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Clinical Kidney Journal May 2024This study aimed to observe the efficacy and safety of tacrolimus in the treatment of refractory immunoglobulin A vasculitis nephritis (IgAVN).
BACKGROUND
This study aimed to observe the efficacy and safety of tacrolimus in the treatment of refractory immunoglobulin A vasculitis nephritis (IgAVN).
METHODS
Sixteen patients with IgAVN who had been previously treated with cyclophosphamide shock therapy at least five times, some of whom had also received mycophenolate but still had persistent proteinuria, were enrolled. The clinical and pathological data were collected and analysed.
RESULTS
The average (mean ± standard deviation) age at the initial assessment for the group of 16 patients was 10 ± 2.7 years. Finally, at the end of their respective follow-up time point, 6 of the 16 patients achieved complete remission (37.5%), 5 achieved partial remission (31.2%), and 5 had no remission (31.2%). A significant difference was found in the median proteinuria before and after a 6-month course of tacrolimus treatment [19.2 (11.2, 31.9) vs 7.8 (4.3, 13.9) mg/kg/day] (< .05). During the first 6 months of tacrolimus treatment, all patients' estimated glomerular filtration rate levels remained normal. The mean tacrolimus blood concentration was 6.0 ± 2.6 ng/mL. The median prednisone dosage was decreased from 10 mg/day to 5 mg/day, and prednisone was eventually stopped in three individuals. No drug-related adverse effects were observed during treatment.
CONCLUSIONS
Tacrolimus has demonstrated efficacy in increasing remission rates, significantly lowering urinary protein levels, and reducing steroid use in children with refractory IgAVN. Further research is required to investigate its optimal blood concentrations, long-term effects and renoprotective properties.
PubMed: 38742208
DOI: 10.1093/ckj/sfae115 -
Surgical Neurology International 2024Rathke's cleft cyst (RCC) is a benign lesion in the sellar and suprasellar compartments. Similarly, pituitary adenomas can present with cystic morphology, making it a...
Accurate preoperative diagnosis of a Rathke cleft cyst with the aid of a novel classification for sellar cystic lesions and a diagnostic algorithm decision: Tools for differentiating cystic sellar lesions with a representative case.
BACKGROUND
Rathke's cleft cyst (RCC) is a benign lesion in the sellar and suprasellar compartments. Similarly, pituitary adenomas can present with cystic morphology, making it a differential diagnosis when evaluating a patient with a cystic lesion in the sellar region. Surgical goals differ between RCCs and pituitary adenomas as the first can achieve remission of symptoms with cyst decompression in contrast to pituitary adenomas where complete resection would be the main goal. Imaging analysis alone may not be sufficient to define a preoperative surgical plan. The combination of imaging and conjoined use of validated tools may provide valuable insights to the clinician when defining a surgical approach.
CASE DESCRIPTION
We present a case of a 27-year-old male with a 3-month history of visual disturbances and headaches. Magnetic resonance imaging showed a cystic lesion in the sellar compartment with compression of nearby structures. The authors were able to accurately diagnose this sellar lesion as an RCC with the conjoined aid of two classifications proposed in the literature. Cyst evacuation was performed with relief of symptoms and improved visual outcomes at follow-up.
CONCLUSION
While cystic adenomas can require total resection for cure, RCCs can show marked improvement with partial resection and evacuation of its contents. An accurate preoperative diagnosis can lead the surgeon to opt for the best surgical approach.
PubMed: 38741985
DOI: 10.25259/SNI_59_2024 -
Folia Neuropathologica 2024MALT lymphoma of the dura is a very rare type of low-grade B-cell lymphoma. Little more than 100 cases have been reported in the literature to date. We report a... (Review)
Review
MALT lymphoma of the dura is a very rare type of low-grade B-cell lymphoma. Little more than 100 cases have been reported in the literature to date. We report a 43-year-old woman who was referred to hospital because of a series of three tonic-clonic seizures on the day of admission. Neurological examination revealed confusion and aphasia. Magnetic resonance imaging (MRI) showed a contrast-enhanced, broad-based lesion along the dura in the left parieto-occipital area. The suspicion of an en plaque meningioma was raised. The tumour invaded the brain parenchyma with visible extension into the brain sulci. There was a marked brain oedema surrounding the lesion and causing the midline shift 8 mm to the right. After stabilization of neurological condition (intravenous diuretics and steroids), the operation was performed. The diagnosis of dural MALT lymphoma was established. During the pathological examination, it was especially problematic to distinguish MALT lymphoma from follicular lymphoma, but the final diagnosis was MALT lymphoma. Surgical partial removal with additional R-CVP immunochemotherapy (rituximab, cyclophosphamide, vincristine and prednisone) resulted in complete remission. The follow-up period is 1 year. Our presented case of a MALT lymphoma highlights the fact that surgical partial removal with additional immunochemotherapy is an available option in these rare intracranial tumours.
