Immunoglobulin γ3-heavy-chain deposition disease:: Report of a case and relationship with hypocomplementemia -: art. no. e2

The authors describe a 54-year-old woman presenting with proteinuria, hematuria, and hypocomplementemia whose renal biopsy results showed diffuse increase in mesangial matrix and nodular formations in several glomuruli with the deposition of immunoglobulin gamma3-heavy-chain and complement components C1q and C3 in the glomeruli and on the tubular basement membranes, without associated light-chain deposits. Staining for the constant domains of gamma-heavy-chain showed a deletion of the first constant domain (CH1). These findings were consistent with those of gamma-heavy-chain deposition disease (gamma-HCDD). The patient was treated monthly with melphalan and prednisolone although a bone marrow aspirate did not show findings suggestive of plasmacytoma. Six courses of melphalan and prednisolone therapy resulted in a marked reduction of urinary protein excretion and marked rise of complement levels. The current case is the fourth HCDD patient reported featuring gamma3-heavy-chain deposition who showed severe hypocomplementemia and responded to chemotherapy with improved renal parameters and complement levels. A review of previously reported cases of HCDD showed that some but not all HCDD cases were associated with hypocomplementemia. The authors also discuss here the relationship of HCDD to hypocomplementemia.