Bone marrow transplantation for CD40 ligand deficiency: a single centre experience

Abstract

(Arch Dis Child 2001;84:508-511)

Allogeneic hematopoietic stem cell transplantation for seven children with X‐linked hyper‐IgM syndrome: A single center experience

“…As for pre-transplantation infections in our series, only one patient had cryptosporidial infection and five out of seven patients had previous episodes of P. carinii pneumonitis. As mentioned in previous reports, cryptosporidial infection seemed to correlate with a poor outcome, but P. carinii infection did not [20]. Although both two patients with poor outcome had P. carinii pneumonitis previously, P. carinii infection itself was well managed after transplantation.…”

“…Nonmyeloablative conditioning regimens consisted of fludarabine and melphalan with or without ATG or anti-lymphocyte globulin and were used in four cases with high-risk features, such as severe pretransplant liver complications or sclerosing cholangitis. Although these preliminary reports are relatively successful, Khawaja et al reported four failures out of eight transplanted patients, and that risk and death were associated with older age, pre-transplant liver damage, and cryptosporidial infection [20].…”

“…Another patient also suffered from pre-transplant complications involving the lung, but the failure was due to severe post-transplantation infection complicated with secondary graft failure and grade 4 acute GVHD. It has been observed that older patients with primary immunodeficiency disorders tend to be at higher risk for HSCT [11,20]. The reason for this is that they may have more severe pre-transplant infections and may have serious damage in critical organs such as lung and liver because of recurrent or persistent infections.…”

Complete recovery from Cryptosporidium parvum infection with gastroenteritis and sclerosing cholangitis after successful bone marrow transplantation in two brothers with X-linked hyper-IgM syndrome

“…8 However, the presence of C. parvum infection, especially when complicated by sclerosing cholangitis, impairs the results of BMT. Khawaja et al 9 reported three cases where patients suffering from severe C. parvum infection died soon after BMT. 9 In this report, we describe two brothers with HIGM1 who were both suffering from severe C. parvum infection with gastroenteritis and sclerosing cholangitis.…”

“…Khawaja et al 9 reported three cases where patients suffering from severe C. parvum infection died soon after BMT. 9 In this report, we describe two brothers with HIGM1 who were both suffering from severe C. parvum infection with gastroenteritis and sclerosing cholangitis. They underwent successful BMT from a genoidentical HLA sibling and recovered from the C. parvum infection.…”