Light-Chain Deposition Disease

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Practice Essentials

Light-chain deposition disease (LCDD) is the deposition of monoclonal light chains in multiple organs. It is a rare disease characterized by deposition of nonamyloid immunoglobulin light chains, which unlike amyloid do not stain with Congo red and do not exhibit a fibrillar structure when examined ultrastructurally.[1, 2, 3, 4]

LCDD is categorized as a monoclonal immunoglobulin deposition disease in the World Health Organization (WHO) classification of tumors of hematopoietic and lymphoid tissues.[5, 6] LCDD was first described in 1976 in two patients with end-stage renal disease as granular deposits of free light chains in multiple organs, including the kidneys, that did not stain with Congo red.[7]

A single clonal plasma cell is responsible for the overproduction of kappa chains and more rarely, lambda light chains.[8] These light chains may be in association with a heavy chain as a complete antibody or be secreted as only a collection of light chains or as a combination of both,[9] although in LCDD only light chains will be observed. A monoclonal population of plasma cells can be detected in the bone marrow, and an altered serum-free light chain ratio is present.[10] In 25% of patients, an abnormal serum free light chain ratio is noted, even without an abnormal finding with serum and/or urine electrophoresis with immunofixation.[11]

The incidence of LCDD is unknown. The median age at diagnosis is 58 years, and it is more common in men than in women.[10] Approximately 50-60% of patients with LCDD have associated multiple myeloma and 17% have monoclonal gammopathy of unknown significance (MGUS) or no evidence of neoplastic plasma cell proliferation.[5]

LCDD can occur in any organ.[12] Kidneys are nearly always involved, and LCDD can present as kidney insufficiency, proteinuria, microscopic hematuria, and nephrotic syndrome. Albuminuria levels do not correlate with the presence of glomerulonephritis and can be present even without glomerulosclerosis.[13] Kidney function declines rapidly as rapidly progressive glomerulonephritis or acute tubulointerstitial nephritis.[5] Extrarenal LCDD most commonly involves the liver but may affect organs with a high degree of vascularity.[14, 15]  

Cardiac LCDD has been noted, manifesting in incidents of arrhythmias and even as an atrial mass.[16] Patients with pulmonary LCDD present with dyspnea, hemoptysis and chest pain, though symptomatic cases are rare; more typically pulmonary LCDD is an incidental finding in asymptomatic patients with lymphoproliferative disorders.[17]  

The approach for diagnosis with LCDD should be the same as for patients with plasma cell dyscrasias.[18] It should include a thorough history, physical examination, and laboratory and imaging studies (see Presentation and Workup). LCDD should be distinguished from Fanconi syndrome, myeloma cast nephropathy, cryoglobulinemia, and amyloidosis, all of which are also associated with monoclonal proteins and may present similarly.

LCDD is a rare disease, so no established guidelines exist and management remains controversial.[19] Treatment options include the following (see Treatment):

Pathophysiology

Renal light-chain deposition disease (LCDD) is characterized by the presence of: nodular sclerosing glomerulonephritis on light microscopy; diffuse linear staining in the glomerular and tubular basement membrane by a single light chain on immunofluorescence; and nonfibrillar, powdery, electron-dense deposits in the basements on electron microscopy.[20] Mesangial nodularity within the glomerulus occurs from the increased deposition of extracellular matrix proteins mixed with kappa light chains.[20]

The necessary criterion to make the diagnosis of LCDD requires that all tissues to be stained for kappa and light chains and the tissue must exhibit kappa fixation along the tubular basement membrane.[21, 22, 23] The tubular deposits are present predominantly along the loops of Henle, distal tubules, and proximal tubules.

Amyloid light-chain (AL)–amyloidosis consists predominantly of lambda light chains, whereas kappa light chains are predominantly involved in LCDD. Electron microscopy is helpful in distinguishing between these lesions.

