Patient 1:
A two-year-old boy, first and the only child of non-consanguineous parents was diagnosed as SCID due to IL2RG mutation at 8 months with a severe CMV pneumonia treated at ICU. Since a matched donor was not available in the family, he underwent unconditioned haploidentical transplantation from his father at 16 months. However, a primary graft failure occurred and seven months later he retransplanted from an HLA 10/10 matched unrelated donor (MUD) following an RIC regimen consisting of Treosulfan (42g/m2), Fludarabine (150mg/m2) and ATG (20mg/kg). CSA was preferred for GvHD prophylaxis. On post-transplant 7th day, grade I skin aGvHD was detected that Methylprednisolone 2mg/kg/day was added to the treatment. Since aGvHD of the skin did not respond to corticosteroid but progressively exaggerated, infusions (3 x) of mesenchymal stem cells and MMF were initiated followed by tacrolimus which was then switched to sirolimus. On post-transplant 63rd day, during the steroid tapering schedule, severe diarrhea (>2000cc/day) and subsequently melena and fresh intestinal bleeding occurred. A Grade IV GvHD was revealed by endoscopic and colonoscopic biopsies. Generalized ulcers were found in the whole colon; however, the area of bleeding could not be detected. Coagulation parameters were normal with insignificant thrombocytopenia (platelet levels ranging from 50 to 120x109/L). However, erythrocyte transfusion twice a day was required to maintain haemoglobin level. Oral budesonide and one additional mesenchymal stem cells infusion were added to the treatment. Furthermore, he did not respond to Tocilizumab (2x) (recombinant humanized anti-interleukin-6 receptor (IL-6R) monoclonal antibody) which was given in every two weeks.12-13 Severe and several immunosuppressive agents refractory intestinal GvHD was controlled by 8 doses of alpha1-antitrypsin.(Table 1) After third dose of alpha1-antitrypsin treatment, although diarrhea started to resolve, intestinal bleeding persisted that Ankaferd Blood Stopper (a haemostatic powder approved in Turkey) 14 initiated orally (2 ml via nasogastric tube, twice a day for 7 days). After 4 days of treatment, frequency and the amount of intestinal bleeding substantially decreased while completely resolved following the first week. His oral intake increased subsequently and he went home on post-transplant 139th day.