Monoclonal Insulin Autoimmune Syndrome Successfully Treated with Plasma Cell Directed Therapy
Abstract
Background Monoclonal insulin autoimmune syndrome (IAS) is a very rare disease characterized by severe attacks of hypoglycemia caused by circulating anti-insulin antibodies produced by a B-cell clone, usually clonal plasma cells. Method We present 2 female Norwegian patients with monoclonal IAS. The anti-insulin antibodies were quantified by immune precipitation and characterized using a 3-step manual in-house assay. Both patients received plasma cell directed therapy. Result The first patient received plasma cell directed therapy for a time-limited period and achieved a sustained clinical remission without detectable anti-insulin antibodies. The second patient receives continuous plasma cell directed therapy and is in clinical remission with low values of detectable anti-insulin antibodies. Conclusion Plasma cell directed therapy was effective and safe in our 2 cases of monoclonal IAS. We recommend considering plasma cell directed therapy for these patients.
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The treatment plan for severe IAS involved bortezomib, lenalidomide, and dexamethasone for three-five cycles, followed by autologous stem cell transplant in one case and daratumumab as maintenance in the other.
“The treatment plan involved the use of bortezomib, lenalidomide, and dexamethasone for three-five cycles, followed by an autologous stem cell transplant in one case and daratumumab as maintenance therapy in the other case.”
From Non-Diabetic Hypoglycemia: Evaluation and Management in Adults (2025) · cites this paper as [78]
Askeland and colleagues describe two cases of successful treatment of IAS where patients remained in remission more than 3 years post therapy.
“Askeland and colleagues describe two cases of successful treatment of IAS where patients remained in remission more than 3 years post therapy [78].”
From Non-Diabetic Hypoglycemia: Evaluation and Management in Adults (2025) · cites this paper as [78]
Cites
Prevalence of monoclonal gammopathy of undetermined significance
Kyle, R. A., Therneau, T. M., Rajkumar, S. V., Larson, D. R., Plevak, M. F., Offord, J. R., Dispenzieri, A., Katzmann, J. A., & Melton, L. J. (2006) · New England Journal of Medicine · doi:10.1056/nejmoa054494 · Cited by 1,423 · see citing works
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Non-Diabetic Hypoglycemia: Evaluation and Management in Adults
Looi, E., & Lawler, H. M. (2025) · Journal of Clinical Medicine · doi:10.3390/jcm14134393 · Cited by 10 · see citing works
“Askeland and colleagues describe two cases of successful treatment of IAS where patients remained in remission more than 3 years post therapy [78].”
- Authors
- Askeland, F. B., Frøen, H. M., Bolstad, N., Thorsby, P. M., Schjesvold, F., Wammer, A. C. P., Følling, I., & Tjønnfjord, G. E.
- Affiliations
- Oslo University Hospital (NO)
- Akershus University Hospital (NO)
- University of Oslo (NO)
- Corresponding author
- Askeland, Frida Bugge
- Tjønnfjord, Geir E.
- ORCID
- Venue
- Clinical Lymphoma Myeloma and Leukemia
- Publisher
- Elsevier BV
- Published
- 2025-03
- Volume
- 25
- Issue
- 3
- Pages
- e127-e130
- ISSN
- 2152-2650 (ISSN-L)
- OpenAlex
- W4403285203
- Cited by
- 2
- References
- 1
- Citations by year
- 2026: 1 · 2025: 1
- FWCI
- 0.62
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Clinical Lymphoma Myeloma & Leukemia (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Hematology › Multiple Myeloma Research and Treatments
- Health Sciences › Medicine › Neurology › Autoimmune Neurological Disorders and Treatments
- Life Sciences › Immunology and Microbiology › Immunology › Immunodeficiency and Autoimmune Disorders
- Keywords
- insulin antibodies, hypoglycemia, B cell clones, clonal plasma cells, clinical remission, antibody quantification
- MeSH terms
- Adult, Aged, Antibodies, Monoclonal, Autoimmune Diseases, Female, Humans, Insulin, Insulin Antibodies, Middle Aged, Plasma Cells, Syndrome, Treatment Outcome
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