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SELECTIVE IGM DEFICIENCY: FOLLOW-UP AND OUTCOME
  • +2
  • Canan Caka,
  • özlem satırer,
  • pınar kahyaoglu,
  • İlhan Tezcan,
  • Deniz Cagdas
Canan Caka
Hacettepe University
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özlem satırer
Hacettepe University
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pınar kahyaoglu
Hacettepe University
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İlhan Tezcan
Hacettepe Universitesi Tip Fakultesi
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Deniz Cagdas
Hacettepe University
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Abstract

Background: Selective IgM deficiency (sIgMD), classified under primary immunodeficiencies, is characterized by low serum IgM(<2SD for age), normal IgG and IgA levels. The aim of this study was to define immunologic and clinical features of sIgMD. Method: We assessed a retrospective medical record of patients who fullfilled the criteria for sIgMD in a Pediatric Immunology department. Results: There were 55 patients with sIgMD. Out of 55 patients, thirteen(23.6%) diagnosed with a well-defined primary immunodeficiency (PID) during the follow-up.The ratio of the sIgMD was %0.12 in the out-patient clinic of pediatric immunology. Out of 33 patients, 8(23.5%) were asymtomatic during the follow-up period. Fifteen(45.4%) patients presented with several type of infections). Six patients (18%) had chromosomal anomaly, or syndrome (trisomy 21, 22q11.2 deletion 1p deletion, CHARGE syndrome, and Cohen Syndrome). Six (18%) had autoimmune/inflammatory diseases, such as Behcet’s disease, immune thrombocytopenic purpura, Crohn disease, Guillain Barre syndrome, and diabetes mellitus. Five(15%) had allergic disorders. Three(9%) have developed malignancy. The diagnoses of thirteen PID patients were combined immunodeficiency, common variable immunodeficiency, autoimmune lymphoproliferative syndrome, chronic granulomatous disease, adenosine deaminase deficiency, and congenital neutropenia. Genetic disorders, autoimmune/inflammatory and allergic diseases may accompany sIgMD. Approximately one third of the patients were asymptomatic in our series. Malignancy risk is relatively increased. We observed that an important ratio of patients with low IgM (23.6%) got sooner the diagnosis of a specific PID in the follow-up period. Conclusion: Thus, patients with sIgMD should be followed regularly in immunology clinics.

Peer review status:Published

12 Oct 2020Submitted to Pediatric Allergy and Immunology
19 Oct 2020Reviewer(s) Assigned
24 Nov 2020Review(s) Completed, Editorial Evaluation Pending
25 Nov 2020Editorial Decision: Revise Major
16 Jan 20211st Revision Received
25 Jan 2021Review(s) Completed, Editorial Evaluation Pending
25 Jan 2021Reviewer(s) Assigned
10 Feb 2021Editorial Decision: Revise Minor
14 Feb 20212nd Revision Received
15 Feb 2021Review(s) Completed, Editorial Evaluation Pending
15 Feb 2021Editorial Decision: Accept
11 Mar 2021Published in Pediatric Allergy and Immunology. 10.1111/pai.13497