New insights into the pathogenesis and treatment of heavy chain deposition disease

Abstract

Heavy chain deposition disease is defined by the presence of tissue deposits of truncated monoclonal Ig heavy chains, usually associated with an underlying plasma cell clone. In this issue of Kidney International, Bridoux et al. described the clinical, histologic, and molecular characterization of 15 patients with heavy chain deposition disease, which is the largest case series to date. Notable findings included the frequent presence of C3 deposits and hypocomplementemia, the uniform finding of truncated heavy chains with the deletion in the heavy chain constant region 1, and the common occurrence of an abnormal serum-free κ:λ ratio, despite the absence of light-chain tissue deposition. Importantly, this study showed that clinical outcomes are improved significantly with modern antiplasma cell therapies such as bortezomib.

Long‐term renal survival of γ3‐heavy‐chain deposition disease complicated by heart failure: A case report

“…Patel et al 6 . and small case series have reported that bortezomib can induce hematological responses and improve renal function in HCDD patients 12–14 . The mechanism by which bortezomib operates in HCDD is not yet fully comprehended, but it is believed to be associated with inhibiting plasma cell proliferation and reducing the levels of circulating monoclonal immunoglobulins.…”

“…11 Patel et al 6 and small case series have reported that bortezomib can induce hematological responses and improve renal function in HCDD patients. [12][13][14] The mechanism by which bortezomib operates in HCDD is not yet fully comprehended, but it is believed to be associated with inhibiting plasma cell proliferation and reducing the levels of circulating monoclonal immunoglobulins. In this report, we present a case of HCDD with renal and hematological involvement along with heart failure, successfully treated with bortezomib-based chemotherapy.…”