Which lab findings are characteristic of X-linked immunodeficiency with hyper IgM syndrome (XHM)?

Updated: Aug 11, 2020
  • Author: Francisco J Hernandez-Ilizaliturri, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

XHM:

  • IgM levels are markedly increased, frequently to higher than 1000 mg/dL. Note: Normal levels do not exclude the diagnosis; in one \study, normal levels were present in 29 of 55 patients with genetically proven XHM. [17]
  •  IgG, IgA, and IgE levels and the number of lymphocytes bearing these antibodies are decreased.
  • An IgM response to antigen exposure is possible, but the IgG and IgA responses are absent or diminished.
  • Cell-mediated immunity is defective in some patients despite a normal T-lymphocyte count.
  • Chronic neutropenia may be present in some patients.

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