Use of intravenous immunoglobulin and adjunctive therapies in the treatment of primary immunodeficiencies: A working group report of and study by the Primary Immunodeficiency Committee of the American Academy of Allergy Asthma and Immunology.

There are an expanding number of primary immunodeficiency diseases (PIDDs), each associated with unique diagnostic and therapeutic complexities. Limited data, however, exist supporting specific therapeutic interventions. Thus, a survey of PIDD management was administered to allergists/immunologists in the United States to identify current perspectives and practices. Among 405 respondents, the majority of key management practices identified were consistent with existing data and guidelines, including the provision of immunoglobulin therapy, immunoglobulin dosing and selective avoidance of live viral vaccines. Practices for which there are little specific data or evidence-based guidance were also examined, including evaluation of IgG trough levels for patients receiving immunoglobulin, use of prophylactic antibiotics and recommendations for complementary/alternative medicine. Here, variability applied to PIDD patients was identified. Differences between practitioners clinically focused upon PIDD and general allergists/immunologists were also identified. Thus, a need for expanded clinical research in PIDD to optimize management and potentially improve outcomes was defined.

Use of granulocyte-macrophage colony-stimulating factor to reverse anergy in otherwise immunologically healthy children.

BACKGROUND: T-cell anergy, as measured by delayed hypersensitivity skin testing, is associated with increased susceptibility to infection. Because the repertoire of effects of granulocyte-macrophage colony-stimulating factor (GM-CSF) includes enhancement of antigen processing and presentation by antigen-presenting cells, GM-CSF has been used to augment immune function in human immunodeficiency virus-induced and other viral illness-induced immune dysfunction and to affect positively immune function in a wide variety of disorders. OBJECTIVE: To attempt reversal of T-cell anergy using GM-CSF in 3 otherwise immunologically healthy children with severe recurrent and persistent viral respiratory tract infections and in one child with recurrent bacterial sepsis. METHODS: After written informed consent and baseline data were obtained, the study participants were administered 3 two-week cycles of GM-CSF. Delayed hypersensitivity skin testing and laboratory tests were repeated 2 weeks after the third cycle and subsequently as clinically indicated. RESULTS: All 4 children developed delayed hypersensitivity by skin testing, and all demonstrated markedly decreased number and severity of infection. Improvement persisted in all patients for at least 1 year. A single cycle of additional treatment in 2 patients reestablished delayed hypersensitivity and decreased infection, both of which persisted throughout the follow-up period of 4 or more years. CONCLUSIONS: GM-CSF treatment reversed T-cell anergy in 4 children. Reestablishment of delayed hypersensitivity was associated with a significant decrease in infection. Although further studies will be needed, use of GM-CSF should be considered as an immune modulator in patients with T-cell anergy and recurrent infections.