Extrarenal involvement

Symptomatic extrarenal LCDD is rare.[24] The liver is the most frequent site involved.[11, 25] The deposits in the liver are usually confined to the sinusoids and basement membrane of biliary ducts, without associated parenchymal lesions. The degree of liver involvement does not correlate with the amount of light chains deposited.[25] Patients may develop cirrhosis and/or portal hypertension and may die from liver failure.[11]

The cardiac manifestations include restrictive cardiomyopathy, cardiomegaly, congestive heart failure, and arrhythmias.[26, 27, 28] Echocardiography and cardiac catheterization may reveal diastolic dysfunction and decreased myocardial compliance.[29]

Pulmonary LCDD is rare, and it usually damages the lung parenchyma. Involvement of the large airways has been reported.[30] Nodular and diffuse pulmonary interstitial diseases of the lungs have been described.[31, 32, 33]

LCDD can affect peripheral nerves, resulting in polyneuropathy.[34] Isolated LCDD restricted to the brain has been reported as well, wherein the periventricular foci of intracerebral vessels were overloaded with amorphous, eosinophilic material that stained for lambda light chains.[35] In general, the blood-brain barrier protects the central nervous system (CNS) from circulating, misfolded proteins, but cases of intracerebral amyloidoma have been reported.[36]

LCDD can also affect other sites, such as lymph nodes, skin, spleen, pancreas, bone marrow, thyroid gland, adrenal gland, and abdominal blood vessels.[10]

LCDD is associated with multiple myeloma is about 58% of cases.[5] LCDD could be present at diagnosis of a new plasma cell dyscrasia or could represent an extramedullary manifestation of multiple myeloma while relapsing after chemotherapy.[29] LCDD can also complicate other B-cell lymphomas, such as lymphoplasmacytic lymphoma, marginal-zone lymphoma, and chronic lymphocytic lymphoma.[37]

Epidemiology

Frequency

United States

The frequency of light-chain deposition disease (LCDD) is unknown. The disease is found in approximately 5% of patients with multiple myeloma at autopsy. 

Mortality/Morbidity

The most common cause of mortality and morbidity in LCDD is related to renal complications, including hypertension, nephrotic syndrome, and progression to end-stage renal disease (ESRD). Liver dysfunction can also occur, with progression to hepatic failure. Other symptoms of LCDD relate to congestive heart failure, peripheral neuropathy, and skin lesions secondary to the deposition of light chains.

Prognosis

Median survival for patients with light-chain deposition disease (LCDD) is about 4 years. The largest series published so far has reported after a median follow-up of 27 months; 57% of patients developed uremia and 59% of patients died.[10]  The prognostic factors are age, associated multiple myeloma, and extrarenal light-chain deposition.[5, 20]  The presence of end-stage renal disease (ESRD) and need for dialysis did not change the outcome for these patients.

Overall survival has been shown to be influenced by patient age, the presence of coexisting multiple myeloma or cast nephropathy, and any evidence of extrarenal light-chain deposition.

The natural or treated history of LCDD is difficult to determine, as this disease is uncommon.

History

Patients are typically found to have light-chain deposition disease (LCDD) when they are evaluated for proteinuria or nephrotic syndrome by kidney biopsy and may have simultaneous dysfunction of other organs, though rarely. The common symptoms and signs are related to associated organ involvement and may manifest as kidney failure, congestive heart failure, and/or liver failure. Most patients present with advanced disease, owing to a delay in the diagnosis and insidious onset of dysfunction.

Physical Examination

Light-chain deposition disease (LCDD) patients may present with end-organ damage, which chiefly manifests as hypertension, peripheral edema, neuropathy, or congestive heart failure. Approximately 50% of patients with LCDD present with nephrotic syndrome. However, in a quarter of patients, the proteinuria is less than 1 g/day as a result of tubulointerstitial involvement. Rare findings on physical examination include new heart sounds and splenomegaly.

Laboratory Studies

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

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] : 

Procedures

Biopsy procedures are as follows:

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.

Medical Care

Treatment of light-chain deposition disease (LCDD) is indicated for patients who present with systemic involvement, kidney dysfunction, and the associated presence of multiple myeloma. The goal of treatment in these patients is to suppress the production of light chains and damage to other organs. Appropriate medical management must be provided for organ dysfunction as needed, such as the use of angiotensin-converting enzyme (ACE) inhibitors or dialysis.

Unlike in multiple myeloma, the plasma cell burden is quite low (< 5%) and the genetic abnormalities associated with adverse prognosis in multiple myeloma are generally absent. In patients with LCDD associated with multiple myeloma, the prognosis is quite poor and they should be treated per multiple myeloma guidelines.[43] In these patients, there is little evidence to support maintenance therapy,[44]  except in cases where the patients would otherwise be receiving therapy for multiple myeloma. LCDD is a rare disease; hence, no placebo-controlled trials have been performed, there are no established guidelines, and management remains controversial.[19]

Treatment options include the following[45] :

Autologous stem cell transplantation

Autologous stem cell transplantation (ASCT) can produce durable responses in patients with LCDD.[19, 46, 47] Stem cells are mobilized using granulocyte colony-stimulating factor (G-CSF), and high-dose chemotherapy with melphalan is given. The dose of melphalan is adjusted to the patient's kidney function, to decrease morbidity.

