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Successful second bone marrow transplantation in Omenn’s syndrome after bone marrow aplasia: A case report
Author(s) -
Wahadneh Adel,
BinDahman Haifa,
Habahbeh Zeyad,
AbuShukear Mohammad,
Ajarmeh Mohammad,
Zyood Raed,
Khaled Ahmad
Publication year - 2012
Publication title -
pediatric transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.457
H-Index - 69
eISSN - 1399-3046
pISSN - 1397-3142
DOI - 10.1111/j.1399-3046.2010.01413.x
Subject(s) - medicine , pancytopenia , surgery , bone marrow aplasia , aplasia , transplantation , bone marrow , immunology
Wahadneh A, Bin‐Dahman H, Habahbeh Z, Abu‐Shukear M, Ajarmeh M, Zyood R, Khaled A. Successful second bone marrow transplantation in Omenn’s syndrome after bone marrow aplasia: A case report.
Pediatr Transplantation 2012: 16: E43–E48. © 2010 John Wiley & Sons A/S. 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.