Celiac Disease Transmitted By Unrelated Cord Blood Stem Cell Transplantation (CBST)

Abstract

Celiac disease is due to intolerance to certain cereal proteins leading to immune-mediated small bowel villous atrophy and malabsorption. Specifically, the gliadin component of wheat, and the prolamin component of rye and barley are implicated in causing disease. BMT and CBST have been known to transmit immune associated diseases such as diabetes mellitus immune thrombocytopenic purpura from an affected donor to transplant recipient. We observed the occurrence of Celiac disease in a patient a year following cord blood stem cell transplantation (CBST) for acute myelogenous leukemia (AML- FAB M2) in complete second remission (CR-2). The patient had no history of celiac disease prior to CBST nor any family member. The cord donor was HLA-identical unrelated male donor with HLA types: A3, B7 (w6), DR (B1), DR (B5). The family history of the donor was unavailable for celiac disease. The CBST was complicated by grade 2 skin Graft versus Host Disease (GVHD), which responded to steroid therapy. A year post transplantation she developed persistent mucous diarrhea with tinge of blood associated with abdominal cramps. Investigations for infectious causes such as CMV enteritis were negative and colonsocopy did not reveal any evidence of GVHD. Gastrointestinal symptoms persisted and did not respond to steroid and prograf therapy. Subsequent duodenal and jejunal biopsy revealed subtotal villous atrophy with cryptic hyperplasia suggestive of celiac disease. Antigliadin IgA and IgG, reticulin IgA and Endomysial IgA andibodies were elevated. A diagnosis of post-CBST coeliac disease was made. She responded well to gluten-free diet and became symptom-free. A literature review identifies only from donor to recipient one previous example of transmission of celiac disease following HSCT. This case could be the second case of celiac disease following transplantation and the first one post CBST. An association of celiac disease with some HLA types, including DQA1.0501, and DQB1.0201, in conjunction with the haplotypes A30, B18, DR3, DRw52, and DQ2 was recently noted. The donor of this case exhibit HLA DQ A1.0501 and DQ B1.0201 alleles. The findings suggest transfer of celiac disease by cord stem cells and confirm the immune nature of the disease. In addition the propensity to develop T-cell non-Hodgkin lymphoma and transmission of celiac disease by CBST support T cell concept in celiac disease. Autoimmune enteropathy should be considered in patient with persistent diarrhea post hemopoietic stem cell transplant.