Topics: Humans; Lymphoma, B-Cell, Marginal Zone; Female; Adult; Meningioma; Dura Mater; Meningeal Neoplasms; Diagnosis, Differential
PubMed: 38741437
DOI: 10.5114/fn.2024.135291 -
Cancer Medicine May 2024Consolidation therapy improves the duration of response among patients with primary central nervous system lymphoma (PCNSL). Lenalidomide maintenance has shown...
BACKGROUND
Consolidation therapy improves the duration of response among patients with primary central nervous system lymphoma (PCNSL). Lenalidomide maintenance has shown encouraging results in older patients with PCNSL. Herein, we performed a retrospective, single-center analysis to evaluate the effect of lenalidomide maintenance on the duration of response in patients with newly-diagnosed PCNSL.
METHODS
Sixty-nine adult patients with PCNSL who achieved complete remission or partial remission (PR) after induction therapy were enrolled. The median age of patients was 58.0 years. The maintenance group (n = 35) received oral lenalidomide (25 mg/day) for 21 days, every 28 days for 24 months; the observation group did not undergo any further treatment.
RESULTS
After a median follow-up of 32.6 months, the maintenance group experienced fewer relapse events. However, the median progression-free survival (PFS) was similar between groups (36.1 vs. 30.6 months; hazard ratio, 0.78; 95% confidence interval, 0.446). Lenalidomide maintenance significantly improved PFS and overall survival (OS) only among patients who experienced PR after induction. The median duration of lenalidomide maintenance was 18 months; lenalidomide was well tolerated and minimally impacted the quality of life.
CONCLUSIONS
The present study was the first to evaluate lenalidomide maintenance as a frontline treatment among patients with PCNSL, PFS and OS did not improve, although the safety profile was satisfactory.
Topics: Humans; Lenalidomide; Female; Male; Middle Aged; Retrospective Studies; Central Nervous System Neoplasms; Aged; Methotrexate; Maintenance Chemotherapy; Adult; Lymphoma; Progression-Free Survival; Treatment Outcome; Antineoplastic Combined Chemotherapy Protocols
PubMed: 38738459
DOI: 10.1002/cam4.7193 -
Cureus May 2024Concurrent malignancy and IgA nephropathy are rare. Despite the lack of solid experimental evidence, there are theoretical hypotheses of pathophysiology for the...
Concurrent malignancy and IgA nephropathy are rare. Despite the lack of solid experimental evidence, there are theoretical hypotheses of pathophysiology for the development of glomerular damage in cancer patients, like aberrant immune activities. Here, we describe a nine-year-old child who was admitted due to nephrotic syndrome. Abdominal imaging examination accidentally revealed a retroperitoneal tumor, and surgical resection was performed with a pathological diagnosis of neuroblastoma. However, complete removal of the tumor had no impact on the clinical manifestation of nephrotic syndrome, like proteinuria. The use of corticosteroids alone only led to a partial resolution of proteinuria, and resistance developed after one month of treatment. A further kidney biopsy was performed, which suggested IgA nephropathy. Clinical remission of IgA nephropathy was achieved after standard combination treatment of corticosteroids and mycophenolate mofetil for 10 months. This study represented the first case report of neuroblastoma associated with IgA nephropathy. We postulated that IgA nephropathy pathogenesis might be associated with neuroblastoma, though a coincidence of these two conditions cannot be fully excluded. Standard treatment for IgA nephropathy is applicable for patients with concomitant cancer.
PubMed: 38736768
DOI: 10.7759/cureus.60089 -
Oncology Letters Jun 2024Diffuse large B-cell lymphoma (DLBCL) is a heterogeneous disease with varying characteristics, in terms of genomic variation, cell morphology and clinical presentation....
Diffuse large B-cell lymphoma (DLBCL) is a heterogeneous disease with varying characteristics, in terms of genomic variation, cell morphology and clinical presentation. At present, only ~66% of patients are cured with initial treatment and those with refractory DLBCL exhibit a poor prognosis. Thus, further investigations into novel effective treatment options for DLBCL are required. The present study reports the case of a patient resistant to multiple therapies, including rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) plus enzastaurin (trial no. CTR20171560), GemOx plus lenalidomide and selinexor (trial no. ATG-010-DLBCL-001). The patient harbored a amplification, as identified via next-generation sequencing (NGS), and exhibited a high programmed death-ligand 1 Tumor Proportion Score of up to 95%. Consequently, the patient was treated with sintilimab monotherapy and the response lasted for 12 months of follow-up without major immune-related adverse events. This case highlights the role of NGS technology in selecting treatment options for refractory DLBCL. Furthermore, the results of the present study suggest that sintilimab may have potential in the treatment of patients with refractory DLBCL.
