Hematology Oncology Board

Review Anas Sawas

Blood Transfusion Reactions

Febrile Non-Hemolytic Transfusion Reaction (FNHTR)

Simple Allergic (Urticarial) Reaction Acute Hemolytic Transfusion Reaction (AHTR) Transfusion Associated Sepsis Severe Allergic (Anaphylactic) Reaction Transfusion Related Acute Lung Injury (TRALI) Transfusion-Associated Circulatory Overload (TACO)

Febrile Non-Hemolytic Transfusion Reaction (FNHTR)

Patient receiving blood transfusion develops chills or fever (<1 C increase)

Occurs during or up to 4 hours after

Incidence: 1:500

Pathophysiology: Cytokine mediated

Treatment:

Stop transfusion while continuing to give patient normal saline

Acetaminophen Resume transfusion if patient symptoms subside while

observing patient for 15-30 min

Simple Allergic (Urticarial) Reaction

Occurs during or a few hours after Incidence: 1:3 – 1:300 ONLY transfusion reaction where if mild you can

continue transfusion Pathophysiology: antigen-antibody interaction

Treatment: Diphenhydramine

Acute Hemolytic Transfusion Reaction (AHTR) Life-threatening reaction caused by acute intravascular

hemolysis of transfused RBCs can lead to DIC/ARF/ Shock

Incidence: 1:38,000 – 1:70,000 and Mortality: 1:30

Usually due to ABO incompatibility often caused by a clerical error

Symptoms: fever, chills, flank pain, and oozing from intravenous sites

Diagnosis: Pink Plasma , Coombs Test

Treatment:

STOP Transfusion Aggressive hydration

Notify Blood Bank

Send labs: Chem, Hg/Hct, Blood Type and screen, Coombs test

Transfusion Associated Sepsis

Occur in first hour of transfusion and is Rare

Caused by transfusion of a product that contains a microorganism and endotoxin

Symptoms: fever, chills, and hypotension.

Treatment:

STOP transfusion Notify blood bank

Culture blood (recipient and donor)

Broad spectrum antibiotics

Fluids and Pressors if needed

Severe Allergic (Anaphylactic) Reaction Occur at the start of the transfusion and up to 4 hrs following

the transfusion

Incidence: 1:20000- 1:50000

Presentation: angioedema, wheezing, respiratory distress and/or hypotension

Treatment:

STOP Transfusion

Supportive Care and Airway management

Epinephrine

Transfusion Related Acute Lung Injury (TRALI) Life-threatening emergency

Occur during or up to 6 hours following transfusion

Incidence: 1:5,000-1:150,000

Pathophysiology: HLA antibodies react with antigens on recipient granulocytes. Triggers an inflammatory response in the pulmonary vasculature

Presentation: pulmonary edema, tachypnea, tachycardia – hypotension, frothy pink sputum and/or fever

Treatment: Stop transfusion

Supportive (monitor, airway, hemodynamics) NO LASIX

Transfusion-Associated Circulatory Overload (TACO)

Occur few hours following transfusion

Pathophysiology : pulmonary edema 2/2 to volume overload and more common in patients with cardio vascular disease.

Presentation: pulmonary edema, tachypnea, tachycardia, Hypertension

Treatment:

STOP Transfusion Supportive (monitor, airway, hemodynamics)

Lasix

What is the appropriate intervention for itching associated with a blood transfusion?

A. AcetaminophenB. Acute hemolytic workupC. DiphenhydramineD. Observation

What is the appropriate intervention for itching associated with a blood transfusion?

A. AcetaminophenB. Acute hemolytic workupC. DiphenhydramineD. Observation

A 56-year-old man presents with an upper GI bleed from ulcer and a HG of 8. A transfusion of PRBC is started. 60 min later, pt has increased shortness of breath and his oxygen saturation drops to 91% on room air chest X-ray is shown. What management is indicated?

A. Ceftriaxone and Vancomycin B. Hydrocorisone and

diphenhydramine C. Intubate and Stop Transfusion D. Slow the rate of infusion

A 56-year-old man presents with an upper GI bleed from ulcer and a HG of 8. A transfusion of PRBC is started. 60 min later, pt has increased shortness of breath and his oxygen saturation drops to 91% on room air chest X-ray is shown. What management is indicated?

A. Ceftriaxone and Vancomycin B. Hydrocorisone and

diphenhydramine C. Intubate and Stop Transfusion D. Slow the rate of infusion

Which of the following viruses represents the most common infection transmitted in blood transfusion?

A. Hepatitis AB. Hepatitis BC. Hepatitis CD. HIV

Which of the following viruses represents the most common infection transmitted in blood transfusion?

