Introduction: There are limited case reports regarding patients who have received solid organ and hematopoietic cell transplant (HCT). Herein we describe the case of a now 20-year-old female with combined immunodeficiency and intestinal atresia who underwent a cord blood transplant, followed by an intestinal transplant(ITx). Our patient had gastroschisis with resultant short-gut syndrome. She developed lymphopenia and hypogammaglobulinemia with absent response to pneumococcal antigens, low number of T-cell receptor excision circles, and recurrent infections. Given these findings, she was diagnosed with combined immunodeficiency. ITx was deemed too risky due to her recurrent infections and immunodeficiency. Thus, she was evaluated for HCT.

Methods: She received a conditioning regimen of hydroxyurea, alemtuzumab, fludarabine, melphalan and thiotepa, with GVHD prophylaxis of tacrolimus (TAC) and mycophenolate mofetil (MMF). One month after HCT, MMF was discontinued. She received a 6/6 antigen-matched unrelated umbilical cord blood transplant in May of 2013. Nineteen months later, she received a 3/6 HLA matched intestine and colon allograft from a deceased donor. She was maintained on TAC and steroids, and also received basiliximab.

Results: One month post-HCT, she had ~50% donor cells in her blood. Her TAC dose was decreased and she received a cryopreserved fraction of her umbilical cord blood graft, with significant improvement. She developed acute GVHD limited to the skin, which was successfully treated with topical creams. Six weeks following her ITx, she was found on surveillance endoscopy to have mild ACR. This resolved with steroid therapy and the addition of MMF. Six months after her ITx, was noted to have an 8.5 mm pulmonary nodule on surveillance CT scan. Pathology revealed EBER+ PLTD. She underwent successful resection of the lesion and her immunosuppression was decreased with no evidence of recurrence. Most recent peripheral blood studies in December 2018 show she is 86-93% donor engrafted. Her immune profile remains normal, she’s had no recent infections, and her only immunosuppressant is TAC with troughs in the 2-3 ng/mL range.

Conclusion: To our knowledge, this is the only reported successful case of a patient who received HCT followed by ITx. Despite not having a perfect HLA match between the HCT and intestinal donors, the patient achieved prope immune tolerance with the need for very low level immunosuppression and no significant GVHD