An Arresting Sickle Cell Disease Complication

Abstract

Editor’s note: Each month, this department features a discussion of an unusual diagnosis in genetics, radiology, or dermatology. A description and images are presented, followed by the diagnosis and an explanation of how the diagnosis was determined. As always, your comments are welcome via email at Pediatrics@Healio.com. A16-year-old female with known homozygous sickle cell anemia presented to the emergency department (ED) with fever, cough, and pain. Five days prior to presentation she was in her normal state of health when she developed a non-productive cough. One day prior to admission she developed pain consistent with prior vaso-occlusive crises in her legs, back, and abdomen. Her medical history was significant for acute chest syndrome and osteomyelitis. On examination she was febrile and mildly tachycardic with normal blood pressure, respiratory rate, and oxygen saturation. Her physical examination was significant for decreased breath sounds and crackles at her right lung base. No hepatosplenomegaly or point tenderness was appreciated on her abdominal exam. A chest X-ray done in the ED showed a right lower-lobe opacity. Her hemoglobin was found to be 6.6 g/dL, which was slightly below her baseline of 7 g/dL to 8 g/dL. Complete metabolic panel done at the time was unremarkable. She was given intravenous fluids, antibiotics, and pain medication and admitted to the hematology service for further management. Hospital Day 1: The patient was started on continuous morphine with patient-controlled analgesia for pain, and antibiotics were continued. A respiratory viral panel sent from the ED was positive for parainfluenza. Repeat liver function tests (LFTs) were done secondary to patient reports of right upper quadrant pain and were again found to be unremarkable. Hospital Day 2: A routine morning complete blood count showed marked elevation in the patient’s white blood count to 62,000. Her hemoglobin dropped to 4.3 g/dL and her reticulocyte count was 4.3% (reference range, 0.5%-1.5%). Due to concern for sepsis, blood cultures were redrawn and antibiotic coverage was expanded. Packed red blood cells (pRBCs) were ordered, but matched units were not immediately available due to the patient’s history of multiple antibodies. The patient became tachycardic and hypotensive and was transferred to the pediatric intensive care unit. The patient stabilized after receiving a simple blood transfusion, and an exchange transfusion was planned for the following morning when a sufficient quantity of cross-matched blood would be available. Hospital Day 3: Prior to exchange transfusion, the patient again became hypotensive and repeat hemoglobin was 3 g/dL with 3.1% retic count. The one available unit of matched pRBCs was given stat. She was electively intubated secondary to worsening acute chest