Management of Osteomyelitis in Sickle Cell Disease: Review Article

Abstract

Sickle cell disease (SCD) is an autosomal recessive disorder that is characterized by abnormal “sickle-shaped” erythrocytes. Because of their shape, these erythrocytes are more likely to become trapped in small slow-flowing vessels, leading to vaso-occlusion. Because this commonly happens in the bones, patients with SCD are at an increased risk for orthopaedic manifestations such as osteomyelitis, septic joint, or osteonecrosis. Osteomyelitis is a serious and potentially disabling condition but can be difficult to differentiate from benign conditions of SCD, such as vaso-occlusive crisis. Diagnosis of osteomyelitis requires careful evaluation of the clinical presentation, laboratory testing, and imaging. Treatment of osteomyelitis in patients with SCD may be medical or surgical, but considerations in antibiotic selection and management preoperatively and postoperatively must be taken to ensure optimal outcomes.

Salmonella Osteomyelitis in Two Immunocompetent Children

“…The "gold standard" to discriminate osteomyelitis from a vaso-occlusive crisis is a positive culture, however blood cultures can remain negative. MRI can be a good adjunct to distinguish as it is highly sensitive (97%) for diagnosing osteomyelitis as well as highly specific (92%) ruling it out [3,7]. We recommend obtaining an MRI after 3 days of onset of complaints and performing a biopsy when blood cultures remain negative, and MRI does not distinguish between the two or is not available.…”

“…Salmonella spp. is known to be able to cause osteomyelitis, especially in immunocompromised children [2] and children with sickle cell disease [3]. Osteomyelitis with a Salmonella species in immunocompetent children however is rare [4,5].…”

Murine bone marrow mesenchymal stromal cells have reduced hematopoietic maintenance ability in sickle cell disease

“…36 A decrease in osteogenic differentiation potential (Figure 1D), along with a decrease in expression of the osteogenic genes Sp7 and Col1a2, was observed with SS MSCs, also resembling MSCs from patients with b-thalassemia 36,37 and consistent with increased risk for developing orthopedic complications in SCD. 38 We next analyzed the transcriptomes of bone marrow MSCs from SCD (SS) mice and control mice (SA) by RNA sequencing and found significant differences among the gene expression patterns in SCD MSCs compared with MSCs from controls (Figure 2A-C; supplemental Table 1). Bone marrow MSCs have been shown to express numerous key genes, which play a role in maintaining HSCs within the bone marrow in steady state.…”

Plain film x-rays in the diagnosis of sickle cell limb pain in children

“…However, blood cultures are often negative despite OM, and bone biopsy is invasive and therefore cannot be used routinely. Radiological evidence can support a diagnosis of OM but plain film x-rays of the limb have a poor sensitivity and specificity for these 5 . Magnetic resonance imaging (MRI) or ultrasound are more likely to guide management, although these can also cause diagnostic uncertainty 6 .…”