Summary The optimal treatment strategy for adult Langerhans cell histiocytosis (LCH) remains unclear. Our previous study demonstrated the remarkable efficacy of combined methotrexate and cytarabine (Ara‐C) [MA] therapy in patients newly diagnosed with LCH, with a median follow‐up of 2 years. The present article reports long‐term follow‐up data spanning a median of 78 months (6.5 years) from a single‐arm, single‐centre, prospective phase 2 clinical trial (NCT 02389400) conducted between January 2014 and December 2020. Ninety‐five adults with newly diagnosed LCH exhibiting multisystem disease or multifocal single‐system involvement underwent MA therapy every 35 days for six cycles. Methotrexate (1 g/m 2 ) was administered by 24 h infusion on day 1 and AraC (0.1 g/m 2 ) by 24 h infusion for 5 days. The primary end‐point was event‐free survival (EFS). The median patient age was 32 years (range 18–65 years). The overall response rate was 89.5%. Seven patients in this cohort died, and 38 experienced disease reactivation. No degenerative central nervous system diseases were observed. The estimated 6‐year overall survival (OS) and EFS rates were 93.2% and 55.2% respectively. Multivariate analysis revealed that risk organ (RO) involvement at baseline (hazard ratio [HR] 6.135 [95% confidence interval (CI) 1.185–32.259]; p = 0.031) and age >40 years at diagnosis (HR 7.299 [95% CI 1.056–21.277]; p = 0.042) were associated with inferior OS. RO (HR 2.604 [95% CI 1.418–4.762]; p = 0.002) and skin (HR 2.232 [95% CI 1.171–4.255]; p = 0.015) involvement at baseline were poor prognostic factors for EFS. Regarding adverse events, four patients developed a second primary malignancy. In conclusion, the MA regimen was a valid and safe therapeutic approach for adult patients newly diagnosed with LCH.
Abstract Background. Anterior cervical discectomy and fusion with instrumentation (ACDFI) have been widely used in the treatment of the degenerative disease or traumatic instability of the cervical spine. This study aimed to investigate the clinical efficacy of the autologous vertebral-filled polyetheretherketone (PEEK) cage in anterior cervical discectomy and fusion with instrumentation (ACDFI). Material and Methods. The clinical data of 368 patients who received ACDFI from September 2015 to September 2020 were retrospectively analyzed. According to the material that filled PEEK cage during the surgery, the patients were divided into two groups, the autologous vertebrate group (n = 185) and the autologous iliac group (n = 183). The operative time, operative blood loss, postoperative complications in two groups were recorded and analyzed. The bone graft fusion and postoperative functional outcomes, including scores of modified Japanese Orthopedic Association score (mJOA), Neck Disability Index (NDI), and visual analog scale (VAS) were compared. Results. Patients were followed-up for 14.04 ± 0.98 months. At a 6-months follow-up, the rate of spinal fusion was 96.29% (178/185) in the vertebral group and 95.94% (176/183) in the iliac group, there was no statistically significant difference between the two groups ( P >0.05). The postoperative VAS, mJOA, and NDI scores were not significantly different between two groups during the follow-up ( P >0.05). The operative time and blood loss in the vertebral group were significantly less than that of the iliac group ( P <0.01). In the iliac group, all patients suffered pain in the iliac donor site, 65 patients suffered numbness, 12 patients had fat liquefaction in donor incision, while all patients in the vertebral group had no postoperative complications. Conclusion. The autologous vertebrae-filled PEEK cage can achieve the same clinical outcome as the autologous iliac, but the autologous vertebrae filled PEEK cage have the advantage of shorter operative time, less intraoperative blood loss, and postoperative complications.
Abstract Background Magnetic resonance imaging (MRI) is a widely used examination for knee injuries, however, the accuracy of MRI in classifying multiple ligament knee injuries (MLKIs) has not been reported. The purpose of this study was to investigate the value of MRI in diagnosing and classifying acute traumatic MLKIs. Methods The clinical data of 97 patients who were diagnosed with acute traumatic MLKIs and managed by multi-ligament reconstruction were retrospectively reviewed. Intraoperative findings were considered as the standard pattern of injured structures. The value of MRI in detecting injuries of ligaments and meniscus was evaluated by calculating the sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), positive likelihood ratio (PLR), negative likelihood ratio (NLR), and kappa coefficients analysis. The value of MRI in classifying MLKIs was evaluated by calculating the agreement between MRI and intraoperative findings. Results For detecting the specific injured structures in MLKIs, MRI had high sensitivity and moderate specificity in detecting cruciate ligament injuries, moderate sensitivity and specificity in detecting collateral ligament injuries, fair sensitivity and low specificity in the diagnosis of injuries to the meniscus. For classifying the MIKIs, MRI had a moderate agreement with intraoperative findings in classifying KD-Ⅴ (kappa value=0.57), poor agreement in the KD-Ⅰ (kappa value=0.39) and KD-ⅢM (kappa value=0.31), meaningless in the KD-Ⅱ and KD-ⅢL (kappa value <0). The overall agreement in classifying MLKIs was poor (kappa value =0.23). Conclusions MRI can be used for the early detection of MLKIs, however, the value of MRI in classifying MLKIs is limited, management of MLKIs should be based on intraoperative findings.
ObjectiveTo describe the clinical features and treatment responses of Rosai-Dorfman disease (RDD) and Erdheim-Chester disease (ECD) with kidney involvement.MethodsWe retrospectively analyzed RDD and ECD patients with kidney involvement from 2005 to 2023, evaluating kidney function changes, CT, and metabolic responses.ResultsThe study included 4 RDD and 44 ECD patients, with median ages of 58 and 51 years. RDD patients lacked kidney symptoms, while 27.3% of ECD patients exhibited lower extremity edema. The median estimated glomerular filtration rate (eGFR) was 80.5 (63-125) in RDD and 100 (22-133) ml/min/1.73 m2 in ECD. All RDD patients had renal masses; 68.2% of ECD cases showed perirenal infiltration. Two RDD patients received steroids (one post-nephrectomy), and two received lenalidomide and dexamethasone. One RDD patient (25%) showed eGFR improvement, and three (75%) had CT and PET-CT responses. Of 34 ECD patients, 26 were treated with interferon-alpha (IFN-α), 5 with BRAF inhibitor, and 3 with cytarabine. The rates of eGFR improvement, CT response, and PET-CT response were 14.7%, 5.9%, and 52.9%. Median follow-up was 27.0 months for RDD and 53.0 months for ECD. The 5-year overall survival was 66.7% for RDD and 81.8% for ECD. The median progression-free survival was 18.3 months for RDD and 59.4 months for ECD.ConclusionWe detailed kidney involvement characteristics and treatment responses in RDD and ECD, highlighting the typical renal mass in RDD and perirenal soft tissue in ECD. ECD patients may experience irreversible kidney dysfunction, indicating the critical need for early diagnosis and timely treatment.
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