A 60-year-old man with Waldenström's macroglobulinemia (WM) was admitted to our hospital for evaluation of rapid progressive renal deterioration despite 3 cycles of oral melphalan and prednisolone (MP) therapy. Renal biopsy just before introducing hemodialysis revealed cast nephropathy and severe tubulo-interstitial infiltration of B lymphocytes. After 6 cycles of rituximab, cyclophosphamide, vincristine and prednisolone (R-COP) therapy, his renal function improved enough to discontinue hemodialysis. This is a rare case of WM-related renal involvement caused by both monoclonal protein and tumor infiltration and, to our knowledge, the second report on improved renal function by rituximab-based therapy.
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http://dx.doi.org/10.2169/internalmedicine.51.7207 | DOI Listing |
Front Immunol
November 2024
Department of Internal Medicine, College of Medicine, Yonsei University, Seoul, Republic of Korea.
Acquired hemophilia A (AHA) is an autoimmune bleeding disorder that is caused by factor VIII (FVIII) autoantibodies with high morbidity and mortality due to bleeding and complications from immunosuppression (IST). To address the real-world implications of the FVIII mimetic antibody, emicizumab, and the role of IST, we retrospectively collected de-identified data on 62 patients with AHA who were treated off-label with emicizumab for a median of 10 weeks at 12 US-based hemophilia treatment centers. Most patients (95.
View Article and Find Full Text PDFSci Rep
September 2024
Department of Hematology/Hematological Lab, The Second Affiliated Hospital of Anhui Medical University, Hefei, Anhui, People's Republic of China.
Int Immunopharmacol
September 2024
Department of Nephropathy, the First Affiliated Hospital of Zhengzhou University, No. 1 East Jianshe Road, Zhengzhou 450052, Henan, China. Electronic address:
Thromb Res
September 2024
Division of Hematology, Department of Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok 10700, Thailand. Electronic address:
Acquired hemophilia A (AHA) presents a significant bleeding risk. Management involves bleeding control and immunosuppressive therapy (IST) to eliminate inhibitors. This study, encompassing a retrospective cohort of 76 newly diagnosed AHA patients (1997-2022), evaluated IST outcomes such as complete remission (CR), relapse, and mortality rates, alongside influencing factors.
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