Successful treatment with daratumumab of a patient with monoclonal lambda light chain disease presenting as nephrotic syndrome and crescentic glomerulonephritis
The Author(s). Published by S. Karger AG, Basel..
Monoclonal immunoglobulin deposition disease (MIDD) are a group of systemic diseases, characterized by deposition of monoclonal immunoglobulin predominantly in the kidney. In the absence of overt hematologic disease, MIDD 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 include anti-plasma cell therapy. Here 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.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2024 |
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| Erschienen: |
2024 |
| Enthalten in: |
Zur Gesamtaufnahme - year:2024 |
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| Enthalten in: |
Acta haematologica - (2024) vom: 16. Jan. |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Bnaya, Alon [VerfasserIn] |
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| Links: |
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Date Revised 16.01.2024 published: Print-Electronic Citation Status Publisher |
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| doi: |
10.1159/000536283 |
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| funding: |
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| PPN (Katalog-ID): |
NLM367186497 |
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| 245 | 1 | 0 | |a Successful treatment with daratumumab of a patient with monoclonal lambda light chain disease presenting as nephrotic syndrome and crescentic glomerulonephritis |
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| 520 | |a The Author(s). Published by S. Karger AG, Basel. | ||
| 520 | |a Monoclonal immunoglobulin deposition disease (MIDD) are a group of systemic diseases, characterized by deposition of monoclonal immunoglobulin predominantly in the kidney. In the absence of overt hematologic disease, MIDD 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 include anti-plasma cell therapy. Here 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 | ||
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