Light chain deposition disease

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Light chain deposition disease
Other namesLCDD
AntibodyChains.svg
Schematic diagram of a typical antibody showing two Ig heavy chains (blue) linked by disulfide bonds to two Ig light chains (green). The constant (C) and variable (V) domains are shown.
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Light chain deposition disease (LCDD) is a rare blood cell disease which is characterized by deposition of fragments of infection-fighting immunoglobulins, called light chains (LCs), in the body. LCs are normally cleared by the kidneys, but in LCDD, these light chain deposits damage organs and cause disease. The kidneys are almost always affected and this often leads to kidney failure. About half of people with light chain deposition disease also have a plasma cell dyscrasia, a spectrum of diseases that includes multiple myeloma, Waldenström's macroglobulinemia, and the monoclonal gammopathy of undetermined significance premalignant stages of these two diseases. [1] [2] Unlike in AL amyloidosis, in which light chains are laid down in characteristic amyloid deposits, in LCDD, light chains are deposited in non-amyloid granules. [3]

Contents

Signs and symptoms

Light chain deposition disease can affect any organ. [3] Renal involvement is always present and can be identified by microscopic hematuria and proteinuria. Due to the gradual buildup of light chains from plasma filtration, renal function rapidly declines in the majority of patients with LCDD as either acute tubulointerstitial nephritis or rapidly progressing glomerulonephritis. This condition can include nephrotic syndrome, proteinuria, and/or renal failure. [4] Regardless of the degree of light chain excretion, renal failure happens with a comparable frequency. Furthermore, hypertension may be present at the time of diagnosis in patients with LCDD. [5] Deposits may form in the skin, lungs, gastrointestinal tract, thyroid glands, pancreas, bone marrow, spleen, lymph nodes, and adrenal glands. [3] Extrarenal deposition with symptoms is uncommon. It is unclear if localized LCDD is a real condition or if it is the first sign of a silent systemic LCDD. [6]

The liver is the most common extra-renal site in LCDD, although involvement is not always limited to this organ. There appears to be no relationship between the degree of light chain deposition within the liver and the severity of liver dysfunction. [7] In addition to portal hypertension and hepatic insufficiency, affected patients may die from hepatic failure. [8]

Cardiac involvement may be linked to paroxysmal atrial fibrillation, [9] severe congestive heart failure, and restrictive cardiomyopathy. [10]

The lungs are rarely affected by light chain deposition disease, which typically damages the parenchyma; [11] bronchial involvement seems to be extremely uncommon. [12] However recent reports have indicated that the major airways are involved. There have been descriptions of nodular as well as diffuse pulmonary interstitial diseases; however, the literature has only reported seven occurrences of pulmonary nodular-type LCDD to date. [13]

The effects of systemic protein deposition on the nerves are comparable to those of amyloidosis, which is clinically characterized by polyneuropathy. [14] Deposits may form in the choroid plexus and along nerve fibers. [15] Additionally, isolated LCDD within the brain has been reported. [16]

Cause

LCDD develops as a result of overproduction and thus deposition of abnormal immunoglobulins. About 60% of cases develop in the context of plasmacytoma, multiple myeloma, and other lymphoproliferative disorders. However, in many cases, an underlying cause cannot be identified. [4]

Mechanism

The main light chain structure in LCDD presumably dictates how the disease manifests in the body. Though κI-IV has been described, [17] the sequenced kappa light chains in LCDD are more likely to belong to the V-region subtype, of which VκIV appears to be overrepresented. [18] The pathogenicity of these proteins has not been linked to any particular structural pattern or residue, but a number of recurring characteristics have been identified. [19] Firstly, somatic mutations, not germline mutations, are the source of the amino acid substitutions. Secondly, the region that determines complementarity is where substitutions happen most frequently. [17] Third, hydrophobic residues are more likely to be introduced by the mutations reported in both the kappa and light chains. [20] This could disrupt protein-protein interactions and destabilize the protein, leading to protein deposition in tissues. [21] The propensity for aggregation is exemplified in a murine model of LCDD where light chain deposition was observed in the kidney of transfected mice using vectors that contained kappa light chain sequence from an individual with LCDD with the VκIV subtype. [22] Lastly, because some patients with LCDD have isolates of kappa light chains with mutations that produce new N-glycosylation sites, posttranslational modification can be linked to the creation of pathologic light chains. [23] It is possible that the new hydrophobic residues along with N-glycosylation sites will make it more likely for the light chains to accumulate in the affected tissues' basement membranes. [24] The mesangial cell is also thought to play a role in the pathogenesis of LCDD. [25]

Diagnosis

A number of laboratory tests are required in order to assist in diagnosing LCDD. Blood and urine samples are collected for evaluation of kidney and liver function and determination of the presence of a monoclonal protein. Imaging studies such as echocardiography and an ultrasound of the abdomen will be performed. A CT scan, magnetic resonance imaging (MRI) or positron emission tomography (PET) may also be indicated. [26]

