest tightness
• Hypotension
• Cyanosis
Post Transfusion Purpura (PTP) symptoms
Thrombocytopenia, hematuria, bleeding
What is the most common transfusion reaction type reported in the blood bank?
Febrile reactions
Which type of transfusion reactions happen immediately?
Acute
Can a brisk primary immune response, or an anamnestic immune response to a blood group antigen, both lead to a delayed transfusion reaction?
Yes
Can transfusion reactions other than febrile non hemolytic present with a fever?
Yes
Which transfusion reaction is the only one where no workup is required?
Mild allergic with hives only.
What is post transfusion purpura usually caused by?
anti-HPA-1a
Which transfusion reaction is non-immune?
TACO
What tests and checks need to be done when a transfusion reaction report and sample are received?
Clerical check, check for hemolysis, ABORh and DAT
If a patient has had multiple febrile non-hemolytic transfusion reactions, what kind of product do we recommend for future transfusions?
Leukocytes reduced
If a person has an anaphylactic transfusion reaction, what kind of
products do we recommend for future transfusion?
Washed products