Primary immune deficiencies and stem cell transplantation

Primary immunodeficiency disorder (PID) can be broadly grouped into disorders of adaptive immunity (T-cell, B-cell or combined immunodeficiencies) and disorders of innate immunity (phagocyte defects in Toll-like receptor– mediated signalling and complement disorders). In the European Society for Immunodeficiencies (ESID) Registry 2010, of 10,747 children diagnosed with PID, humoral deficiencies were the most common (Figure 1). This is similar to the data from Sri Lanka, where among 73 cases registered, common variable immune deficiency (CVID) was the commonest, followed by X linked agammaglobulinaemia.