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Selective IgM deficiency

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Disease definition

A rare primary immunodeficiency characterized by recurrent and/or invasive bacterial, viral, and fungal infections, associated with low to absent blood IgM levels, while IgG, IgG subclasses, and IgA levels, as well as IgG antibody response to vaccinations, are normal. Patients may also present allergic diatheses, and the prevalence of autoimmune diseases is increased.

ORPHA:331235

Classification level: Disorder

Synonym(s):
  • Selective immunoglobulin M deficiency

Prevalence: Unknown

Inheritance: -

Age of onset: All ages

ICD-10: D80.4

ICD-11: 4A01.04

UMLS: C0154275

GARD: 12547

MedDRA: 10039916

A summary on this disease is available in Français, Español, Deutsch, Italiano, Nederlands
Detailed information

Logo ERN: produced/endorsed by ERN(s) Logo FSMR: produced/endorsed by FSMR(s)

Guidelines
Emergency guidelines
Français (2020.pdf) - Orphanet Urgences
Clinical practice guidelines
The documents contained in this website are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.