Long-term renal survival of γ3-heavy-chain deposition disease complicated by heart failure: A case report.
Clin Case Rep
; 12(7): e9091, 2024 Jul.
Article
en En
| MEDLINE
| ID: mdl-38962462
ABSTRACT
Key Clinical Message Heavy-chain deposition disease (HCDD), a rare monoclonal immunoglobulin deposition disease, involves truncated heavy-chain deposition in kidneys. Limited long-term data exist. We report a case of renal and cardiac failure with favorable outcomes post bortezomib-based therapy. Stable renal function observed over 4 years suggests efficacy in HCDD with multisystem involvement. Abstract Heavy-chain deposition disease (HCDD) is an extremely rare form of monoclonal immunoglobulin deposition disease (MIDD) that involves the deposition of truncated immunoglobulin heavy chains in the kidneys. Only a few cases of HCDD with a favorable long-term renal prognosis have been reported, resulting in limited long-term follow-up data for this patient population. In this report, we present the case of a 52-year-old patient with nephrotic syndrome who experienced renal failure and cardiac failure. Renal biopsy confirmed the presence of γ3-HCDD and monoclonal Immunoglobulin G (IgG)κ in the serum. The patient exhibited low voltage on electrocardiogram (ECG) and unexplained left ventricular hypertrophy on cardiac ultrasound. The patient underwent eight cycles of bortezomib-based chemotherapy, which led to hematological remission. After 4 years of follow-up, the patient's renal function remained stable, with serum creatinine levels ranging from 0.7 to 0.9 mg/dL and proteinuria of 0.3-0.5 g/24 h. Our findings suggest that bortezomib-based chemotherapy is equally effective in HCDD patients with combined multisystem damage.
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Colección:
01-internacional
Base de datos:
MEDLINE
Idioma:
En
Revista:
Clin Case Rep
Año:
2024
Tipo del documento:
Article
Pais de publicación:
Reino Unido