Successful Treatment with Daratumumab of a Patient with Monoclonal Lambda Light Chain Disease Presenting as Nephrotic Syndrome and Crescentic Glomerulonephritis.

Abstract

Introduction: Monoclonal immunoglobulin deposition diseases (MIDDs) are a group of systemic diseases, characterized by deposition of monoclonal immunoglobulin predominantly in the kidney. In the absence of overt hematologic disease, MIDDs are classified as a part of monoclonal gammopathy of renal significance. Patients with MIDD may present with a nephrotic syndrome and kidney function impairment. Treatment usually includes anti-plasma cell therapy.

Case presentation: We report a case of a 54-year-old female who presented with nephrotic syndrome related to light chain deposition disease of lambda type. Due to a complicated clinical course (including cardiac injury and thromboembolic stroke), plasma cell-targeted therapy was stopped. A few months later, the patient presented with severe acute kidney injury. Kidney biopsy revealed crescentic glomerulonephritis, and immunofluorescence staining was positive for lambda chain. Treatment with daratumumab was initiated resulting in stabilization of kidney function and partial nephrotic syndrome remission.

Conclusion: This case highlights an uncommon histologic manifestation in a patient diagnosed with light chain deposition disease. Furthermore, it underscores the significance of plasma cell-targeted therapy and the favorable clinical and hematological response observed with daratumumab.