Successful second bone marrow transplantation in Omenn's syndrome after bone marrow aplasia: a case report.

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.

journal_name

Pediatr Transplant

authors

Wahadneh A,Bin-Dahman H,Habahbeh Z,Abu-Shukear M,Ajarmeh M,Zyood R,Khaled A

doi

10.1111/j.1399-3046.2010.01413.x

subject

Has Abstract

pub_date

2012-03-01 00:00:00

pages

E43-8

issue

2

eissn

1397-3142

issn

1399-3046

journal_volume

16

pub_type

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