Light chain deposition disease with renal involvement: clinical characteristics and prognostic factors

2003 ◽  
Vol 42 (6) ◽  
pp. 1154-1163 ◽  
Author(s):  
Claudio Pozzi ◽  
Marco D'Amico ◽  
Giovanni B Fogazzi ◽  
Simona Curioni ◽  
Franco Ferrario ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Light Chain ◽  
Clinical Characteristics ◽  
Renal Involvement ◽  
Light Chain Deposition Disease ◽  
Deposition Disease ◽  
Light Chain Deposition

scholarly journals Clinical characteristics and prognostic factors in multiple myeloma patients with light chain deposition disease

2017 ◽  
Vol 92 (8) ◽  
pp. 739-745 ◽  
Author(s):  
Meera Mohan ◽  
Amy Buros ◽  
Pankaj Mathur ◽  
Neriman Gokden ◽  
Manisha Singh ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Prognostic Factors ◽  
Light Chain ◽  
Clinical Characteristics ◽  
Light Chain Deposition Disease ◽  
Deposition Disease ◽  
Light Chain Deposition

Light-chain deposition disease

2018 ◽  
Author(s):  
Dirk R. J. Kuypers ◽  
Morie A. Gertz
Keyword(s):  
Light Chain ◽  
Plasma Cells ◽  
Renal Involvement ◽  
Bone Marrow Examination ◽  
Therapeutic Monitoring ◽  
Light Chains ◽  
Monoclonal Immunoglobulin ◽  
Light Chain Deposition Disease ◽  
Deposition Disease ◽  
Light Chain Deposition

Light-chain deposition disease (LCDD) is characterized by extracellular tissue deposition of non-amyloid monoclonal immunoglobulin light chains (predominantly kappa light chains) in various organs including kidneys, heart, and liver. It is a rare cause of renal insufficiency. In two-thirds of cases it is associated with multiple myeloma, while in the remainder their monoclonal B cell proliferation does not meet the criteria for that diagnosis.Renal involvement occurs almost invariably and dominates the clinical course of the disease: greater than 90% of patients with LCDD have renal functional impairment; acute or rapidly progressive kidney failure usually develops over a period of months. Nephrotic-range proteinuria is present in 40–50% of patients while approximately 20% of patients develop nephrotic syndrome. Arterial hypertension and microscopic haematuria can be present. Extrarenal symptoms are related to affected organs with cardiomyopathy, cachexia, haemorrhages, infections, and MM progression as main causes of death.The diagnosis of LCDD is often delayed and whilst bone marrow examination will often identify associated MM, renal biopsy frequently provides the final diagnostic proof. Abnormal light chains can be detected and quantified by serum or urine protein electrophoresis and immunofixation. Quantification of urine and serum free kappa/lambda light chains has proven a useful screening tool and might also plays a role in therapeutic monitoring.Treatment consists of chemotherapy directed against the monoclonal immunoglobulin-producing plasma cells.

Induction treatment of light chain deposition disease with bortezomib: rapid hematological response with persistence of renal involvement

2011 ◽  
Vol 53 (2) ◽  
pp. 330-331 ◽  
Author(s):  
Jiri Minarik ◽  
Vlastimil Scudla ◽  
Tomas Tichy ◽  
Tomas Pika ◽  
Jaroslav Bacovsky ◽  
...  
Keyword(s):  
Light Chain ◽  
Renal Involvement ◽  
Induction Treatment ◽  
Light Chain Deposition Disease ◽  
Deposition Disease ◽  
Hematological Response ◽  
Light Chain Deposition

Recovery from LCDD-associated Severe Liver Cholestasis: a Case Report and Literature Review

2016 ◽  
Vol 25 (1) ◽  
pp. 99-103 ◽  
Author(s):  
Benoit Brilland ◽  
Johnny Sayegh ◽  
Anne Croue ◽  
Frank Bridoux ◽  
Jean-François Subra ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Light Chain ◽  
Intrahepatic Cholestasis ◽  
Relevant Literature ◽  
Gamma Glutamyl Transferase ◽  
Light Chain Deposition Disease ◽  
Deposition Disease ◽  
Uncommon Presentation ◽  
Light Chain Deposition ◽  
Glutamyl Transferase

Light chain deposition disease (LCDD) is a rare multisystemic disorder associated with plasma cell proliferation. It mainly affects the kidney, but liver and heart involvement may occur, sometimes mimicking the picture of systemic amyloidosis. Liver disease in LCDD is usually asymptomatic and exceptionally manifests with severe cholestatic hepatitis. We report the case of a 66-year-old female with κ-LCDD and cast nephropathy in the setting of symptomatic multiple myeloma who, after a first cycle of bortezomib-dexamethasone chemotherapy, developed severe and rapidly worsening intrahepatic cholestasis secondary to liver κ-light chain deposition. Intrahepatic cholestasis was attributed to LCDD on the basis of the liver histology and exclusion of possible diagnoses. Chemotherapy was maintained and resulted in progressive resolution of cholestasis. We report here an uncommon presentation of LCDD, with prominent liver involvement that fully recovered with bortezomib-based chemotherapy, and briefly review the relevant literature. Abbreviations: AKI: Acute kidney injury; ALP: alkaline phosphatase; ALT: alanine aminotransferase; AST: aspartate aminotransferase; CMV: Cytomegalovirus; EBV: Epstein–Barr virus; GGT: gamma-glutamyl transferase; HSV: Herpes simplex virus; LC: light chain; LCDD: Light chain deposition disease; MIDD: Monoclonal immunoglobulin deposition disease; MM: Multiple myeloma.

scholarly journals LUNG INVOLVEMENT WITH CYSTIC CHANGES IN THE SETTING OF LIGHT CHAIN DEPOSITION DISEASE

CHEST Journal ◽  
2020 ◽  
Vol 158 (4) ◽  
pp. A1638
Author(s):  
Yahya Almodallal ◽  
Vivek Iyer ◽  
Hasan Ahmad Hasan Albitar
Keyword(s):  
Light Chain ◽  
Lung Involvement ◽  
Light Chain Deposition Disease ◽  
Deposition Disease ◽  
Cystic Changes ◽  
Light Chain Deposition

scholarly journals Light Chain Deposition Disease Diagnosed Using Computed Tomography-Guided Kidney Biopsy

Cureus ◽  
2021 ◽  
Author(s):  
Yoshinosuke Shimamura ◽  
Yayoi Ogawa ◽  
Hideki Takizawa ◽  
Toshiaki Hayashi ◽  
Yasuo Sakurai
Keyword(s):  
Computed Tomography ◽  
Light Chain ◽  
Kidney Biopsy ◽  
Light Chain Deposition Disease ◽  
Deposition Disease ◽  
Light Chain Deposition

scholarly journals Complete Primary Sequences of Two λ Immunoglobulin Light Chains in Myelomas with Nonamyloid (Randall-Type) Light Chain Deposition Disease

1998 ◽  
Vol 153 (1) ◽  
pp. 313-318 ◽  
Author(s):  
Catherine Decourt ◽  
Guy Touchard ◽  
Jean-Louis Preud'homme ◽  
Ruben Vidal ◽  
Hélène Beaufils ◽  
...  
Keyword(s):  
Light Chain ◽  
Light Chains ◽  
Immunoglobulin Light Chains ◽  
Light Chain Deposition Disease ◽  
Deposition Disease ◽  
Light Chain Deposition
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