PubMed: 38736746
DOI: 10.3892/ol.2024.14423 -
Diagnostics (Basel, Switzerland) Apr 2024The present report describes the history of a 58-year-old woman with a rapidly progressing neuroendocrine pancreatic tumor (initially G2) presenting with extensive...
Impressive Response to TANDEM Peptide Receptor Radionuclide Therapy with Lu/AcDOTA-LM3 Somatostatin Receptor Antagonist in a Patient with Therapy-Refractory, Rapidly Progressive Neuroendocrine Neoplasm of the Pancreas.
The present report describes the history of a 58-year-old woman with a rapidly progressing neuroendocrine pancreatic tumor (initially G2) presenting with extensive liver, bone, and lymph node metastases. Previous treatments included chemotherapy, hemithyroidectomy for right lobe metastasis, Peptide Receptor Radionuclide Therapy (PRRT) with [Lu]Lu-DOTATATE, Lanreotide, Everolimus, and liver embolization. Due to severe disease progression, after a liver biopsy revealing tumor grade G3, PRRT with the somatostatin receptor antagonist LM3 was initiated. [Ga]GaDOTA-LM3 PET/CT showed intense tracer uptake in the liver, pancreatic tumor, lymph nodes, and bone metastases. Three TANDEM-PRRT cycles using [Lu]LuDOTA-LM3 and [Ac]AcDOTA-LM3, administered concurrently, resulted in significant improvement, notably in liver metastases, hepatomegaly reduction, the complete regression of bone and lymph node metastases, and primary tumor improvement. Partial remission was confirmed by positron emission tomography/computed tomography, chest-abdomen-pelvis contrast-enhanced computed tomography, and magnetic resonance of the abdomen, with marked clinical improvement in pain, energy levels, and quality of life, enabling full resumption of physical activity.
PubMed: 38732321
DOI: 10.3390/diagnostics14090907 -
Clinical Gastroenterology and... May 2024The impact of thiopurine de-escalation while on vedolizumab versus continuing thiopurine therapy in ulcerative colitis (UC) is unclear. We aimed to determine the effect...
BACKGROUND & AIMS
The impact of thiopurine de-escalation while on vedolizumab versus continuing thiopurine therapy in ulcerative colitis (UC) is unclear. We aimed to determine the effect of thiopurine withdrawal for patients with UC in remission on vedolizumab.
METHODS
This multicenter randomized controlled trial recruited UC patients on vedolizumab 300 mg intravenously every 8 weeks and a thiopurine. Patients in steroid-free clinical remission for ≥6 months and endoscopic remission/improvement (Mayo endoscopic subscore ≤1) were randomized 2:1 to withdraw or continue thiopurine. Primary outcome was comparing week 48 vedolizumab trough concentrations. Secondary outcomes were clinical relapse (partial Mayo score ≥3 and fecal calprotectin >150 μg/g or increase in Mayo endoscopic subscore ≥1 from baseline), fecal calprotectin remission (<150 μg/g), C-reactive protein remission (<5 mg/L), centrally read endoscopic remission (Mayo endoscopic subscore = 0), histologic remission (Nancy index = 0), histo-endoscopic remission, and adverse events.
RESULTS
In total, 62 patients were randomized to continue (n = 20) or withdraw (n = 42) thiopurine. At week 48, vedolizumab trough concentrations were not significantly different between continue and withdrawal groups (14.7 μg/mL, interquartile rate [IQR], 12.3-18.5 μg/mL versus 15.9 μg/mL, IQR, 10.1-22.7 μg/mL, respectively, P = 0.36). The continue group had significantly higher fecal calprotectin remission (95.0%, 19/20 versus 71.4%, 30/42; P = .03), histologic remission (80.0%, 16/20 versus 48.6%, 18/37; P = .02), and histo-endoscopic remission (75.0%, 15/20 versus 32.4%, 12/37; P = .002) than the withdrawal group. Histologic activity (hazard ratio [HR], 15.5; 95% confidence interval [CI], 1.6-146.5; P = .02) and prior anti-tumor necrosis factor exposure (HR, 6.5; 95% CI, 1.3-33.8; P = .03) predicted clinical relapse after thiopurine withdrawal.
CONCLUSIONS
Thiopurine withdrawal did not affect vedolizumab trough concentrations. However, it may increase fecal calprotectin, histologic, and histo-endoscopic activity. Histologic activity and prior anti-tumor necrosis factor exposure may predict disease relapse on thiopurine withdrawal for patients using vedolizumab for UC. Australian and New Zealand Trial Registry, number ACTRN12618000812291.