A long-term analysis of 6 patients with LCDD who underwent ASCT demonstrated that this is an effective therapy for patients with kidney dysfunction due to LCDD.[19] Proteinuria was reduced by 92% and the glomerular filtration rate improved by 95% in these patients. The authors also suggest that if kidney dysfunction persists after ASCT, a hematologic response may permit successful kidney transplantation with improved graft viability and decreased risk of recurrence.

Another study of patients with LCDD treated with high-dose melphalan followed by ASCT also demonstrated that patients with kidney dysfunction have improvement in kidney function following ASCT.[48] Of the 5 evaluable patients with hematologic response, one had a complete response and four had a partial response.

A systematic review and pooled analysis of the outcomes of 46 patients in 11 studies who underwent bortezomib-based regimens or ASCT (with/without bortezomib induction therapy) reported bortezomib-based chemotherapy followed by ASCT appeared to be an effective treatment for LCDD with durable hematologic remission. Bortezomib-dexamethasone induction chemotherapy followed by ASCT achieved a complete response (CR) rate of 61.5% whereas bortezomib-based regimens without subsequent ASCT were associated with a CR rate of 55.6%.[49]

The use of high-dose chemotherapy followed by ASCT is associated with toxicities such as mucositis, sepsis, bacteremia, and diarrhea. In general, LCDD patients are younger; thus, ASCT should be considered in these patients. However, associated comorbidities, cardiac involvement, concomitant multiple myeloma, and number of organs affected may predict for a worse outcome. Therefore, age and comorbidities should be considered prior to ASCT. Multiorgan failure following ASCT has been reported in patients with extrarenal disease.[19]

Bortezomib

In LCDD, monoclonal light chains interact with the receptors in mesangial cells and activate many pathways, including the nuclear factor (NFkB) pathway. This results in increased cytokine production, leading to cell proliferation and activation of genes responsible for collagen and tenascin production. These changes lead to changes in the mesangial matrix, causing glomerulosclerosis.[12, 20] Bortezomib inhibits the 26S proteasome, and through secondary effectors modulates the NFkB pathways, decreases cytokine production, and decreases collagen production.[50, 51, 52]  Interruption of the downstream cascade by bortezomib prevents rapid progression of glomerulosclerosis and proteinuria and improves kidney function.[51, 53]

Bortezomib has been used in small series of patients with LCDD, including as induction therapy.[47, 54, 55] In one series, 3 patients were treated with bortezomib as induction therapy. This led to rapid hematologic response after a mean of 2 cycles, based on decrease in serum-free light-chain levels.[54] Another series on the use of bortezomib with dexamethasone as induction therapy prior to ASCT in 4 patients reported rapid response, with 50% of patients achieving a complete hematologic response.[55] A Canadian group reported on the use of bortezomib and dexamethasone as induction therapy in 2 patients prior to ASCT; both achieved partial response after 3 cycles and organ response 6 months after ASCT.[46]

The Canadian group also reported the only randomized study of 6 patients with LCDD. Patients were randomized to either dexamethasone alone or bortezomib with dexamethasone prior to high-dose chemotherapy with melphalan followed by ASCT. After completion of induction therapy, 4 of 6 patients achieved partial response based on the decrease in serum-free light-chain ratio, and 2 of 6 achieved stable disease and both were in dexamethasone alone group.

At day 100, the post-ASCT overall response rate was 100%; 4 patients achieved complete hematological response, 1 exhibited near-complete response, and 1 attained partial response. All patients derived clinical benefits, including those who achieved less than complete response. At 6 months post-ASCT, all 6 patients showed organ response, manifested mainly by decreased proteinuria of greater than 50%. Patients receiving bortezomib and dexamethasone induction showed a median time of kidney response of 3 months versus 6 months for the group receiving only dexamethasone. All 6 patients were alive after a median follow-up of 2 years and have remained dialysis-free.[56]

Based on the limited available data, induction with bortezomib helps improve kidney function. This may possibly permit more high-dose chemotherapy followed by ASCT, enabling for a better outcome. With bortezomib-based therapy, hematologic responses are rapid and are normally accompanied by rapid and significant reduction of proteinuria and improvement of kidney function.