Patients suspected of having LCDD should be evaluated using the screening panel for plasma cell proliferative disorders. [27] However, the sensitivity of laboratory testing strategies for detecting monoclonal gammopathies has increased with the development of quantitative serum assays to test for immunoglobulin free light chain; [28] this increased diagnostic sensitivity is easily noticeable in the monoclonal light chain diseases. [29] The most recent diagnostic screening guidelines state that serum immunofixation in addition to immunoglobulin free light chain is an adequate screening panel for plasma cell proliferative disorders apart from AL amyloidosis and LCDD due to the increased sensitivity for free light chain diseases. It is advised, nevertheless, that urine immunofixation be used in addition to LCDD and AL amyloidosis screening. [27]

The immunohistologic examination of tissue from an afflicted organ—which is not congophilic in nature—confirms the diagnosis of LCDD. The tissue's light chain restriction evaluation will determine whether the heavy or light chain is monoclonal. An abdominal ultrasound and echocardiography should be part of the workup when an individual is diagnosed with LCDD in order to evaluate the spleen, liver, and lymph nodes. A bone marrow aspirate as well as biopsy are necessary to rule out light amyloidosis and/or multiple myeloma. [5]

Similar to cardiac amyloid, diastolic dysfunction and a decrease in myocardial compliance may be discovered via echocardiography and catheterization. [30]

By using specific light chain stains in glomeruli as well as negative Congo red stain, tubular basement membranes, and punctate amorphous, ground-pepper-like appearance of deposits on electron microscopy, LCDD can be differentiated from other causes of nodular sclerosis and mesangial expansion. Diabetic nephropathy exhibits no deposits; fibrillary glomerulonephritis is Congo red negative and has a proliferative appearance along with polyclonal immunoglobulin G; other monoclonal immunoglobulin deposition disease exhibit both light and heavy chain staining or just heavy chain staining. Additional reasons for a membranoproliferative glomerulonephritis pattern exhibit electron microscopy appearances and immunofluorescence specific to the disease. [31]

Treatment

Decreasing production of the organ-damaging light chains is the treatment goal. Options include chemotherapy using bortezomib, autologous stem cell transplantation, immunomodulatory drugs, and kidney transplant. [32] There is no standard treatment for LCDD. High-dose melphalan in conjunction with autologous stem cell transplantation has been used in some patients. A regimen of bortezomib and dexamethasone has also been examined. [1]

Outlook

Different light chain deposition does not appear to have an impact on the clinical course of LCDD patients, as the clinical presentation is known to depend on the quantity and type of affected organs. [33] The median survival time is roughly four years. Following a median follow-up of 27 months, the most comprehensive series to date found that 59% of cases resulted in death and 57% of cases reached uremia. [4] LCDD prognostic factors include age, extrarenal light chain deposition, and plasma cell myeloma. [34]

Epidemiology

Being a relatively rare condition, LCDD is commonly misdiagnosed as a protein disease. Up to 50% of patients receive an LCDD diagnosis as a result of lymphoproliferative disorders such as multiple myeloma. [35] LCDD is diagnosed at a median age of 58 years. [4] LCDD affects men 2.5 times more than women [8] and is frequently linked with monoclonal gammopathies of unknown significance in 17% of patients. [5]

See also

Related Research Articles

<span class="mw-page-title-main">Multiple myeloma</span> Cancer of plasma cells

Multiple myeloma (MM), also known as plasma cell myeloma and simply myeloma, is a cancer of plasma cells, a type of white blood cell that normally produces antibodies. Often, no symptoms are noticed initially. As it progresses, bone pain, anemia, kidney dysfunction, and infections may occur. Complications may include hypercalcemia and amyloidosis.

<span class="mw-page-title-main">Amyloidosis</span> Metabolic disease involving abnormal deposited amyloid proteins

Amyloidosis is a group of diseases in which abnormal proteins, known as amyloid fibrils, build up in tissue. There are several non-specific and vague signs and symptoms associated with amyloidosis. These include fatigue, peripheral edema, weight loss, shortness of breath, palpitations, and feeling faint with standing. In AL amyloidosis, specific indicators can include enlargement of the tongue and periorbital purpura. In wild-type ATTR amyloidosis, non-cardiac symptoms include: bilateral carpal tunnel syndrome, lumbar spinal stenosis, biceps tendon rupture, small fiber neuropathy, and autonomic dysfunction.

<span class="mw-page-title-main">Cryoglobulinemia</span> Medical condition

Cryoglobulinemia is a medical condition in which the blood contains large amounts of pathological cold sensitive antibodies called cryoglobulins – proteins that become insoluble at reduced temperatures. This should be contrasted with cold agglutinins, which cause agglutination of red blood cells.

