1. Hematology Oncology Board Review Anas Sawas

2. 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)

3. 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

4. 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

5. 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

6. 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

7. 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

8. 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

9. 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

10. What is the appropriate intervention for itching associated with a blood transfusion?  A. Acetaminophen  B. Acute hemolytic workup  C. Diphenhydramine  D. Observation

11. What is the appropriate intervention for itching associated with a blood transfusion?  A. Acetaminophen  B. Acute hemolytic workup  C. Diphenhydramine  D. Observation

12. 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

13. 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

14. Which of the following viruses represents the most common infection transmitted in blood transfusion?  A. Hepatitis A  B. Hepatitis B  C. Hepatitis C  D. HIV

15. Which of the following viruses represents the most common infection transmitted in blood transfusion?  A. Hepatitis A  B. Hepatitis B  C. Hepatitis C  D. HIV The risk of hepatitis B transmission is 1 in 200,000 – 500,000

17. Coagulopathy  Hemophilia  Hemophilia A : Factor VIII deficient  Hemophilia B : Factor IX deficient  Von Willebrand Disease  Medication Complications

19. 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

20. 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

21. 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

22. 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

23. Warfarin  Pathophysiology: Vit K antagonist affecting factors II, VII, IX, X. Protein C and S.  Monitor activity by INR  Reversal:  FFP  Vit K  PCC

24. 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

25. 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

26. 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

27. In which of the following conditions is the patient most likely to have a normal platelet count?  A. Disseminated intravascular coagulation  B. Excessive hemorrhage  C. Hemolytic uremic syndrome  D. von Willebrand disease

28. In which of the following conditions is the patient most likely to have a normal platelet count?  A. Disseminated intravascular coagulation  B. Excessive hemorrhage  C. Hemolytic uremic syndrome  D. von Willebrand disease

29. 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

30. 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

31. 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

32. 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

33. 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

34. 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