Bone Marrow Transplantation

TABLE 2

FROM:

Reduced intensity conditioning and allogeneic stem cell transplantation in childhood malignant and nonmalignant diseases

P Satwani, N Cooper, K Rao, P Veys and P Amrolia

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Table 2. Primary immunodeficiencies treated with RIC-SCT

WASP deficiency  
CD40 ligand deficiency  
XLP  
Hemophagocytic syndromes  
 Immunodeficiency with partial albinism 
 Familial HLH 
 Griscelli disease 
 Chediak–Higashi syndrome 
  
Phagocytic cell disorders  
 Schwachman's syndrome 
 Granule deficiency 
 LAD 
 X-linked/AR CGD 
 Kostmann's syndrome 
 IFN-gamma receptor deficiency 
 Other 
  
Autoimmune/immune dysregulatory  
 ALPS, IPEX 
 Other 
  
Severe combined immunodeficiency (SCID) 
Functional Genetic
T- B- NK- ADA deficiency
 Reticular dysgenesis
T- B- NK+ RAG deficiency
 SCID with Artemis
T- B+ NK- gamma deficiency (X-linked)
 Jak 3 kinase deficiency (AR)
T- B+ NK+ IL7 Ralpha deficiency
Unspecified 
Other 
  
T-cell immunodeficiency/SCID variants  
 CD4 lymphopenia 
 Zap 70 kinase deficiency 
 MHC class II deficiency 
 PNP deficiency 
 Omenn's syndrome 
 Severe DiGeorge complex (22q 11del) 
 CID with skeletal dysplasia 
 Cartilage hair hypoplasia 

 Abbreviations: ADA=adenosine deaminase deficiency; ALPS=autoimmune lymphoproliferative syndrome; AR CGD=chronic granulomatous disease; CID=combined immunodeficiency disease; HLH=hemophagocytic lymphohistiocytosis; IFN=interferon; IPEX=immune dysregulation, polyendocrinopathy and enteropathy, X-linked; LAD=leukocyte adhesion deficiency; RAG=recombinase activating gene; RIC=reduced intensity conditioning; MHC=major histocompatibility complex; PNP=purine nucleoside phosphorylase; SCT=stem cell transplantation; WASP=Wiskott–Aldrich syndrome protein; XLP=X-linked lymphoproliferative disorder.

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