Laboratory Studies

The laboratory workup for light-chain deposition disease (LCDD) includes the following:

Routine serum chemistries to evaluate liver and kidney function
Complete blood cell (CBC) count to assess for anemia
Serum and urine electrophoresis with immunofixation to evaluate for the presence of a monoclonal protein
Repeated studies of serum and urine protein immunoelectrophoresis (PEL) with immunofixation (IFE), to demonstrate the presence of monoclonal protein (in a minority of patients, a monoclonal protein may never be found in either serum or urine)
Quantitative serum assays for immunoglobulin free light chains: The introduction of this test has increased the sensitivity of laboratory tests to identify monoclonal gammopathies. ^{[38, 39]}

The most recent diagnostic screening recommendations are serum PEL with IFE and quantitative serum assays for free light chains.^[40]Including urine PEL and IFE when screening for amyloid light-chain (AL)–amyloidosis or LCDD is also recommended.^{[18, 38]}When all three are combined, the sensitivity for LCDD is 83%; it decreases to 77.8% when urine PEL with IFE is omitted. Excluding the quantitative serum assays for free light chains also decreases the sensitivity for the detection of LCDD.

Sometimes these tests can still miss the presence of a monoclonal protein, so kidney biopsy is needed for adequate and timely diagnosis.^{[12, 41]}Tissue should be sent for immunohistologic analysis for confirmation; results supporting a diagnosis of LCDD will be Congo Red negative with monoclonal light chain deposition. Light-chain restriction analyses confirm the presence of monoclonal light or heavy chains.

Imaging Studies

Echocardiography and ultrasonography of the abdomen should be performed. Other studies that are performed as needed include the following^[42]:

Nerve conduction studies
Computed tomography (CT)
Magnetic resonance imaging (MRI)
Positron emission tomography (PET)

Procedures

Biopsy procedures are as follows:

Biopsy of the specific organ suspected of involvement, with immunohistochemical staining for kappa and lambda chains
Rectal biopsy or subcutaneous fat aspiration to exclude amyloidosis
Bone marrow aspiration and biopsy to rule out multiple myeloma

Histologic Findings

The electron microscopic changes characteristic of light-chain deposition disease (LCDD) are finely granular, electron-dense deposits in the mesangial nodules and along the endothelial aspect of the glomerular basement membrane.^{[22, 23, 41]}In cases of tubular involvement, the deposits are noted along the outer aspect of the membrane.

Treatment & Management

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Author

Eric J Vick, MD, PhD Clinical Instructor and Fellow, Division of Hematology and Oncology, Department of Internal Medicine, University of Cincinnati Medical Center

Eric J Vick, MD, PhD is a member of the following medical societies: American Association for Cancer Research, American Society for Transplantation and Cellular Therapy, American Society of Clinical Oncology, American Society of Hematology, Society for Immunotherapy of Cancer

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Salomon's House LLC<br/>Have a 5% or greater equity interest in: Salomon's House LLC<br/>Received income in an amount equal to or greater than $250 from: American Society of Hematology.

Coauthor(s)

Ronald A Sacher, MD, FRCPC, DTM&H Professor Emeritus of Internal Medicine and Hematology/Oncology, Emeritus Director, Hoxworth Blood Center, University of Cincinnati Academic Health Center

Ronald A Sacher, MD, FRCPC, DTM&H is a member of the following medical societies: American Association for the Advancement of Science, American Association of Blood Banks, American Clinical and Climatological Association, American Society for Clinical Pathology, American Society of Hematology, College of American Pathologists, International Society of Blood Transfusion, International Society on Thrombosis and Haemostasis, Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Emmanuel C Besa, MD Professor Emeritus, Department of Medicine, Division of Hematologic Malignancies and Hematopoietic Stem Cell Transplantation, Kimmel Cancer Center, Jefferson Medical College of Thomas Jefferson University

Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American Society of Clinical Oncology, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, New York Academy of Sciences

Disclosure: Nothing to disclose.

Additional Contributors

Paul Schick, MD † Emeritus Professor, Department of Internal Medicine, Jefferson Medical College of Thomas Jefferson University; Research Professor, Department of Internal Medicine, Drexel University College of Medicine; Adjunct Professor of Medicine, Lankenau Hospital

Paul Schick, MD is a member of the following medical societies: American College of Physicians, American Society of Hematology

Disclosure: Nothing to disclose.

Swapna Boppana, MD Fellow, Division of Hematology-Oncology, University of Cincinnati Medical Center

Disclosure: Nothing to disclose.

Acknowledgements

Suzanne R Fanning, DO Fellow, Department of Hematology and Medical Oncology, Cleveland Clinic Foundation, 2004-2007 Director, Hematology, Greenville Memorial Health System, Greenville, SC Medical Oncologist/Hematologist/Transplant Physician, Cancer Centers of the Carolinas

Suzanne R Fanning, DO is a member of the following medical societies: American College of Physicians, American Medical Association, American Society for Blood and Marrow Transplantation, American Society of Clinical Oncology, and American Society of Hematology

Disclosure: Millenium Pharmaceuticals Consulting fee Review panel membership; Celgene Pharmaceuticals Consulting fee Review panel membership

Mohamad A Hussein, MD Clinical Director, Malignant Hematology, Moffitt Cancer Center

Mohamad A Hussein, MD is a member of the following medical societies: American Association of Blood Banks, American College of Physicians, American Medical Association, and American Society of Hematology

Disclosure: Nothing to disclose.

Yasodah Jayamohan, MD Transfusion Medicine Fellow, Hoxworth Blood Center, University of Cincinnati Medical Center

Yasodah Jayamohan, MD is a member of the following medical societies: American Society for Clinical Pathology, American Society of Cytopathology, College of American Pathologists, and United States and Canadian Academy of Pathology

Disclosure: Nothing to disclose.

Jaya Juturi, MD Fellow, Department of Hematology and Oncology, Texas Cancer Associates

Disclosure: Nothing to disclose.