Canan Caka, Ozlem Cimen, Pinar Kahyaoğlu, İlhan Tezcan, Deniz Cagdas
Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology 2021 AugSelective IgM deficiency (sIgMD) is classified under primary immunodeficiencies (PID). This study aimed to define the clinical and immunologic features of sIgMD. We assessed a retrospective medical record of patients who fulfilled the diagnostic criteria for sIgMD in a pediatric immunology department. There were 55 patients with sIgMD. Out of 55 patients, 13 (23.6%) patients, diagnosed with a well-defined PID disease, and nine, evaluated as transient hypogammaglobulinemia, were excluded in the follow-up. The ratio of the sIgMD was %0.12 in the outpatient clinic of pediatric immunology (33/27,000). Out of 33 patients, eight (24,2%) were asymptomatic during the follow-up period. Fifteen (45.4%) patients presented with upper/lower respiratory and skin 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's disease, Guillain-Barre syndrome, and diabetes mellitus. Five (15%) had allergic disorders. Three patients (9%) developed malignancy. The PID diagnoses were combined immunodeficiency, common variable immunodeficiency, chronic granulomatous disease, adenosine deaminase deficiency, and congenital neutropenia. Genetic disorders, autoimmune/inflammatory, and allergic diseases may accompany sIgMD. Approximately 25% of the patients were asymptomatic in our series. Patients had increased malignancy risk. We diagnosed about 25% of the patients having low IgM with a specific PID in the follow-up period. Thus, patients with sIgMD should be followed up regularly in immunology clinics. © 2021 EAACI and John Wiley and Sons A/S. Published by John Wiley and Sons Ltd.
Canan Caka, Ozlem Cimen, Pinar Kahyaoğlu, İlhan Tezcan, Deniz Cagdas. Selective IgM deficiency: Follow-up and outcome. Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology. 2021 Aug;32(6):1327-1334
PMID: 33706406
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