eMedicine Specialties > Allergy and Immunology > Immunodeficiencies

Immunoglobulin M Deficiency: Differential Diagnoses & Workup

Author: Iftikhar Hussain, MD, Director of Allergy, Asthma, and Immunology Center, PC
Coauthor(s): Srividya Sridhara, MD, Resident Physician, Department of Internal Medicine, University of Oklahoma; Bilal Ahmed, MD, MBBS, Clinical Research Assistant, Allergy, Asthma and Immunology Center, PC
Contributor Information and Disclosures

Updated: Jul 21, 2009

Differential Diagnoses

DiGeorge Syndrome
Hypocomplementemia
Hypogammaglobulinemia
Immunoglobulin A Deficiency
Immunoglobulin G Deficiency
Wiskott-Aldrich Syndrome

Other Problems to Be Considered

Common variable immunodeficiency
Defects in B lymphocytes and T lymphocytes

Wiskott-Aldrich syndrome (WAS) also presents with low levels of IgM, but levels of IgA and IgE are often elevated. WAS is an X-linked disorder manifesting in infancy with eczema, thrombocytopenia, and recurrent pyogenic infections.

A case report of 37-year-old female presenting with recurrent pneumonia associated with a bronchial polyp and dermal symptoms found to have low levels of IgM and IgG4 was reported.9 Subclass deficiencies might be considered in patients with IgM deficiency.

Workup

Laboratory Studies

  • Quantitative levels of IgM, IgG, IgA, and IgE, along with IgG subclasses, are measured. This is done to exclude more common immunodeficiency disorders, such as common variable immunodeficiency and IgA deficiency. Particularly in adults, serum immunoelectrophoresis should be done to rule out the possibility of a concomitant clonal B-cell disorder such as multiple myeloma or chronic lymphocytic leukemia.
  • The biological significance of a seemingly isolated low IgM level may be assessed by immunizing the patient with protein (eg, tetanus) and polysaccharide (eg, unconjugated Streptococcus pneumoniae) vaccines and assessing whether antigen-specific IgG responses are normal.

Imaging Studies

  • Imaging studies are not required routinely for SIgM deficiency. However, if patients report a history of recurrent sinopulmonary infection, CT scans of the sinuses and chest may be helpful for diagnosing chronic sinusitis and bronchiectasis, respectively.

Other Tests

  • Flow cytometry for B cells with surface IgM may be helpful.
  • Other tests are not required routinely for SIgM deficiency. However, other tests may be needed for further evaluation of associated diseases.

Procedures

  • Diagnostic procedures are not routinely required for SIgM deficiency. They may be needed for diagnosis of infections, complications, or associated diseases.

More on Immunoglobulin M Deficiency

Overview: Immunoglobulin M Deficiency
Differential Diagnoses & Workup: Immunoglobulin M Deficiency
Treatment & Medication: Immunoglobulin M Deficiency
Follow-up: Immunoglobulin M Deficiency
References
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References

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  2. Goldstein MF, Goldstein AL, Dunsky EH, Dvorin DJ, Belecanech GA, Shamir K. Selective IgM immunodeficiency: retrospective analysis of 36 adult patients with review of the literature. Ann Allergy Asthma Immunol. Dec 2006;97(6):717-30. [Medline].

  3. Goldstein MF, Goldstein AL, Dunsky EH, Dvorin DJ, Belecanech GA, Shamir K. Pediatric selective IgM immunodeficiency. Clin Dev Immunol. 2008;2008:624850. [Medline].

  4. Ballow M. Primary immunodeficiency disorders: antibody deficiency. J Allergy Clin Immunol. 2002;109:581-91.

  5. Ohno T, Inaba M, Kuribayashi K. Selective IgM deficiency in adults: phenotypically and functionally altered profiles of peripheral blood lymphocytes. Clin Exp Immunol. Jun 1987;68(3):630-7. [Medline].

  6. De la Concha EG, Garcia-Rodriguez MC, Zabay JM. Functional assessment of T and B lymphocytes in patients with selective IgM deficiency. Clin Exp Immunol. Sep 1982;49(3):670-6. [Medline].

  7. Inoue T, Okumara Y, Shirahama M, et al. Selective partial IgM deficiency: Functional assessment of T and B lymphocytes in vitro. J Clin Immunol. 1986;6(2):130-5.

  8. Hong R, Gupta S. Selective immunoglobulin M deficiency in an adult with Streptococcus pneumoniae sepsis and invasive aspergillosis. J Investig Allergol Clin Immunol. 2008;18(3):214-8. [Medline].

  9. Ideura G, Agematsu K, Komatsu Y, Hatayama O, Yasuo M, Tsushima K, et al. Selective IgM Deficiency Accompanied with IgG4 Deficiency, Dermal Complications and a Bronchial Polyp. Allergol Int. Mar 2008;57(1):99-105. [Medline].

  10. Belgemen T, Suskan E, Dogu F, Ikinciogullari A. Selective Immunoglobulin M Deficiency Presenting with Recurrent Impetigo: A Case Report and Review of the Literature. Int Arch Allergy Immunol. Feb 12 2009;149(3):283-288. [Medline].

  11. Antar M, Lamarche J, Peguero A, Reiss A, Cole S. A case of selective immunoglobulin M deficiency and autoimmune glomerulonephritis. Clin Exp Nephrol. Aug 2008;12(4):300-4. [Medline].

  12. Makay B, Unsal E, Anal O, Gunes D, Men S, Cakmakci H, et al. Chronic recurrent multifocal osteomyelitis in a patient with selective immunoglobulin M deficiency. Rheumatol Int. May 2009;29(7):811-815. [Medline].

  13. Yamasaki T. Selective IgM deficiency: functional assessment of peripheral blood lymphocytes in vitro. Intern Med. Jul 1992;31(7):866-70. [Medline].

