Chronic norovirus infection in primary immune deficiency disorders: an international case series

• Published in: Diagnostic microbiology and infectious disease Vol. 93; no. 1; pp. 69 - 73
• Main Authors: Rolfes, M.C., Sriaroon, P., Dávila Saldaña, B.J., Dvorak, C.C., Chapdelaine, H., Ferdman, R.M., Chen, K., Jolles, S., Patel, N.C., Kim, Y.J., Tarrant, T.K., Martelius, T, Seppanen, M., Joshi, A.Y.
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01-01-2019
• Subjects: Chronic gastroenteritis; CVID, SCID, transplant, immunocompromised; Norovirus; Primary immunodeficiency diseases PIDD; GI; Norovirus; GII; GI/II; IgG; WAS; Primary immunodeficiency diseases PIDD; Chronic gastroenteritis; SCID; CVID; NoV; PIDD; NK; RT-qPCR; CNI; CID; BMI; CVID, SCID, transplant, immunocompromised
• ISSN: 0732-8893; 1879-0070
• DOI: 10.1016/j.diagmicrobio.2018.08.002

Predictive factors associated with clinical outcomes of chronic norovirus infection (CNI) in primary immunodeficiency diseases (PIDD) are lacking.
We sought to characterize CNI using a multi-institutional cohort of patients with PIDD and CNI using the Clinical Immunology Society's CIS-PIDD Listserv e-mail group.
Thirty-four subjects (21 males and 13 females) were reported from centers across North America, Europe, and Asia. All subjects were receiving high doses (median IgG dose: 1200 mg/kg/month) of supplemental immunoglobulin therapy. Fifty-three percent had a complete absence of B cells (median B-cell count 0; range 0–139 cells/μL). Common Variable Immune Deficiency (CVID) subjects manifested a unique phenotype with B-cell lymphopenia, non O+ blood type, and villous atrophy (logistic regression model, P = 0.01). Five subjects died, all of whom had no evidence of villous atrophy.
While Norovirus (NoV) is thought to replicate in B cells, in this PIDD cohort of CNI, B-cell lymphopenia was common, indicating that the presence of B lymphocytes is not essential for CNI.