#3922 CLINICOPATHOLOGICAL CHARACTERISTICS OF LIGHT AND HEAVY CHAIN DEPOSITION DISEASE: A CASE SERIES FROM A SINGLE CENTER

Abstract Background and Aims Light heavy chain deposition disease (LHCDD) is the rarest form of monoclonal immunoglobulin (MIDD) characterized by light chain. At present, large-scale clinical research data LHCDD limited. We explore clinical-pathologic characteristics outcome in a single center China, to obtain better understanding this disease. Method reviewed 65291 native kidney biopsies from January 2008 December 2022. Ten patients with biopsy-proven diagnosed were studies retrospectively. Results 4 males 6 females, an average age 52.5 ± 8.9 years enrolled. The presented hypertension (80%), anemia (90%), elevated serum creatinine (70%), proteinuria (100%), nephrotic syndrome (30%) microscopic hematuria (90%). Serum immunofixation electrophoresis showed that 8 (80%) positive for (IgG-κ/IgG-λ/IgA-λ: 3/2/3). free (FLC) ratio was abnormal 9 (90%) patients. Among them, 5 had Elevated FLC (κ:λ > 1.65), < 0.26 observed Two multiple myeloma. Histologically, nodular mesangial sclerosis identified Immunofluorescence patientshad depositions IgG (γ1-κ/γ1-λ/γ2-κ/γ2-λ/γ3-κ:2/1/1/1/1), IgA (α-κ/α-λ:1/3). During median 28 months follow-up (range, 6–104 months) patients, received chemotherapy. Three (33%) complete hematologic remission (CR), 2 (22%) very good partial response (VGPR) or (PR), (44%) no (NR). One (11%) stable/improved renal function, (67%) worsening 3 progressed ESRD. All survived. Conclusion In case series tissues most frequent IgG-κ, followed IgA-λ, IgG-λ IgA-κ. More than half but poor prognosis.