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Selective IgM deficiency

Guha Krishnaswamy, MD, FACP, FCCP, FACAAI, FAAAI, CC-D
Section Editor
Luigi D Notarangelo, MD
Deputy Editor
Anna M Feldweg, MD


Selective immunoglobulin M deficiency (sIgMD) is a rare immune disorder that has been reported in association with serious infections, such as bacteremia. The disorder can occur in infants, children, and adults. Cumulatively, there have been fewer than 300 cases reported in the literature, and understanding of this condition is therefore preliminary. This topic review discusses the normal immunophysiology of IgM, theories of pathogenesis, and the clinical features, diagnosis, and treatment of sIgMD. More general discussions of antibody deficiency are found separately. (See "Primary humoral immunodeficiencies: An overview".)


Selective immunoglobulin M deficiency (sIgMD) is characterized by the following clinical and laboratory features:

Isolated absence or deficiency of IgM

Normal levels of other immunoglobulins (notably immunoglobulin G [IgG] and immunoglobulin A [IgA])

Recurrent infections from infancy onwards, associated with susceptibility to Staphylococcus aureus, encapsulated pathogens (Streptococcus pneumoniae, Haemophilus influenzae), and viral infections

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Literature review current through: Nov 2017. | This topic last updated: Jul 26, 2017.
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