Case Report: An abnormal PTLD onset with epithelial mucosal damage that was successfully managed with anti-B cell therapy.

RUMBO C; CABANNE A; MARTINEZ M; PUCCI MOLINERIS M; MEIER D; GRACIA HERVAS D; GONDOLESI G; RUMBO M

Simposio; International Small Bowel Transplant Symposium 2015; 2015

Intestinal Transplant Association

The incidence of PTLD in intestinal transplant (ITx) patients is higherthan in other types of solid organ transplant. Although in early stagesthe diagnosis may not be simple due to non specific symptoms, thepresence of highly proliferating lymphoid masses is pathognomonic.The treatment is not standardized and usually rituximab is used asfirst line treatment. Here we report a case of pediatric PTLD withatypical presentation.Patient and Method: This is a 25 months old boy that was admitted13 months after isolated ITx presenting with fever, profuse diarrhea,and low albumin level (1.5 mg/dL). Initial endoscopy and histologyshowed non-specific inflammation. Infectious diarrhea was ruled outby stool tests. Intravenous antibiotics were started and enteral dietwas discontinued. Four days later histology of intestinal mucosashowed extensive epithelial damage compatible with severe acutecellular rejection affecting jejunal and ileal graft but also native duodenum.High EBV load was detected in serum (57,000 copies/mL)corresponding to admission day (primo-infection). Seven days postadmissionmucosal histology showed presence of EBER positivecells with a increased proportion of CD20+ o CD138+ cells in thelamina propria detected by immunohistochemistry, either in nativeintestine or in the graft mucosa, compatible with PTLD diagnosis.Chest, abdomen and pelvis CT scan and a biopsy of an inguinal nodewere done as part of the work up showing negative results.Serum EBV load continued to rise in spite of treatment with IV ganciclovirstarted at day 4 post-admission. Upon PTLD diagnosis, treatmentwith rituximab 375 mg/kg was installed in 3 doses spared by 3days each and immunosuppression was decreased. After the first rituximabdose, complete disappearance of CD20+ cells in serum wasconfirmed by flow cytometry; also, a remarkable decrease of CD20+cells in graft mucosa was observed. Recovery of normal intestinalhistology was observed at day 4 post-rituximab treatment. Enteralnutrition was successfully restarted at day 20 post admission. EVBviral load in serum become negative at day 39 after hospital admission.Patient is free of disease 5 months after treatment completion.Conclusion: Abnormal B cell proliferation in PTLD cases is not expectedto generate epithelial damage; therefore, the presentation ofthis case was atypical. The use of matched biopsies in graft and nativeintestine is highly useful to determine the presence of immunologicalvs. infectious events Treatment with rituximab was highlyeffective in this case, suggesting including it as first treatment optionin early stages of PTLD.References:1. Pediatr Transplantation 2013: 17: 765?7732. BLOOD, 21 FEBRUARY 2013 VOLUME 121, NUMBER 8, 13373. Pediatr Transplantation 2013: 17: 472?478