Isolated Jejunal Graft-Versus-Host-Disease Following Peripheral Blood Stem Cell Transplantation

Abstract

Purpose: Acute graft-versus-host disease (GVHD) of the gastrointestinal tract is a known complication of peripheral blood stem cell transplantation (PBSCT). Acute GVHD occurs more commonly in cases that involve matched unrelated donors (MUD) and in haploidentical related donors than in related donors. Lower gastrointestinal tract-GVHD is more common than the GVHD of the upper GI tract, in particular, the esophagus and the stomach. Here, we are presenting a patient with an isolated acute GVHD of the jejunum after MUD peripheral blood stem cell transplantation. A 41-year-old female with past medical history of myelodysplastic syndrome - refractory anemia with excess blasts (MDS RAEB-2) was admitted to our institution one month after MUD-PBSCT. She had exhibited minimal response to a five-month treatment of Vidaza prior to stem cell transplantation. She was presented with generalized maculopapular rash, pruritus and scaling of the skin. In addition, she complained of gradually worsening anorexia, food intolerance, nausea, and high volume non-bloody diarrhea. The patient was on an intensive prophylaxis regiment for GVHD with immunosuppressants, in addition to daily PPI and Acyclovir. The initial work-up including the assessment for infectious diarrhea was negative. Subsequently, the patient had an EGD and colonoscopy which did not show any mucosal abnormalities. Random biopsy samples obtained during these procedures were not compatible with GVHD. Patient's skin biopsy showed a complex morphologic pattern compatible with chemotherapy side effects, however, GVHD was not excluded based on the skin biopsy result. For further evaluation of the GI related symptoms, the patient had a CT scan of the abdomen which showed nondilated fluid filled loops of small bowel with possible mild bowel wall thickening. In order to evaluate these abnormal CT scan findings, the patient had a small bowel enteroscopy. During the small bowel enteroscopy, the upper GI tract was found to be normal up to the proximal jejunum. The jejunal mucosa was inflamed and friable with multiple aphthous shallow ulcers and white plaques up to the mid jejunum (180 cm from the incisors). The biopsy of these lesions demonstrated active enteritis with focal apoptosis of crypt epithelium and loss of crypts, consistent with graft versus host disease, grade 2-3. After a thorough review of the literature, we were not able to identify any reported cases of isolated acute GVHD of jejunum in the setting of matched unrelated donor peripheral blood stem cell transplantation. To our knowledge, this is the first reported case of a patient with isolated acute GVHD of jejunum that is caused by PBSCT.