Successful second bone marrow transplantation in Omenn's syndrome after bone marrow aplasia: a case report |
| |
Authors: | Wahadneh Adel Bin-Dahman Haifa Habahbeh Zeyad Abu-Shukear Mohammad Ajarmeh Mohammad Zyood Raed Khaled Ahmad |
| |
Affiliation: | Pediatric Immunology & Allergy & Rheumatology Section, Queen Rania Children Hospital, King Hussein Medical Center, Royal Medical Services, Amman, Jordan. |
| |
Abstract: | Omenn's syndrome is a rare inherited variant of SCID. It is inevitably fatal, unless treated by bone marrow or stem cell transplantation. However, treatment-related complications and graft rejection are major obstacles to the success of transplantation. In this report, we describe an eight-month-old baby with Omenn's syndrome and disseminated BCGosis, who underwent allogenic BMT from his HLA-matched sister using anti-GVHD prophylaxis but without a conditioning regimen. Ten days after BMT, he developed acute GVHD involving the skin only (grade 1) with a flare of local BCGosis. Engraftment of 55% was demonstrated by FISH as early as the 11th day post-BMT. On day +48 post-BMT, he developed GVHD that was complicated by refractory pancytopenia, resistant to three doses of rituximab. Patient was re-transplanted (from the same donor) after receiving fludarabine and ATG, with successful engraftment and complete recovery from pancytopenia. |
| |
Keywords: | allogenic stem cell transplantation graft‐versus‐host disease pancytopenia rituximab Omenn’s syndrome |
本文献已被 PubMed 等数据库收录! |
|