Multiple myeloma presenting with retroperitoneal fibrosis and elevated IgG4
Kensuke Kayamori1,2, Katsuhiro Shono3, Masahiro Onoda3
1Department of Hematology, Chiba Aoba Municipal Hospital, 1273-2 Aoba-cho, Chuo-ku, Chiba, 260-0852, Japan. k.kaya0713@gmail.com.
International Journal of Hematology
|May 11, 2025
Summary
This case study highlights a patient with IgG4-related disease manifesting as retroperitoneal fibrosis, successfully treated with chemotherapy for multiple myeloma. It suggests a potential link between IgG4 myeloma and IgG4-related disease development.
Area of Science:
- Immunology
- Oncology
- Nephrology
Background:
- The precise role of immunoglobulin G4 (IgG4) in IgG4-related disease (IgG4-RD) pathogenesis is debated.
- It remains unclear if IgG4 actively drives disease or is a byproduct of immune responses.
Purpose of the Study:
- To present a unique case linking multiple myeloma with elevated IgG4 to IgG4-RD.
- To explore the relationship between IgG4 myeloma and clinical manifestations of IgG4-RD.
Main Methods:
- Case report of a 66-year-old male with asymptomatic multiple myeloma and elevated IgG4.
- Clinical observation of retroperitoneal fibrosis leading to hydronephrosis.
- Assessment of treatment response to chemotherapy for multiple myeloma.
Main Results:
- The patient developed retroperitoneal fibrosis, a manifestation of IgG4-RD, causing ureteral obstruction and hydronephrosis.
- Chemotherapy for multiple myeloma resulted in remission of both the myeloma and the IgG4-RD.
- This contrasts with prior reports of IgG4 myeloma patients lacking IgG4-RD symptoms.
Conclusions:
- This case suggests a potential direct link between IgG4 myeloma and the development of IgG4-related disease.
- It offers a new perspective on the interplay between these conditions and their treatment.
