A 5-year-old girl is brought to the emergency department due to decreased energy and alertness. The patient has sickle cell disease and has been fatigued since yesterday. She has no fever or cough. She takes penicillin and folic acid. Temperature is 37.1 C (98.8 F), blood pressure is 80/40 mm Hg, pulse is 190/min, and respirations are 30/min. Pulse oximetry is 97% on room air. The patient is lethargic but can follow simple commands. There is faint scleral icterus, and mucous membranes are dry. Cardiac examination shows tachycardia with a 2/6 systolic murmur at the left sternal border. The lungs are clear to auscultation. Abdominal examination shows tenderness, and the spleen is palpated 6 cm below the costal margin. Strength is 4/5 in all extremities, and sensations are intact. Reflexes are equal bilaterally, and cerebellar examination is unremarkable. The results of a complete blood count are as follows: In addition to intravenous fluids, which of the following is the best next step in management of this patient's condition?

Question

A 5-year-old girl is brought to the emergency department due to decreased energy and alertness.  The patient has sickle cell disease and has been fatigued since yesterday.  She has no fever or cough.  She takes penicillin and folic acid.  Temperature is 37.1 C (98.8 F), blood pressure is 80/40 mm Hg, pulse is 190/min, and respirations are 30/min.  Pulse oximetry is 97% on room air.  The patient is lethargic but can follow simple commands.  There is faint scleral icterus, and mucous membranes are dry.  Cardiac examination shows tachycardia with a 2/6 systolic murmur at the left sternal border.  The lungs are clear to auscultation.  Abdominal examination shows tenderness, and the spleen is palpated 6 cm below the costal margin.  Strength is 4/5 in all extremities, and sensations are intact.  Reflexes are equal bilaterally, and cerebellar examination is unremarkable.  The results of a complete blood count are as follows:  In addition to intravenous fluids, which of the following is the best next step in management of this patient's condition?

Quizplus · Accepted Answer

11ec25c8_d5e7_fc3f_bc81_5f9ae7c15573_MD0003_00 This patient with sickle cell disease has relatively acute-onset fatigue and lethargy, along with splenomegaly, abdominal pain, and signs of anemia (low hemoglobin, tachycardia, flow murmur). These findings are concerning for acute splenic sequestration crisis, a life-threatening complication of sickle cell disease in which red blood cells (RBCs) become entrapped within the spleen. Splenic sequestration presents in early childhood, before the spleen becomes fibrotic from repeated infarction. A large volume of blood pools in the spleen, causing splenomegaly (often tender) and an acute drop in hemoglobin. The presentation includes fatigue, lethargy, and pallor, which can quickly progress to hypotension and hypovolemic shock (as in this patient). Because cardiac output increases to compensate for decreased RBC oxygen-carrying capacity, tachycardia and a systolic flow murmur are common. In addition to anemia, laboratory findings include thrombocytopenia (due to splenic trapping of platelets) and reticulocytosis (ie, bone marrow response to decreased RBCs). Initial management of acute splenic sequestration crisis is resuscitation with intravenous isotonic fluids. A packed RBC transfusion is also indicated to help restore circulatory volume and correct the anemia. However, the transfusion should be administered cautiously (eg, half of normal volume) because RBCs in the spleen may return to circulation, resulting in a rapid increase in hemoglobin and complications such as hyperviscosity syndrome. (Choice A) Norepinephrine is a vasoconstrictor that increases systemic vascular resistance in patients with septic shock, which is unlikely in this afebrile patient. Volume resuscitation is the mainstay of treatment for hypovolemic shock. (Choice C) The recurrence rate of splenic sequestration is high and may warrant splenectomy as definitive management. However, splenectomy is not typically performed in the acute setting and is often unnecessary due to the natural history of splenic autoinfarction in patients with sickle cell disease. (Choice D) Tranexamic acid promotes clotting in a patient with anemia due to significant blood loss (eg, traumatic hemorrhage). In contrast, this patient's anemia is secondary to blood sequestration in the spleen. (Choice E) Whole blood (ie, RBCs, platelets, plasma) transfusion is rarely required except in cases of massive blood loss. Although this patient has thrombocytopenia, she is not actively bleeding or at risk for spontaneous bleeding and therefore does not require blood components other than RBCs. Educational objective: Splenic sequestration crisis is a life-threatening complication of sickle cell disease in which red blood cells become entrapped within the spleen. Patients have anemia, splenomegaly, and hypovolemic shock. Treatment focuses on the restoration of circulatory volume with intravenous fluids and packed red blood cell transfusion. References: Splenic sequestration crisis. (http://www.ncbi.nlm.nih.gov/pubmed/31985957)

A 5-Year-Old Girl Is Brought to the Emergency Department Due