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First Aid Step 3 Hematology 5
Thrombophilia and Transfusion Rxns
28
Medical
Professional
12/25/2013

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Term
28 yo G2P1 at 28 wks comes or routine pregnancy apt complaint of unilateral ankle swelling. L is 2cm>R leg. US shows non-compressible L popliteal vein. What medication do you give?
Definition
LMWH until 24 hours prior to delivery.
Restart after delivery x 4 weeks
Warfarin is an option post-partum.
Term
What are some inherited causes of thrombophilia?
Definition
Factor V Leiden deficiency
Prothrombin 20210 mutation
protein C or S deficiency
Anti-thrombin III deficiency
Homocystinemia
Fibrinolysis defects
Term
What are some acquired causes of thrombophilia?
Definition
Prolonged rest
Immobilization
Smoking
OCP
Pregnancy
Nephrotic syndrome
Cancer
DIC
Lupus anticoagulant
Term
How do you evaluate thrombophilia?
Definition
Two weeks after anticoagulation..... look for primary causes.
Hx first thrombotic event before age 50
Recurrent thrombotic episodes
First degree relative with thromboembolic event before age 50
Term
What kind of screening is used with thrombophilia?
Definition
APC resistance (Activated protein C) suggests Factor V Leiden
Prothrombin mutation, antiphospholipid antibody
Plasma hommocysteine
Antithrombin deficiency
Protein C deficiency
Protein S deficiency
Term
What do you do if APC resistance is confirmed?
Definition
Check Factor V Leiden genotypic mutation assay.
Term
How do you treat homocystinemia?
Definition
Vitamin B12 and folate supplements
Term
Transfusion Reaction:
Major/Minor hemolytic reaction presentation?
Definition
Chills, fever, SOB, nausea, chest/flank pain, hypotension, flushing
Complications: ARF, DIC
Term
Transfusion Reaction:
Major/Minor hemolytic reaction tests?
Definition
Coombs'test: +
Agglutination of RBC on smear
Haptoglobin: Low
Hemoglobinuria: UA + hematuria wiht few RBCs on microscopy
Term
Transfusion Reaction:
Major/Minor hemolytic reaction management?
Definition
Stop transfusion
Maintain BP and urine output with IVF
Give furosemide if UOP <100ml/hr
Type and cross RBCs just transfused
Term
Transfusion Reaction:
Delayed hemolysis presentation?
Definition
Onset 4-14 days post-transfusion
Jaundice
Anemia
Hemoglobinuria
Fever
Term
Transfusion Reaction:
Delayed hemolysis Tests?
Definition
LDH: Increased
Hyperbilirubinemia: Unconjgated
Haptoglobin: Decreased
Term
Transfusion Reaction:
Delayed hemolysis Management?
Definition
Type and screen blood before future transfusions
Acetaminophen for fever
Term
Transfusion Reaction:
Febrile, nonhemolytic reaction presentation?
Definition
Onset within two hours post-transfusion
Fever
Rigors
Nausea
Vomiting
Chills
Term
Transfusion Reaction:
Febrile, nonhemolytic reaction Tests?
Definition
Rule out biochemical evidence of hemolysis
Term
Transfusion Reaction:
Febrile, nonhemolytic reaction management?
Definition
For future transfusions, use leukocyte-reduced RBCs
Avoid transfusion when febrile
Term
Transfusion Reaction:
Anaphylaxis presentation?
Definition
Increased risk with IgA deficiency
Sudden onset
flushing
HTN followed by Hypotension
Edema
Respiratory distress
Shock
Wheezing
Chest Pain
Term
Transfusion Reaction:
Anaphylaxis Tests?
Definition
None
Term
Transfusion Reaction:
Anaphylaxis Management?
Definition
IV epinephrine
Use saline-washed PRBCs in the future
Term
Transfusion Reaction:
Urticaria presentation?
Definition
Rash, pruritus
Term
Transfusion Reaction:
Urticaria tests?
Definition
None
Term
Transfusion Reaction:
Urticaria management?
Definition
Stop transfusion: monitor for anaphylaxis
Give diphenhydramine
Resume transfusion at a slower rate when symptoms resolve.
Term
Transfusion Reaction:
Transfusion related acute lung injury presentation?
Definition
Occurs 1-6 hours posttransfusion
Looks like ARDS
Acute respiratory distress, cyanosis, fever; gone in 24 hours
DDx: Fluid overload
Term
Transfusion Reaction:
Transfusion related acute lung injury tests?
Definition
CXR shows bilateral pulmonary infiltrates without CHF.
Term
Transfusion Reaction:
Transfusion related acute lung injury management?
Definition
Ventilation, diuretics, steroids
Term
Transfusion Reaction:
Bacterial infection presentation?
Definition
More likely with platelet transfusions
Fever
Hypotension; onset within four hours
Term
Transfusion Reaction:
Bacterial infection tests?
Definition
Blood culture transfusion product
Term
Transfusion Reaction:
Bacterial infection management?
Definition
Antibiotics
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