An international multicenter retrospective analysis of patients with extranodal marginal zone lymphoma and histologically confirmed central nervous system and dural involvement.

Marginal zone lymphoma of the central nervous system (CNS MZL) is rare. The clinical features, treatment, and prognosis are not well characterized. We performed a multicenter retrospective study of CNS MZL. Twenty-six patients were identified: half with primary and half with secondary CNS involvement. The median age was 59 years (range 26-78), 62% female and 79% with ECOG performance status ≤ 1. The most common disease site was the dura (50%). Treatment was determined by the treating physician and varied substantially. After a median follow up of 1.9 years, the estimated 2-year progression-free (PFS) and overall survival (OS) rates were 59% and 80%, respectively. Secondary CNS MZL was associated with 2-year OS of 58%. CNS MZL is rare, but relative to other forms of CNS lymphoma, outcomes appear favorable, particularly among the subset of patients with dural presentation and primary CNS presentation.

Peripheral T-Cell Lymphoma, Not Otherwise Specified: A Review of Current Disease Understanding and Therapeutic Approaches.

Peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS), corresponds with a heterogeneous group of mature T-cell lymphomas. Recent gene expression profiling studies have identified at least two molecular subgroups (GATA3 and TBX2). Standard treatment and outcomes remain poor. High-dose chemotherapy with autologous stem cell transplantation is incorporated into primary therapy for young fit patients but remains ineffective for most and has not been tested in a randomized study. Several novel agents have been approved for use in relapsed/refractory PTCLs, and although response rates are modest for most, durable remissions have been reported. Selecting rationale combinations and incorporating predictive biomarkers will be important moving forward to improve outcomes in patients with PTCL.

High prevalence of celiac disease among Saudi children with type 1 diabetes: a prospective cross-sectional study.

BACKGROUND: There is lack of data on prevalence of celiac disease (CD) in children with type 1 diabetes (T1D) in Arabs in the Middle East. The present investigation aims to study the prevalence rate and clinical characteristics of CD among Saudi children with T1D using a combination of the most sensitive and specific screening serologic tests (anti- tissue transglutaminase antibodies IgA [anti-TTG] and ednomyseal antibodies [EMA]) and to determine the lower cut-off value of anti- anti-TTG level that best predicts CD in children with T1D. METHODS: Children with T1D following in diabetic clinic have been prospectively screened for presence of CD, over a two-year period (2008-2010), by doing anti-TTG, EMA, and total IgA. Children with positive anti-TTG titres (>50 U/ml) and/or EMA and children with persistently low positive anti-TTG titres (two readings 20-50 U/ml; within 6 months intervals) had upper endoscopy and 6 duodenal biopsies. RESULTS: One hundred and six children with T1D have been screened for CD: age ranged between 8 months to 15.5 years (62 females). Nineteen children had positive anti-TTG and/or EMA, however only 12 children had biopsy proven CD (11.3%). Five of 12 had gastrointestinal symptoms (42%). Children with T1D and CD had significantly lower serum iron than children with T1D alone (8.5 µgm/L Vs 12.5 µgm/L; P = 0.014). The sensitivity and specificity of anti-TTG were 91.6% and 93.6%, with a positive and negative predictive value of 64.7% and 98.8%, respectively. Receiver operated characteristics analysis for the best cut-off value of anti-TTG level for diagnosis of CD was 63 units (sensitivity 100% and specificity 98.8%). CONCLUSION: CD is highly prevalent among Saudi children with T1D. Anti-TTG titres more than 3 times the upper limit of normal has very high sensitivity and specificity for diagnosis of CD in T1D children.