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Selective IgM deficiency

INTRODUCTION

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 immune deficiencies: An overview".)

DEFINITION

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, Hemophilus influenza), and viral infections

                                

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Literature review current through: Jul 2014. | This topic last updated: May 12, 2014.
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