A 17-year-old girl is admitted to the hospital due to heavy menses and a hemoglobin of 5.2 g/dL. On admission, the patient is started on intravenous (IV) estrogen and receives 1 unit of packed red blood cell transfusion. She is then started on a second unit of blood. Five minutes later, the patient becomes anxious and short of breath. Temperature is 38.6 C (101.5 F), blood pressure is 104/66 mm Hg, pulse is 120/min, and respirations are 30/min. Pulse oximetry shows 98% on room air. The patient appears pale. The oropharynx is clear, and lung examination reveals clear breath sounds bilaterally. There is pain at the IV insertion site, and skin examination shows no rashes. The patient's urine appears dark. The transfusion is stopped. Which of the following is the best next step in management of this patient?

Question

A 17-year-old girl is admitted to the hospital due to heavy menses and a hemoglobin of 5.2 g/dL.  On admission, the patient is started on intravenous (IV) estrogen and receives 1 unit of packed red blood cell transfusion.  She is then started on a second unit of blood.  Five minutes later, the patient becomes anxious and short of breath.  Temperature is 38.6 C (101.5 F), blood pressure is 104/66 mm Hg, pulse is 120/min, and respirations are 30/min.  Pulse oximetry shows 98% on room air.  The patient appears pale.  The oropharynx is clear, and lung examination reveals clear breath sounds bilaterally.  There is pain at the IV insertion site, and skin examination shows no rashes.  The patient's urine appears dark.  The transfusion is stopped.  Which of the following is the best next step in management of this patient?

Quizplus · Accepted Answer

11ec25c8_da02_66e9_bc81_5546785e22db_MD0003_00 This patient has heavy menses leading to a critically low hemoglobin, for which she received intravenous estrogen (sometimes used for acute abnormal uterine bleeding) and packed red blood cell (pRBC) transfusions. She then developed dyspnea, fever, and hemoglobinuria a few minutes after receiving her second pRBC transfusion. This presentation is most consistent with an acute hemolytic transfusion reaction (AHTR), a rare but life-threatening reaction often caused by the transfusion of mismatched blood (ie, ABO incompatibility), secondary to clerical error. Pathogenesis involves the reaction of recipient antibodies with donor blood antigens, causing complement fixation and intravascular hemolysis. Within an hour after transfusion initiation, patients classically develop fever, chills, hemoglobinuria (ie, dark urine due to hemolysis), and flank pain. Pain at the IV insertion site and hypotension are also common. Patients may feel anxious or short of breath, but oxygen saturation and lung sounds are typically normal. Severe cases may progress to acute renal failure (eg, oliguria, anuria) or disseminated intravascular coagulation (eg, oozing from the IV insertion site). AHTR is a medical emergency. The first step in management is immediate cessation of the transfusion. Aggressive hydration with IV normal saline is indicated for hemodynamic stabilization and prevention of renal injury. In addition to hydration, supportive care measures (eg, supplemental oxygen, vasopressors) may be required (Choice E). Extensive workup, including laboratory evaluation to exclude other causes of hemolysis and attempts at identifying clerical errors (to ensure no other patients were affected), should be performed. (Choice A) Epinephrine is the treatment for anaphylactic transfusion reaction, which causes angioedema, hypotension, and respiratory distress (eg, wheezing) within minutes after transfusion initiation. In rare cases, anaphylactic reactions can occur with IV estrogen administration. However, fever and dark urine are not associated with anaphylaxis. (Choice B) Diuresis with IV furosemide is indicated for transfusion-associated circulatory overload, which presents with respiratory distress and tachycardia. Unlike this case, patients are typically hypoxic, hypertensive, and afebrile. (Choice D) Oral diphenhydramine is used for urticarial transfusion reaction, characterized by normal vital signs and the development of hives within 2-3 hours after transfusion initiation. This patient has no rash. Educational objective: Acute hemolytic transfusion reaction is a life-threatening condition in which hemolysis following transfusion of incompatible blood classically causes fever, flank pain, and hemoglobinuria. Hypotension, dyspnea, and disseminated intravascular coagulation may occur. Management includes immediate cessation of the transfusion and administration of intravenous fluids for hemodynamic stabilization and prevention of renal injury. References: Transfusion reactions. (http://www.ncbi.nlm.nih.gov/pubmed/29489247)

A 17-Year-Old Girl Is Admitted to the Hospital Due to Heavy