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Purpose: The aim of this study was to describe the clinical features of osteonecrosis (ON) in sickle cell disease (SCD) patients in Bahia, a Northeast state with the highest prevalence of the disease in Brazil., Methods: Between 2006 and 2017, 283 cases of osteonecrosis in SCD patients were enrolled to analyse the age at diagnosis, genotype, gender, pain, distribution of the lesions and disease staging. MRI and radiograph were obtained at the participation., Results: Of the 283 SCD cases, 120 (42.4%) were haemoglobin SS genotype while 163 (57.6%) were SC genotype. Two hundred and forty-six cases were bilateral and 37 were unilateral, with an average age at diagnosis of 33.7 (range 10-67) years. The most frequent identified ON site not only was the hip (74.6%), but also affected shoulder, knee and ankle. Most cases presented at early stage I (172, 60.8%) disease. No significant differences on the features of osteonecrosis were identified between haemoglobin SS and haemoglobin SC cases., Conclusions: Given the relatively high prevalence of bilateral osteonecrosis at early stages, painful symptoms and rather late age at diagnosis, SCD patients should have radiological examination of their joints more often in order to prevent severe functional disability and increase patient's life quality.

Background: The purpose of our study was to evaluate safety, feasibility and clinical results of bone marrow mononuclear cell (BMC) implantation for early-stage osteonecrosis of the knee (OK) secondary to sickle cell disease., Methods: Thirty-three SCD patients (45 knees) with OK treated with BMC implantation in the osteonecrotic lesion were clinically and functionally evaluated through the American Knee Society Clinical Score (KSS), Knee Functional Score (KFS) and Numeric Rating Scale (NRS) pain score. MRI and radiographic examinations of the knee were assessed during a period of five years after intervention., Results: No complications or serious adverse event were associated with BMC implantation. From preoperative assessment to the latest follow-up, there was a significant (p < 0.001) improvement of clinical KSS (64.3 ± 9.7, range: 45-80 and 2.2 ± 4.1, range: 84-100, respectively), KFS (44.5 ± 8.0, range: 30-55 and 91.6 ± 5.8, range: 80-100, respectively) and reduction of NRS pain score (6.7 ± 1.2, range: 4-9 and 3.4 ± 1.0, range: 2-5, respectively). In total, 87% of patients (29/33) consistently experienced improvements in joint function and activity level as compared to preoperative score. No patient had additional surgery following BMC implantation. Radiographic assessment showed joint preservation and no progression to subchondral collapse at most recent follow-up., Conclusions: The technique of BMC implantation is a promising, relatively simple and safe procedure for OK in SCD patients. Larger and long-term controlled trials are needed to support its clinical effectiveness., Trial Registration: ClinicalTrials.gov NCT02448121 . Retrospectively registered 19 May 2015.

Background: Little is known about the rate of, and factors affecting, progression of talar osteonecrosis related to sickle cell disease. Adult patients with sickle cell disease who presented with hip osteonecrosis were evaluated for talar osteonecrosis with radiographs and magnetic resonance imaging (MRI). Forty-five of them (75 tali) were diagnosed with talar osteonecrosis, and this group was evaluated for factors influencing the progression of the disease., Methods: Forty-five patients with sickle cell disease and osteonecrosis of the talus were identified with radiographs and MRI between 1985 and 1995. Seven of these patients were homozygous for hemoglobin S (S/S genotype), 26 had hemoglobin S/hemoglobin C, and 12 had hemoglobin S/beta-thalassemia. The talar osteonecrosis was graded with radiographs and MRI. The patients were followed with clinical examination and radiographs every 6 months until talar collapse and every year after the collapse., Results: The osteonecrosis was unilateral in 15 patients and bilateral in 30 at the time of the initial examination. Forty-five ankles were asymptomatic and 30 were symptomatic at the initial evaluation. MRI performed at the time of the most recent follow-up, and compared with MRI performed at diagnosis, did not show partial or total regression of the osteonecrosis in any of the patients, even those with asymptomatic stage-I osteonecrosis. At the time of the most recent follow-up (mean, 20 years; range, 15 to 25 years), pain and collapse had developed in all except 12 ankles. The stage of the osteonecrosis at the initial visit, pain, the genotype of the sickle cell disease, and the extent and location of the lesion in the talus were risk factors for progression of the disease., Conclusions: In the majority of the patients with sickle cell disease, osteonecrosis of the talus should be expected to show relevant clinical and radiographic evidence of progression over a long period., Level of Evidence: Prospective Level II. See Instructions for Authors for a complete description of levels of evidence., (Copyright © 2016 by The Journal of Bone and Joint Surgery, Incorporated.)