A. Hepatitis AB. Hepatitis BC. Hepatitis CD. HIV

The risk of hepatitis B transmission is 1 in 200,000 – 500,000

Coagulopathy

Hemophilia Hemophilia A : Factor VIII deficient Hemophilia B : Factor IX deficient

Von Willebrand Disease

Medication Complications

Hemophilia

X-linked recessive mutation Incidence: Hemophilia A 1:5000 male live births,

Hemophilia B 1:30000 Presentations: bleeding and bruising easily Diagnosis: factor VIII and IX activity levels and aPTT Treatment:

Factor VIII or IX replacement: wt x 0.5 x desired activity %

FFP ( 1 u of Factor VIII per 1 ml)

Cryoprecipitate ( 100 u of Factor VIII per bag)

DDAVP

Von Willebrand Disease Autosomal Dominant, most common bleeding disorder

Function: involved in platelet adhesion to collagen, platelet aggregation and protects Factor VIII

Pathophysiology: Attaches to platelets by glycoprotein Ib receptor and connects subendothelium with platelets. Protects FVIII and delivers FVIII to the site of injury

Presentations: bleeding, epistaxis, hemarthroses, hematuria.

Diagnosis: Increased bleeding time and decreased vWF activity

Treatment:

DDAVP

Non-recombinant Factor VIII

Cryoprecipitate

Anti-Fibirolytics: Amicar and TXA

Estrogen OCP

Heparin

Pathophysiology: activates ATIII and inactivates Xa and Thrombin. Prevents fibrin clot

Monitor activity by aPTT

Antidote: Protamine Sulfate Transfuse PRBCs or PLT if needed

Heparin Induced Thrombocytopenia

Patient on heparin develops > 50 % drop in PLTs Pathophysiology: immune mediated destruction of

platelets Diagnosis: when suspected send HIT assay,

calculate 4 Ts score ( Timing, Thrombocytopenia, Thrombosis or other causes for thrombocytopenia )

Treatment : STOP Heparin

Give non-heparin anticoagulant

Warfarin

Pathophysiology: Vit K antagonist affecting factors II, VII, IX, X. Protein C and S.

Monitor activity by INR Reversal:

FFPVit K PCC

Reversal for Newer Agents

Plavix Platelets

Pradaxa FFP, Cryoprecipitate (Factor VIII

and vWF) , PCC (Factors II, VII, IX and X) and dialysis. Antidote on the way

Apixaban, Rivaroxaban (Xalerto) FFP, Cryo, PCC

A 6-year-old boy is brought in by his father 1 hour after sustaining a head injury. He was riding his bicycle down a hill and fell off after it struck a branch; he was not wearing a helmet. Medical history is significant for hemophilia A. Which of the following is the first step in management? A. Blood transfusion using 0-negative whole

blood B. CT C. Factor VIII therapy to 100% activity D. Factor IX therapy to 50% activity

A 6-year-old boy is brought in by his father 1 hour after sustaining a head injury. He was riding his bicycle down a hill and fell off after it struck a branch; he was not wearing a helmet. Medical history is significant for hemophilia A. Which of the following is the first step in management? A. Blood transfusion using 0-negative whole

blood B. CT C. Factor VIII therapy to 100% activity D. Factor IX therapy to 50% activity

In which of the following conditions is the patient most likely to have anormal platelet count?

A. Disseminated intravascular coagulation B. Excessive hemorrhage C. Hemolytic uremic syndrome D. von Willebrand disease

In which of the following conditions is the patient most likely to have anormal platelet count?

A. Disseminated intravascular coagulation B. Excessive hemorrhage C. Hemolytic uremic syndrome D. von Willebrand disease

A 43-year-old woman with a history of von Willebrand disease presents with epistaxis. Vital signs are unremarkable and exam reveals oozing from the right nares despite pressure. Which treatment is indicated in this patient’s management?

A. Cryoprecipitate B. Desmopressin C. Factor VIII concentrate D. FFP

A 43-year-old woman with a history of von Willebrand disease presents with epistaxis. Vital signs are unremarkable and exam reveals oozing from the right nares despite pressure. Which treatment is indicated in this patient’s management?

A. Cryoprecipitate B. Desmopressin C. Factor VIII concentrate D. FFP

A 4-year-old boy with hemophilia B presents to the ED after he fell from the monkey bars and struck his head on the ground. On exam, he has a large occipital hematoma and a GCS of 14. Which of the following should be administered? A. Cryoprecipitate B. Factor IX concentrate C. Factor VIII concentrate D. Recombinant human factor VIIa

A 4-year-old boy with hemophilia B presents to the ED after he fell from the monkey bars and struck his head on the ground. On exam, he has a large occipital hematoma and a GCS of 14. Which of the following should be administered? A. Cryoprecipitate B. Factor IX concentrate C. Factor VIII concentrate D. Recombinant human factor VIIa

A 44-year-old man is transferred from a nursing facility for thrombocytopenia. He was transferred 6 days ago for pulmonary embolism and is on low-molecular weight heparin. His platelets have fallen from 352 to 100. There is no active bleeding at this time. Which of the following is the most likely management indicated? A. Continue current therapy B. Order heparin-induced platelet aggregation

studies and continue therapy while awaiting results C. Stop heparin and start Fondaparinux D. Transfuse platelets

A 44-year-old man is transferred from a nursing facility for thrombocytopenia. He was transferred 6 days ago for pulmonary embolism and is on low-molecular weight heparin. His platelets have fallen from 352 to 100. There is no active bleeding at this time. Which of the following is the most likely management indicated? A. Continue current therapy B. Order heparin-induced platelet aggregation studies

and continue therapy while awaiting results C. Stop heparin and start fondaparinux D. Transfuse platelets