Family Medicine Resident Virginia Tech Carilion Clinic Roanoke, Virginia
Introduction: Avascular necrosis is a common complication in patients with sickle cell disease secondary to vaso-occlusion. Patients with sickle cell disease are considered a medical emergency when temperatures reach > 101F due to functional asplenia. Our case presented a unique clinical course when our patient spiked a 102-degree fever shortly after admission without a source of infection. Infection remains the highest risk for death in patients with sickle cell disease with acute chest syndrome being the most common cause of fever and death. Following an extensive workup with hematology and infectious disease, the fever was thought to be secondary to avascular necrosis and hemolysis leading to an acute systemic inflammatory response.
Case Study: A high school basketball player presented to the ED complaining of bilateral anterior hip pain, lumbar pain, left-sided superolateral hip pain, and painful swelling over his sacrum following a basketball game. Patient had a history of sickle cell hemoglobin-C disease. On exam, patient had reproduction of pain with provocative testing of the bilateral hips, sacrum, and lumbar spine. The sacrum had local tissue texture changes near the left PSIS with somatic dysfunction of the lumbar spine, sacrum, and pelvis. POCUS was completed over the sacrum without obvious source of pain. XRs of the hips and pelvis were completed without abnormality. Laboratory workup showed thrombocytopenia, reticulocytosis, elevated inflammatory markers, and elevated alkaline phosphatase. Workup also revealed elevated bilirubin and lactate dehydrogenase with decreased haptoglobin. MRI showed left greater than right femoral head avascular necrosis without collapse and infarcts in the left intra-trochanteric region, right sub-trochanteric region, left sacral ala, L5 vertebral body, and left iliac wing. Overnight, the patient became febrile. Repeat infectious evaluation was unchanged. Patient was started on broad spectrum antibiotics. A PET/CT scan was completed and showed scattered skeletal accumulations in the left hemipelvis, lumbar spine, several ribs, and bilateral scapula consistent with bony infarcts from sickle cell vaso-occlusive crisis. Patient’s fever was believed to be secondary to an acute phase reaction from diffuse bony infarcts.
Discussion: This represents a rare case when fever was secondary to inflammatory cascades from musculoskeletal injury and hemolysis rather than an infectious source as was initially suspected and subsequently ruled out – demonstrating the body’s ability to promote self-regulation and self-healing. We were unable to find similar case studies in patients with avascular necrosis. This case will add to a growing body of research on avascular necrosis and fevers in sickle cell disease.