Introduction: Stem cell therapy with bone marrow-derived mononuclear cells (BMMCs) is an option for improving joint function in osteonecrosis of the femoral head (ONFH). Bone marrow-derived mesenchymal stromal cell (MSC) numbers and their osteogenic differentiation are decreased in patients with ONFH. However, whether this decrease also extends to the early stages of ONFH in sickle cell disease (SCD) is still unclear., Methods: We conducted a phase I/II, non-controlled study to determine efficacy and safety of BMMC implantation using a minimally invasive technique in SCD patients with ONFH. Eighty-nine patients were recruited and followed up for 60 months after surgery. Clinical and radiographic findings were assessed, and data were completed by in vitro analysis., Results: At the final follow-up (60 months) there was a significant improvement in clinical joint symptoms and pain relief as measured by the Harris Hip Score (P = 0.0005). In addition, after the BMMC implantation procedure, radiographic assessment showed disease stabilization and only 3.7 % of the treated patients did not achieve a satisfactory clinical result. The amount of fibroblast colony-forming units was 28.2 ± 13.9 per 1 million BMMCs after concentration. Flow cytometry analysis showed a significantly higher number of hematopoietic stem/endothelial progenitor cell markers in concentrated BMMCs when compared with bone marrow aspirate, indicating an enrichment of these cell types. Isolated MSCs from SCD patients with pre-collapse ONFH maintained the replicative capacity without significant loss of their specific biomolecular characteristics, multi-differentiation potential, and osteogenic differentiation activities. Cytokines and growth factors (interleukin-8, transforming growth factor-beta, stromal cell-derived factor-1alpha and vascular endothelial growth factor) that mediate endogenous bone regeneration were also produced by expanded MSCs from SCD patients., Conclusion: The autologous BMMC implantation with a minimally invasive technique resulted in significant pain relief and halted the progression of early stages of ONFH in SCD patients. MSCs from SCD patients display biological properties that may add to the efficiency of surgical treatment in ONFH. In summary, our results indicate that infusion of BMMCs enriched with stem/progenitor cells is a safe and effective treatment for the early stages of ONFH in SCD patients., Trial Registration: ClinicalTrials.gov NCT02448121; registered 15 May 2015.

Background: Septic arthritis is a known complication of sickle cell disease (SCD) in children, and the association with osteomyelitis and osteonecrosis has been described. However, it is unclear whether this association applies to adults., Questions/purposes: We therefore asked whether septic arthritis is a frequent complication in adults with SCD and whether it also is associated with osteomyelitis or osteonecrosis., Methods: We retrospectively reviewed the charts of 2000 consecutive adult patients diagnosed with SCD and recorded symptoms, select findings during physical examination, laboratory data, and select radiographic CT, and MRI observations., Results: Fifty-nine of the 2000 patients (3%) had septic arthritis, 56 of the 59 patients had hemoglobin SS. Thirty-six of the 59 infections (61%) were in the hip. The most frequent findings were pain, swelling, fever greater than 38.2 degrees C (71% of cases), a leukocyte count exceeding 15,000/mm(3) (range, 7900-32,300/mm(3)), a Westergren sedimentation rate greater than 24 mm/hour, and C-reactive protein exceeding 20 mg/L. Cultures were positive in 96% of the joint aspirates. Staphylococcus and Gram-negative infection predominated; no patients had Salmonella joint infections. Preexisting factors of bacterial arthritis included osteonecrosis (29 patients) and osteomyelitis (37 cases) in childhood. Diabetes, rheumatoid arthritis, glucocorticoids, and immunoparesis related to medical treatment by hydroxyurea were associated comorbidities. CT and MRI confirmed the diagnosis of associated osteonecrosis or osteomyelitis and allowed joint aspiration and detection of soft tissue abscess., Conclusions: The incidence of septic arthritis in adults with SCD is low, but often is associated with osteomyelitis or osteonecrosis., Level of Evidence: Level II, prognostic study. See Guidelines for Authors for a complete description of levels of evidence.