National Institutes of Health Consensus Development Project on Criteria for Clinical Trials in Chronic Graft-versus-Host Disease: I. Diagnosis and Staging Working Group Report

Abstract

This consensus document is intended to serve 3 functions. First, it standardizes the criteria for diagnosis of chronic graft-versus-host disease (GVHD). Second, it proposes a new clinical scoring system (0-3) that describes the extent and severity of chronic GVHD for each organ or site at any given time, taking functional impact into account. Third, it proposes new guidelines for global assessment of chronic GVHD severity that are based on the number of organs or sites involved and the degree of involvement in affected organs (mild, moderate, or severe). Diagnosis of chronic GVHD requires the presence of at least 1 diagnostic clinical sign of chronic GVHD (e.g., poikiloderma or esophageal web) or the presence of at least 1 distinctive manifestation (e.g., keratoconjunctivitis sicca) confirmed by pertinent biopsy or other relevant tests (e.g., Schirmer test) in the same or another organ. Furthermore, other possible diagnoses for clinical symptoms must be excluded. No time limit is set for the diagnosis of chronic GVHD. The Working Group recognized 2 main categories of GVHD, each with 2 subcategories. The acute GVHD category is defined in the absence of diagnostic or distinctive features of chronic GVHD and includes (1) classic acute GVHD occurring within 100 days after transplantation and (2) persistent, recurrent, or late acute GVHD (features of acute GVHD occurring beyond 100 days, often during withdrawal of immune suppression). The broad category of chronic GVHD includes (1) classic chronic GVHD (without features or characteristics of acute GVHD) and (2) an overlap syndrome in which diagnostic or distinctive features of chronic GVHD and acute GVHD appear together. It is currently recommended that systemic therapy be considered for patients who meet criteria for chronic GVHD of moderate to severe global severity.

Outcomes after Unrelated Umbilical Cord Blood Transplantation for Children with Osteopetrosis

“…Our analysis confirms that UCBT in children with OP, together with HSCT from other alternative donors, constitutes a significant clinical challenge, due to the high incidence of graft failure and transplant-related mortality 7 in this specific disease.…”

“…Chronic GvHD was evaluated considering only patients surviving more than 100 days after transplantation with sustained donor engraftment, using previously published criteria 7 .…”

Reduced intensity compared with high dose conditioning for allotransplantation in acute myeloid leukemia and myelodysplastic syndrome: A comparative clinical analysis

“…GVHD was diagnosed using established criteria [38,39]. Acute GVHD most often developed within 100 days of HSCT (with cumulative incidence reported at day 100), and chronic GVHD was diagnosed when patients developed distinct clinical manifestations unique to chronic GVHD [40]. The cumulative incidence was reported at 1 year.…”

Better outcomes of modified myeloablative conditioning without antithymocyte globulin versus myeloablative conditioning in cord blood transplantation for hematological malignancies: A retrospective (development) and a prospective (validation) study

“…Primary graft failure was defined as the failure to reach an ANC ≥0.5 × 10 9 /L for at least three consecutive days or a mixed chimerism of donor cells ≥10% on Day 42 following transplantation based on chimerism analysis using polymorphic genetic markers 17. The assessment of pre‐engraftment syndrome, grading of acute GVHD (aGVHD) and grading of chronic GVHD (cGVHD) was performed according to standard criteria 18, 19, 20. Patients surviving in remission for least 100 days after transplantation were regularly evaluated for chronic GVHD.…”