关键词: 重链沉积病, 浆细胞病, 达雷妥尤单抗

Abstract: A middle-aged female patient presented with “intermittent bilateral lower extremity edema with facial edema for more than 7 months，nausea and vomiting for 2 days”. The clinical symptoms of this case were non-specific. The kidney injury showed proteinuria，microscopic hematuria，abnormal renal function，and hypocomplementeemia. The main pathological features were glomerular nodular sclerosis with mesangial proliferative glomerulonephritis. Immunofluorescence showed massive deposition of IgG1 and complement C3 in the mesangial region，and linear deposition along the glomerular vascular loops and the basement membrane of the renal tubules. Fine particles or fine fibril with a diameter of 18~20 nm could be seen in the mesangial region of the glomerulus. Serum M protein spectrum analysis showed the highest abundance of heavy chain γ，low relative abundance of light chain λ and κ. Heavy chain deposition disease was diagnosed with fibrous structure deposition. After bortezomib based treatment，renal function improved and hematologic partial remission was achieved. However，the disease recurred after drug withdrawal，continued bortezomib based treatment，and the disease did not remission，and then changed to daratumumab based treatment，and hematologic partial remission was achieved.

Key words: heavy chain deposition disease, plasmacytosis, daratumumab