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Selective IgM deficiency (SIgMD) is a rare immune disorder that has been reported in association with serious infections, such as bacteria in the blood (bacteremia, also known as septicemia). Although SIgMD was first described in two children, the disorder can occur in babies, children, and adults. It is characterized by isolated absence or deficiency of immunoglobulin M (IgM), normal levels of other immunoglobulins, and recurrent infections (especially by Staphylococcus aureus, Streptococcus pneumoniae, Hemophilus influenza). The cause is still unclear. The diagnosis includes isolated deficiency ofIgM in the blood and no other immunodeficiency or secondary cause of low IgM. Patients with SIgMD and recurrent infections are managed like other antibody defects and deficiencies. It is suggested that people with SIgMD have pneumococcal and meningococcal vaccines, people with SIgMD who have recurrent infections should have prophylactic antibiotics and immune globulin replacement.
Biomarker and diagnostic research for selective IgM deficiency has been reported in the published literature.
Estimated prevalence: Unknown (Unknown prevalence).
No clinical trials have been registered for selective IgM deficiency.
37 publications have been identified in PubMed for selective IgM deficiency. Research spans Case Report / Case Series (28%), Review / Meta-Analysis (22%), and Epidemiology / Natural History (22%).
Research Type | Count | % of Total |
|---|---|---|
Patient case studies | 10 | 28% |
Data assembled from 3 of 12 sources · Last updated Sep 18, 2026, 4:32 PM UTC
European rare disease database
Genetic and Rare Diseases Info Center
8 |
22% |
Disease patterns and progression | 8 | 22% |
Clinical study results | 4 | 11% |
Testing and diagnosis research | 3 | 8% |
Laboratory research | 3 | 8% |
Justiz Vaillant AA (2026). [PMID: 29939682](https://pubmed.ncbi.nlm.nih.gov/29939682/). *Unknown Journal*. [Basic Science / Preclinical]
Naing A (2026). [PMID: 42103356](https://pubmed.ncbi.nlm.nih.gov/42103356/). *J Immunother Cancer*. [Clinical Trial Publication]
Lecký P (2026). [PMID: 41988554](https://pubmed.ncbi.nlm.nih.gov/41988554/). *Eur J Case Rep Intern Med*. [Case Report / Case Series]
Killeen RB (2026). [PMID: 30855793](https://pubmed.ncbi.nlm.nih.gov/30855793/). *Unknown Journal*. [Basic Science / Preclinical]
Yu U (2026). [PMID: 42081979](https://pubmed.ncbi.nlm.nih.gov/42081979/). *Transplant Cell Ther*. [Epidemiology / Natural History]
Khalaf L (2026). [PMID: 41618198](https://pubmed.ncbi.nlm.nih.gov/41618198/). *BMC Pediatr*. [Case Report / Case Series]
Justiz Vaillant AA (2026). [PMID: 29763203](https://pubmed.ncbi.nlm.nih.gov/29763203/). *Unknown Journal*. [Review / Meta-Analysis]
Gumusburun R (2026). [PMID: 41979700](https://pubmed.ncbi.nlm.nih.gov/41979700/). *J Clin Immunol*. [Epidemiology / Natural History]
Ozdemir E (2025). [PMID: 41428115](https://pubmed.ncbi.nlm.nih.gov/41428115/). *Immunol Res*. [Clinical Trial Publication]
Demirtaş Güner D (2025). [PMID: 40270942](https://pubmed.ncbi.nlm.nih.gov/40270942/). *Front Pediatr*. [Basic Science / Preclinical]