Background
In Egypt, the characterization of Neuromyelitis Optica Spectrum Disorder (NMOSD) is lacking.
Objectives
To determine the demographics, clinical features, aquaporin4 antibodies (AQP4-IgG) status, and neuroimaging of Egyptian NMOSD patients.
Methods
Retrospective analysis of 70 NMOSD patients’ records from the MS clinic, Kasr Alainy hospital, between January 2013 and June 2018.
Results
Patients’ mean age was 34.9 ± 9.2 years, and the mean at disease onset was 28.9 ± 10.5 years. Fifty-nine patients had an initial monosymptomatic presentation. AQP4-IgG was measured using either enzyme-linked immunosorbent assay (ELISA) (22 patients) or cell-based assay (CBA) (34 patients). Six and 29 patients had positive results, respectively (p < 0.001). 84% had typical NMOSD brain lesions. Longitudinally extensive myelitis was detected in 49 patients, and 9 had either short segments or normal cords. Treatment failure was higher in seropositive patients. Rituximab significantly reduced the annualized relapse rate (ARR) compared to Azathioprine with a percentage reduction of (76.47 ± 13.28) and (10.21 ± 96.07), respectively (p = 0.04). Age at disease onset was the only independent predictor for disability (p < 0.01).
Conclusion
Treatment failure was higher in seropositive patients. However, there was no difference in clinical or radiological parameters between seropositive and seronegative patients. Patients, who are polysymptomatic or with older age of onset, are predicted to have higher future disability regardless of the AQP4-IgG status.
Purpose: The diagnosis of pediatric autoimmune gastritis (AIG) is critical due to the poor outcome and risk of malignancy. Therefore, we evaluated the prevalence of autoimmune gastritis in children with autoimmune thyroid disease (ATD) and type 1 diabetes mellitus (T1D), using parietal cell antibody (PCA) to identify its use as a screening test. Methods: PCA was measured in 90 patients; 45 patients with ATD (Hashimoto Thyroiditis and Graves' Disease) and 45 patients with T1D. Their ages ranged from 5 to 18 years. Hemoglobin, ferritin, and HbA1c (in diabetic patients) were measured. Results: PCA demonstrated a statistically significant difference between the two groups of patients with a p-value (0.024). The mean value of PCA in patients with T1D was 196.45, while it reached 148.58 in patients with ATD, with a p-value (0.003). Thirty-one (68.9%) and twelve (26.7%) of patients with T1D had high (30-200) and extremely high (> 200) values of PCA, respectively, compared to twenty-six (57.8%) and eight (17.8%) of patients with ATD who had high (30-200) and extremely high (> 200) values of PCA, respectively. Seven (87.5%) of patients with ATD with extremely high PCA( above 200) also had normocytic anemia.
BACKGROUND: Despite the growing landscape of genetic drivers in Diffuse Large B-cell Lymphoma, yet their clinical implication is still unclear and R-CHOP regimen remains a “one size fits all” therapy. We aimed in this study to examine the prevalence of EZH2, BCL211 and MYD 88 genetic polymorphisms in DLBCL patients and correlate the results with various clinical and survival outcomes.
METHODS: Genotyping of MYD88 (rs387907272 T/C), EZH2 (rs3757441 C/T), and BCL2L11 (rs3789068 A/G) polymorphisms were conducted using real time polymerase chain reaction analysis in a total of 75 DLBCL patients.
RESULTS: Most of our cases carried the wild TT genotype of MYD88 gene (64%), the mutant TT genotype of EZH2 gene (52%) and the wild AA genotype of BCL2L11 gene (48%). Regarding cell of origin, Germinal Centre (GC) phenotype was present in 56% of cases while 44% expressed the Post-GC (PGC) phenotype. Poor response outcome to first line R-CHOP was significantly correlated with the mutated CC genotype of MYD 88 (p=0.02), while better response to R-CHOP was significantly associated with younger age <50 years (p <0.0001), good PS (p=0.046), normal LDH level (p=0.003), earlier stage (p <0.0001), good IPI score (p=0.009), absence of extranodal disease (p <0.0001) and absence of bulky disease (p=0.004). The median PFS and the 2 year OS were significantly higher in younger age, earlier stage, good IPI score, absence of extranodal disease, absence of bulky disease and in GC phenotype.
CONCLUSIONS: Our results emphasized that the mutated genotype of MYD 88 gene polymorphism is significantly associated with poor response to R-CHOP therapy.
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