cord colitis syndrome

Cord colitis syndrome (CCS) is a recently described diarrheal illness of uncertain pathogenesis that affects recipients of umbilical cord blood transplant and is associated with negative cultures.

CCS exhibits a peculiar histopathologic appearance, as it commonly manifests as granulomatous inflammation involving the upper and lower gastrointestinal tract, with features of chronicity in the colon.

Importantly, the treatment for CCS differs from that for acute graft-versus-host disease, which is commonly in the clinical differential diagnosis: CCS responds to antibiotic treatment, whereas acute graft-versus-host disease responds to immunosuppression.

Endoscopic biopsies of the stomach and colon reveal granulomatous inflammation, consisting of scattered ill-defined aggregates of epithelioid histiocytes, with associated mild neutrophilic inflammation and mildly increased epithelial cell apoptosis.

In the colon, the granulomatous inflammation is associated with surface epithelial injury (including surface erosions) and contained occasional multinucleated epithelioid giant cells.

Paneth cell metaplasia is present in the distal colon, but crypt architecture is preserved, and there is no basal lymphoplasmacytosis.

Special stains and immunohistochemical stains for infectious organisms are negative.

Reference

 Cord colitis syndrome: a cause of granulomatous inflammation in the upper and lower gastrointestinal tract. Gupta NK, Masia R. Am J Surg Pathol. 2013 Jul;37(7):1109-13. doi : 10.1097/PAS.0b013e31828a827a PMID: 23715165