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Puzzled by the lack of family history and adult onset of symptoms in most, the name \u201cacquired\u201d hypogammaglobinemia was given, but later altered to the current name common variable immune deficiency. Pathology reports remarked on the loss of lymph node architecture and paucity of plasma cells in lymphoid tissues in these subjects. While characterized by reduced serum IgG and IgA and often IgM, and thus classified among the B\u2010cell defects, an increasing number of cellular defects in these patients have been recognized over time. In the early years, severe respiratory tract infections commonly led to a shortened life span, but the wide spread availability of immune globulin concentrates for the last 25\u00a0years has improved survival. However, chronic non\u2010infectious inflammatory and autoimmune conditions have now emerged as challenging clinical problems; these require further immunologic understanding and additional therapeutic measures. Recent study of this phenotypic syndrome have provided an increasingly fertile ground for the identification of autosomal recessive and now more commonly, autosomal dominant gene defects which lead to the loss of B\u2010cell development in this syndrome.<\/jats:p>","DOI":"10.1111\/imr.12728","type":"journal-article","created":{"date-parts":[[2018,12,19]],"date-time":"2018-12-19T01:12:24Z","timestamp":1545181944000},"page":"145-161","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":83,"title":["Common variable immune deficiency: Dissection of the variable"],"prefix":"10.1111","volume":"287","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0725-0320","authenticated-orcid":false,"given":"Charlotte","family":"Cunningham\u2010Rundles","sequence":"first","affiliation":[{"name":"Division of Clinical Immunology Icahn School of Medicine at Mount Sinai  New York New York"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2018,12,18]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.9.6.722"},{"key":"e_1_2_11_3_1","first-page":"200","article-title":"Agammaglobulinemia","volume":"66","author":"Janeway CA","year":"1953","journal-title":"Trans Assoc Am Physicians"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM195406172502403"},{"key":"e_1_2_11_5_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM195502172520702"},{"key":"e_1_2_11_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9343(55)90376-4"},{"key":"e_1_2_11_7_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM195502172520703"},{"issue":"10","key":"e_1_2_11_8_1","first-page":"611","article-title":"Gamma globulin deficiency; report of a case and survey of the literature","volume":"57","author":"Greenhouse AH","year":"1956","journal-title":"J Kans Med Soc"},{"key":"e_1_2_11_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9343(54)90448-9"},{"issue":"2","key":"e_1_2_11_10_1","first-page":"162","article-title":"The role of immature plasma cells, lymphoblasts, and lymphocytes in the formation of antibodies, as established in tissue culture experiments","volume":"36","author":"Keuning FJ","year":"1950","journal-title":"J Lab Clin Med"},{"key":"e_1_2_11_11_1","doi-asserted-by":"publisher","DOI":"10.1038\/205143a0"},{"issue":"5","key":"e_1_2_11_12_1","first-page":"927","article-title":"Primary immunodeficiencies. 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