Successful second bone marrow transplantation in Omenn's syndrome after bone marrow aplasia: a case report.
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.
Pediatric Immunology & Allergy & Rheumatology Section, Queen Rania Children Hospital, King Hussein Medical Center, Royal Medical Services, Amman, Jordan.
Adel
A
Wahadneh
Haifa
H
Bin-Dahman
Zeyad
Z
Habahbeh
Mohammad
M
Abu-Shukear
Mohammad
M
Ajarmeh
Raed
R
Zyood
Ahmad
A
Khaled
D016026:Bone Marrow Transplantation; D006086:Graft vs Host Disease / Q000175:diagnosis / Q000209:etiology / Q000517:prevention & control; D006801:Humans; D007223:Infant; D008297:Male; D009163:Mycobacterium bovis; D010198:Pancytopenia / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*; D036102:Peripheral Blood Stem Cell Transplantation; D011183:Postoperative Complications / Q000175:diagnosis / Q000517:prevention & control / Q000601:surgery*; D012086:Reoperation; D016511:Severe Combined Immunodeficiency / Q000150:complications / Q000175:diagnosis / Q000601:surgery*; D019172:Transplantation Conditioning; D014376:Tuberculosis / Q000175:diagnosis / Q000209:etiology