Clinical and Immunological Features in IgM Deficiency

Abstract

Background: IgM deficiency is a dysgammaglobulinemia characterized by isolated low levels of serum IgM. Patients with IgM deficiency may exhibit various clinical manifestations. However, IgM deficiency still remains to be explored with regard to diagnosis and treatment. Methods: Through a retrospective chart review, we investigated the clinical and immunological features of 15 symptomatic adult IgM-deficient patients who were referred to our immunology clinics over a 4-year period. Results: The patients were comprised of 6 males and 9 females, with a mean age of 57.2 years. On initial evaluation, 12 patients (80%) presented with susceptibility to infections, 5 (33%) had atopic manifestations such as asthma and allergic rhinitis, 3 (20%) had both infections and atopy, 4 patients (28%) had fibromyalgia-like symptoms, 3 (20%) had autoimmune manifestations, and 1 patient had lymphoma. The mean serum IgM level was 27.4 mg/dl (range 14–39). Impaired specific antibody response to pneumococcal antigens in 5 out of 11 studied patients (45%) appeared to be a notable association. Subtle abnormalities in IgG subclasses, lymphocyte subsets and in vitro proliferative lymphocyte responses were observed. Five patients who were treated with intravenous immunoglobulin responded very well. Conclusion: We propose that a thorough immunological evaluation including specific antibody responses be undertaken in patients with IgM deficiency. IgM-deficient patients who present with recurrent/severe infections may benefit from immunoglobulin treatment particularly in the presence of impaired pneumococcal antibody responses.

Selective IgM Deficiency with T Cell Defects and Mycobacterium Avium Complex (MAC) Infection

“…The most common presentation is recurrent and chronic upper and lower respiratory tract infections and sepsis [2][3][4][5]23]. B cells, T cells and T cell subsets, and NK cells are normal [5,15,[21][22][23][24]. T cell functions are normal [5,15].…”

“…B cells, T cells and T cell subsets, and NK cells are normal [5,15,[21][22][23][24]. T cell functions are normal [5,15]. Innate immune functions are normal [15].…”

“…T cell functions are normal [5,15]. Innate immune functions are normal [15]. Specific antibody response to pneumococci is impaired in 50% of symptomatic cases [15].…”

“…Patients with selective IgM deficiency have been reported to have normal proliferative response to mitogens and antigens [15]. Proliferative response to mitogens (PHA, ConA, PWM) and antigens (mumps, Candida albicans, and tetanus toxoid) were markedly decreased in all three patients; none of the patients responded to purified protein derivative (PPD).…”

“…Recently, we reported that adult patients with selective primary IgM deficiency and recurrent pyogenic bacterial infections display relatively normal T cell functions [15]. Here we report an extensive immunological analysis of three patients with primary selective IgM deficiency who presented *Address correspondence to this author at the Medical Sciences I, C-240, University of California at Irvine, Irvine, CA 92-697, USA; Tel: (949) 824-5818; Fax: (949) 824-4362; E-mail: sgupta@uci.edu with atypical mycobacterial infection, and had significant T cell defects.…”