PubMed: 38729400
DOI: 10.1016/j.cgh.2024.04.019 -
Frontiers in Oncology 2024Sarcomatoid carcinoma (SC) is a rare, complex, aggressive tumor that spreads rapidly, is highly malignant, and has metastasized. Surgical resection is the primary...
Sarcomatoid carcinoma (SC) is a rare, complex, aggressive tumor that spreads rapidly, is highly malignant, and has metastasized. Surgical resection is the primary treatment, and it usually occurs in the lungs and kidneys but rarely in the neck. Patients with advanced sarcomatoid carcinoma (SC) of the head and neck (HN) have a poor progonsis. In recent years, immune checkpoint inhibitors (ICIs) have been established as treatments for many solid tumors; however, the effectiveness of ICIs in treating SC of HN is still little recognized. We report a case study of a middle-aged woman with primary sarcomatoid carcinoma of the neck. She developed sarcomatoid carcinoma of the contralateral neck 7 months after the first surgical treatment. Subsequently, disease recurrence and metastasis occurred 8 months after the second surgery. The patient did not receive any treatment after both surgeries. The tumor showed high programmed death-ligand 1 (PD-L1) expression, with a combined positive score (CPS): 95. The patient's response to treatment was assessed as partial remission (PR) after 2 cycles of anlotinib combined with sintilimab. The patient has survived for over 2 years and remains in PR status, despite experiencing grade 2 hypothyroidism as an adverse event during treatment. The case highlights the efficacy and safety of anlotinib and sintilimab as a first-line treatment.
PubMed: 38725630
DOI: 10.3389/fonc.2024.1362160 -
PloS One 2024M-type phospholipase A2 receptor (PLA2R) is a major auto-antigen of primary membranous nephropathy(PMN). Anti-PLA2R antibody levels are closely associated with disease...
BACKGROUND
M-type phospholipase A2 receptor (PLA2R) is a major auto-antigen of primary membranous nephropathy(PMN). Anti-PLA2R antibody levels are closely associated with disease severity and therapeutic effectiveness. Analysis of PLA2R antigen epitope reactivity may have a greater predictive value for remission compared with total PLA2R-antibody level. This study aims to elucidate the relationship between domain-specific antibody levels and clinical outcomes of PMN.
METHODS
This retrospective analysis included 87 patients with PLA2R-associated PMN. Among them, 40 and 47 were treated with rituximab (RTX) and cyclophosphamide (CTX) regimen, respectively. The quantitative detection of -immunoglobulin G (IgG)/-IgG4 targeting PLA2R and its epitope levels in the serum of patients with PMN were obtained through time-resolved fluorescence immunoassays and served as biomarkers in evaluating the treatment effectiveness. A predictive PMN remission possibility nomogram was developed using multivariate logistic regression analysis. Discrimination in the prediction model was assessed using the area under the receiver operating characteristic curve (AUC-ROC).Bootstrap ROC was used to evaluate the performance of the prediction model.
RESULTS
After a 6-month treatment period, the remission rates of proteinuria, including complete remission and partial remission in the RTX and CTX groups, were 70% and 70.21% (P = 0.983), respectively. However, there was a significant difference in immunological remission in the PLA2R-IgG4 between the RTX and CTX groups (21.43% vs. 61.90%, P = 0.019). Furthermore, we found differences in PLA2R-CysR-IgG4(P = 0.030), PLA2R-CTLD1-IgG4(P = 0.005), PLA2R-CTLD678-IgG4(P = 0.003), and epitope spreading (P = 0.023) between responders and non-responders in the CTX group. Multivariate logistic analysis showed that higher levels of urinary protein (odds ratio [OR], 0.49; 95% confidence interval [CI], 0.26-0.95; P = 0.035) and higher levels of PLA2R-CTLD1-IgG4 (OR, 0.79; 95%CI,0.62-0.99; P = 0.041) were independent risk factors for early remission. A multivariate model for estimating the possibility of early remission in patients with PMN is presented as a nomogram. The AUC-ROC of our model was 0.721 (95%CI, 0.601-0.840), in consistency with the results obtained with internal validation, for which the AUC-ROC was 0.711 (95%CI, 0.587-0.824), thus, demonstrating robustness.
CONCLUSIONS
Cyclophosphamide can induce immunological remission earlier than rituximab at the span of 6 months. The PLA2R-CTLD1-IgG4 has a better predict value than total PLA2R-IgG for remission of proteinuria at the 6th month.
Topics: Humans; Glomerulonephritis, Membranous; Receptors, Phospholipase A2; Male; Female; Retrospective Studies; Middle Aged; Rituximab; Remission Induction; Autoantibodies; Adult; Immunoglobulin G; Cyclophosphamide; Aged; ROC Curve; Treatment Outcome
PubMed: 38718066
DOI: 10.1371/journal.pone.0302100