Proposed diagnostic scoring system for CNS-cGvHD

A proposed diagnostic scoring system for central nervous system (CNS) manifestations of chronic graft-versus-host disease (cGvHD; CNS-cGvHD) was published in Transplantation and Cellular Therapy by Denk et al. The 10-point scoring system was retrospectively applied to a single-center cohort of 770 patients who survived ≥3 months after allogeneic hematopoietic stem cell transplantation (allo-HSCT). Using the scoring system, CNS-cGvHD was categorized as unlikely, possible, or probable. 

Key data: The three CNS-cGvHD phenotypes proposed were vasculitis-like, meningoencephalitis, and demyelinating. Among 770 patients screened, 19 presented with possible (n = 6) or probable (n = 13) CNS-cGvHD. For each phenotype, a 10-point scoring system integrated clinical features, neuroimaging, cerebrospinal fluid (CSF) findings, extraneurologic cGvHD manifestations, onset after immunosuppression taper ≥3 months after allo-HSCT or donor lymphocyte infusion (DLI) / stem cell backup, histology, and response to immunosuppressive therapy (IST). Of 19 suspected CNS-cGvHD cases, 12 were classified as meningoencephalitis, four as vasculitis-like, one as demyelinating, and two as mixed-phenotype. Most patients scored 4–6 points; no patients had a score ≥7 points, largely attributed to the lack of CNS biopsies. 

Key learning: The proposed diagnostic scoring system provides a framework for consistent diagnosis and classification of atypical presentations of cGvHD in the CNS. Prospective validation in larger, multicenter cohorts is required to support its clinical utility.