The measurement of serum-free light chains was useful in the follow-up of patients with LCDD, and the reduction of involved light chains was associated with a significant improvement of proteinuria. High-dose chemotherapy followed by ASCT is a safe and well-tolerated treatment for LCDD, showing a good overall response rate.

Immunomodulatory agents

Thalidomide and its analogs lenalidomide and pomalidomide are immunomodulatory drugs that have been extensively studied in amyloid light-chain (AL)–amyloidosis and multiple myeloma. They have shown promise in LCDD, but their use has been limited and their role needs to be further explored in prospective studies. Case reports have included the following:

A case report describes a patient in whom melphalan and prednisolone therapy was started upon diagnosis of LCDD and continued for 10 years. Serial evaluations of kidney histology revealed that nodular lesions resolved and the glomeruli became nearly normal.[60]  

Daratumumab

Daratumumab is a monoclonal antibody targeting CD38 that has become a cornerstone in the treatment of multiple myeloma and can be used with both lenalidomide and bortezomib-based regimens. Milani et al described successful use of daratumumab in the treatment of eight patients with LCDD. All eight had bone marrow involvement of > 10% with disease confined to the kidney and had been heavily pretreated (most having had more than 2 lines of therapy). Mean time from diagnosis to initiation of daratumumab was 57 months. Daratumumab was administered at 16 mg/kg weekly for 8 weeks, followed by every other week for 8 doses, and then every 4 weeks as maintenance.[61]

Seven of the eight patients achieved a partial response, defined as a > 50% decrease in serum free light chains. Two patients achieved a renal response, and four had improvement or stabilization of kidney function. Responses were durable to 20 months.[61]  These promising results suggest that daratumumab represents a fresh option for treatment of LCDD, although the study population was small.

Kidney transplantation

A few patients with LCDD with end-stage renal disease (ESRD) have undergone kidney transplantation.[62]  Long-term benefits are seen, but allograft survival is significantly reduced in this patient population.[63] LCDD patients who have detectable light chains in urine or serum have worse outcomes, with early recurrences despite pretransplantation treatment.[64] Thus, transplantation should be reserved for select patients with a relatively slow course in whom light-chain production can be controlled by directed therapy.

Despite careful consideration, LCDD may recur in kidney transplant recipients. Recurrences sometimes can be confused with acute rejection. A case report suggests that bortezomib can successfully reverse the early recurrence of LCDD in the allograft.[65] Rituximab could also be considered for delaying early LCDD recurrence in patients in whom treatment of the underlying bone marrow disorder failed or is contraindicated, but maintenance therapy is apparently necessary to consolidate this response.[66] The possibility of maintenance with bortezomib, thalidomide, or daratumumab needs to be further explored.

What is light-chain deposition disease (LCDD)?What is the pathophysiology of light-chain deposition disease (LCDD)?What is the pathophysiology of extrarenal light-chain deposition disease (LCDD)?What is the prevalence of light-chain deposition disease (LCDD)?What is the morbidity and mortality associated with light-chain deposition disease (LCDD)?Which clinical history findings are characteristic of light-chain deposition disease (LCDD)?Which physical findings are characteristic of light-chain deposition disease (LCDD)?What causes light-chain deposition disease (LCDD)?What are the differential diagnoses for Light-Chain Deposition Disease?What is the role of lab tests in the workup of light-chain deposition disease (LCDD)?What is the role of imaging studies in the workup of light-chain deposition disease (LCDD)?What is the role of biopsy in the workup of light-chain deposition disease (LCDD)?Which histologic findings are characteristic of light-chain deposition disease (LCDD)?How is light-chain deposition disease (LCDD) treated?What is the role of autologous stem cell transplantation in the treatment of light-chain deposition disease (LCDD)?What is the role of bortezomib in the treatment of light-chain deposition disease (LCDD)?What is the role of immunomodulatory drugs in the treatment of light-chain deposition disease (LCDD)?What is the role of renal transplantation in the treatment of light-chain deposition disease (LCDD)?

Author

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

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

Disclosure: Nothing to disclose.

Specialty Editors

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

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

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.

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