Sickle cell disease (SCD) is an autosomal recessive hemolytic disorder common in Brazil. Its complex pathophysiology includes anemia, chronic inflammation, autosplenectomy and vaso-occlusive crises. There is a risk of severe infections, particularly by encapsulated organisms, due to defects in opsonization. Osteomyelitis is a bone infection affecting up to 12% of patients with SCD. Bone infarctions increase susceptibility. The main etiologic agents of osteomyelitis in these patients are Staphylococcus aureus and Salmonella sp. A 2-year-old boy with SCD was admitted to a tertiary public hospital where he followed in the pediatric hematology clinic, with intense pallor, prostration and splenomegaly. He had had two days of diarrhea one week before these symptoms. About five days before admission, he complained of pain in the left lower limb, limping and then refusing to walk. He received dipyrone and an anti-inflammatory drug regularly. On admission, he was diagnosed with splenic sequestration and received IV fluids and red blood cell transfusion. He was transferred to the pediatric intensive care unit and empirically started on cefepime and vancomycin. Blood culture grew Salmonella sp susceptible to tested antimicrobials (identification and susceptibility by Vitek® 2 bioMérieux and MALDI-TOF). He maintained hip flexion due to pain. Ultrasound of the left hip and CT of the abdomen and pelvis showed iliac osteomyelitis and collections in the left iliopsoas muscle. He underwent surgical drainage, and Salmonella sp was isolated from both sites. He became afebrile with decreased pain and was transferred to the ward, with a planned 4–6-week course of antibiotic therapy. The regimen was de-escalated to ceftriaxone, the drug of choice for pediatric osteomyelitis caused by Salmonella sp. Diagnosing osteomyelitis in SCD patients is challenging because of the difficulty in distinguishing bone infection from vaso-occlusive crisis. Both conditions may present with bone pain, fever, elevated inflammatory markers and leukocytosis. Imaging tests may also be nonspecific. Positive blood cultures and cultures from bone fragments or collections help confirm the diagnosis. Rapid initiation of treatment that covers Salmonella sp is crucial for prognosis.
Almeida et al. (2026) studied this question.