  14. Herrod, HG. Common variable hypogammaglobulinemia and other humoral immune deficiencies. [Full Text].

  15. Iraji F, Faghihi G. Epidermodysplasia verruciformis: association with isolated IgM deficiency and response to treatment with acitretin. Clin Exp Dermatol. Jan 2000;25(1):41-3. [Medline].

  16. Gul U, Soylu S, Yavuzer R. Epidermodysplasia verruciformis associated with isolated IgM deficiency. Indian J Dermatol Venereol Leprol. Nov-Dec 2007;73(6):420-2. [Medline].

  17. Zaka-ur-Rab Z, Gupta P. Pseudomonas septicemia in selective IgM deficiency. Indian Pediatrics. 2005;42:961-2.

  18. Stagi S, Azzari C, Bindi G, et al. Undetectable serum IgA and low IgM concentration in children with congenital hypothyroidism. Clin Immunol. 2005;116:94-8.

  19. Lim SH, Zhang Y, Wang Z, et al. Rituximab administration following autologous stem cell transplantation for multiple myeloma is associated with severe IgM deficiency. Blood. 2004;103:1971-2.

  20. Al-Herz W, McGeady SJ, Gripp KW. 22q11.2 deletion syndrome and selective IgM deficiency: an association of a common chromosomal abnormality with a rare immunodeficiency. Am J Med Genet. 2004;127A:99-100.

  21. Kung SJ, Gripp KW, Stephan MJ, Fairchok MP, McGeady SJ. Selective IgM deficiency and 22q11.2 deletion syndrome. Ann Allergy Asthma Immunol. Jul 2007;99(1):87-92. [Medline].

  22. Fallon KE. Inability to train, recurrent infection, and selective IgM deficiency. Clin J Sport Med. 2004;14:357-9.

  23. Faulk WP, Kiyasu WS, Cooper MD. Deficiency of IgM. Pediatrics. Feb 1971;47(2):399-404. [Medline].

  24. Guill MF, Brown DA, Ochs HD. IgM deficiency: clinical spectrum and immunologic assessment. Ann Allergy. Jun 1989;62(6):547-52. [Medline].

  25. Ideura G, Agematsu K, Komatsu Y, et al. Selective IgM deficiency accompanied with IgG4 deficiency, dermal complications and a bronchial polyp. Allergol Int. Mar 2008;57(1):99-105. [Medline].

  26. Nies KM, Stevens RH, Louie JS. Impaired immunoglobulin M synthesis by peripheral blood lymphocytes in systemic lupus erythematosus: a primary B-cell defect. Clin Immunol Immunopathol. Apr 1981;19(1):118-30. [Medline].

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Further Reading

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Keywords

immunoglobulin M deficiency, IgM deficient, selective IgM deficiency, SIgMD, hypogammaglobulinemia, IgM, IgM deficiency, DiGeorge syndrome, hypocomplementemia, hypogammaglobulinemia, IgA deficiency, IgG deficiency, Wiskott-Aldich syndrome, IgG reference range, Ig reference ranges, common variable immunodeficiency

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Contributor Information and Disclosures

Author

Iftikhar Hussain, MD, Director of Allergy, Asthma, and Immunology Center, PC
Iftikhar Hussain, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American College of Allergy, Asthma and Immunology, American College of Physicians, American Thoracic Society, and Association of Clinical Research Professionals
Disclosure: Nothing to disclose.

Coauthor(s)

Srividya Sridhara, MD, Resident Physician, Department of Internal Medicine, University of Oklahoma
Disclosure: Nothing to disclose.

Bilal Ahmed, MD, MBBS, Clinical Research Assistant, Allergy, Asthma and Immunology Center, PC
Disclosure: Nothing to disclose.

Medical Editor

Melvin Berger, MD, PhD, Adjunct Professor of Pediatrics and Pathology, Case Western Reserve University; Senior Medical Director, Clinical Research and Development, CSL Behring, LLC
Melvin Berger, MD, PhD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American Association of Immunologists, American Pediatric Society, American Society for Clinical Investigation, and Clinical Immunology Society
Disclosure: CSL Behring Salary Employment

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Michael R Simon, MD, MA, Clinical Professor Emeritus, Departments of Internal Medicine and Pediatrics, Wayne State University School of Medicine; Adjunct Staff, Division of Allergy and Immunology, Department of Internal Medicine, William Beaumont Hospital
Michael R Simon, MD, MA is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American College of Allergy, Asthma and Immunology, American College of Physicians, American Federation for Medical Research, Michigan Allergy and Asthma Society, Michigan State Medical Society, Royal College of Physicians and Surgeons of Canada, and Society for Experimental Biology and Medicine
Disclosure: Secretory IgA, Inc. Ownership interest Board membership

CME Editor

Timothy D Rice, MD, Associate Professor, Departments of Internal Medicine and Pediatrics and Adolescent Medicine, Saint Louis University School of Medicine
Timothy D Rice, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Physicians
Disclosure: Nothing to disclose.

Chief Editor

Michael A Kaliner, MD, Clinical Professor of Medicine, George Washington University School of Medicine; Chief, Section of Allergy and Immunology, Washington Hospital Center; Medical Director, Institute for Asthma and Allergy
Michael A Kaliner, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Association of Immunologists, American College of Allergy, Asthma and Immunology, American Society for Clinical Investigation, American Thoracic Society, and Association of American Physicians
Disclosure: Abbott Consulting fee Consulting; Alcon Consulting fee Consulting; Glaxo Consulting fee Consulting; Greer Consulting fee Consulting; Sanofi Consulting fee Consulting; Schering Consulting fee Consulting; Teva  Consulting; Meda Honoraria Speaking and teaching

 
 
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