(1) Long-term Reversal of Hypogammaglobulinemia in two Common Variable Immunodeficiency (CVID) Patients after Discontinuation of Intravenous Immunoglobulin (IVIG)

RATIONALE: Life long immunoglobulin supplementation is required to maintain a normal IgG in CVID patients. Normal immunoglobulins after discontinuation of IVIG in 2 adults is reported.METHODS: Chart review of 2 CVID patients who had normalization of their IgA and IgM observed were followed after stopping IVIG.RESULTS: Patient A 33 year old female presented with frequent sinopulmonary infections including pneumonias. Initial IgG, IgA, and IgM in mg/dL were 325, 0, and 0, normal B lymphocytes and Tcell subsets. She received IVIG for 22 years and when stopped 3 years ago, her IgG has been at 601 to 823 (mg/dL), her IgA and IgM at 188 to 265 (mg/dL) and 99.5 to 138 (mg/dL), with normal response to immunizations and no recurrent infections; Patient B 50 year old female presented with frequent cutaneous and sinopulmonary infections, dermatits and alopecia. She had reduced IgG 258, IgA 28.3 and IgM 13.4, lymphopenia with 130 CD4 cells, 30 CD8 cells, no NK cells but 220 B lymphocytes, no lymphocytoxic antibodies and depressed lymphocyte proliferation. She received IVIG for five years. Over the 6 years since discontinuation, her IgG has been 670 - 1310 (mg/dL) and IgA and IgM levels stayed at 233 to 627 (mg/dL) and 38.3 to 92 (mg/dL) with normal antibody vaccine responses. Recurrent infections, dermatits and alopecia resolved.No HIV infection, multiple myeloma, lymphoma, malignancy or any autoimmune disorders have developed.CONCLUSIONS: Hypogammaglobulinemia in some CVID patients may be reversible and measurements of IgA and IgM during therapy may detect this rare outcome. RATIONALE: Life long immunoglobulin supplementation is required to maintain a normal IgG in CVID patients. Normal immunoglobulins after discontinuation of IVIG in 2 adults is reported. METHODS: Chart review of 2 CVID patients who had normalization of their IgA and IgM observed were followed after stopping IVIG. RESULTS: Patient A 33 year old female presented with frequent sinopulmonary infections including pneumonias. Initial IgG, IgA, and IgM in mg/dL were 325, 0, and 0, normal B lymphocytes and Tcell subsets. She received IVIG for 22 years and when stopped 3 years ago, her IgG has been at 601 to 823 (mg/dL), her IgA and IgM at 188 to 265 (mg/dL) and 99.5 to 138 (mg/dL), with normal response to immunizations and no recurrent infections; Patient B 50 year old female presented with frequent cutaneous and sinopulmonary infections, dermatits and alopecia. She had reduced IgG 258, IgA 28.3 and IgM 13.4, lymphopenia with 130 CD4 cells, 30 CD8 cells, no NK cells but 220 B lymphocytes, no lymphocytoxic antibodies and depressed lymphocyte proliferation. She received IVIG for five years. Over the 6 years since discontinuation, her IgG has been 670 - 1310 (mg/dL) and IgA and IgM levels stayed at 233 to 627 (mg/dL) and 38.3 to 92 (mg/dL) with normal antibody vaccine responses. Recurrent infections, dermatits and alopecia resolved. No HIV infection, multiple myeloma, lymphoma, malignancy or any autoimmune disorders have developed. CONCLUSIONS: Hypogammaglobulinemia in some CVID patients may be reversible and measurements of IgA and IgM during therapy may detect this rare outcome.