<span class="mw-page-title-main">Monoclonal gammopathy of undetermined significance</span> Medical condition

Monoclonal gammopathy of undetermined significance (MGUS) is a plasma cell dyscrasia in which plasma cells or other types of antibody-producing cells secrete a myeloma protein, i.e. an abnormal antibody, into the blood; this abnormal protein is usually found during standard laboratory blood or urine tests. MGUS resembles multiple myeloma and similar diseases, but the levels of antibodies are lower, the number of plasma cells in the bone marrow is lower, and it rarely has symptoms or major problems. However, since MGUS can lead to multiple myeloma, which develops at the rate of about 1.5% a year, or other symptomatic conditions, yearly monitoring is recommended.

<span class="mw-page-title-main">Bence Jones protein</span> Urinary protein

Bence Jones protein is a monoclonal globulin protein or immunoglobulin light chain found in the urine, with a molecular weight of 22–24 kDa. Detection of Bence Jones protein may be suggestive of multiple myeloma, or Waldenström's macroglobulinemia.

<span class="mw-page-title-main">Monoclonal gammopathy</span> Excess myeloma protein or monoclonal gamma globulin in the blood

Monoclonal gammopathy, also known as paraproteinemia, is the presence of excessive amounts of myeloma protein or monoclonal gamma globulin in the blood. It is usually due to an underlying immunoproliferative disorder or hematologic neoplasms, especially multiple myeloma. It is sometimes considered equivalent to plasma cell dyscrasia. The most common form of the disease is monoclonal gammopathy of undetermined significance.

<span class="mw-page-title-main">Cardiac amyloidosis</span> Medical condition

Cardiac amyloidosis is a subcategory of amyloidosis where there is depositing of the protein amyloid in the cardiac muscle and surrounding tissues. Amyloid, a misfolded and insoluble protein, can become a deposit in the heart's atria, valves, or ventricles. These deposits can cause thickening of different sections of the heart, leading to decreased cardiac function. The overall decrease in cardiac function leads to a plethora of symptoms. This multisystem disease was often misdiagnosed, with a corrected analysis only during autopsy. Advancements of technologies have increased earlier accuracy of diagnosis. Cardiac amyloidosis has multiple sub-types including light chain, familial, and senile. One of the most studied types is light chain cardiac amyloidosis. Prognosis depends on the extent of the deposits in the body and the type of amyloidosis. New treatment methods are actively being researched in regards to the treatment of heart failure and specific cardiac amyloidosis problems.

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<span class="mw-page-title-main">Myeloma protein</span> Abnormal immunoglobulin fragment

A myeloma protein is an abnormal antibody (immunoglobulin) or a fragment thereof, such as an immunoglobulin light chain, that is produced in excess by an abnormal monoclonal proliferation of plasma cells, typically in multiple myeloma or Monoclonal gammopathy of undetermined significance. Other terms for such a protein are monoclonal protein, M protein, M component, M spike, spike protein, or paraprotein. This proliferation of the myeloma protein has several deleterious effects on the body, including impaired immune function, abnormally high blood viscosity, and kidney damage.

<span class="mw-page-title-main">LECT2</span> Protein-coding gene in the species Homo sapiens

Leukocyte cell-derived chemotaxin-2 (LECT2) is a protein first described in 1996 as a chemotactic factor for neutrophils, i.e. it stimulated human neutrophils to move directionally in an in vitro assay system. The protein was detected in and purified from cultures of Phytohaemagglutinin-activated human T-cell leukemia SKW-3 cells. Subsequent studies have defined LECT2 as a hepatokine, i.e. a substance made and released into the circulation by liver hepatocyte cells that regulates the function of other cells: it is a hepatocyte-derived, hormone-like, signaling protein.

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Amyloid light-chain (AL) amyloidosis, also known as primary amyloidosis, is the most common form of systemic amyloidosis. The disease is caused when a person's antibody-producing cells do not function properly and produce abnormal protein fibers made of components of antibodies called light chains. These light chains come together to form amyloid deposits which can cause serious damage to different organs. An abnormal light chain in urine is known as Bence Jones protein.

AA amyloidosis is a form of amyloidosis, a disease characterized by the abnormal deposition of fibers of insoluble protein in the extracellular space of various tissues and organs. In AA amyloidosis, the deposited protein is serum amyloid A protein (SAA), an acute-phase protein which is normally soluble and whose plasma concentration is highest during inflammation.

<span class="mw-page-title-main">Mesangial proliferative glomerulonephritis</span> Medical condition

Mesangial proliferative glomerulonephritis (MesPGN) is a morphological pattern characterized by a numerical increase in mesangial cells and expansion of the extracellular matrix within the mesangium of the glomerulus. The increase in the number of mesangial cells can be diffuse or local and immunoglobulin and/or complement deposition can also occur. MesPGN is associated with a variety of disease processes affecting the glomerulus, though can be idiopathic. The clinical presentation of MesPGN usually consists of hematuria or nephrotic syndrome. Treatment is often consistent with the histologic pattern of and/or disease process contributing to mesangial proliferative glomerulonephritis, and usually involves some form of immunosuppressant.

Free light chains (FLCs) are immunoglobulin light chains that are found in the serum (blood) in an unbound (free) state. In recent decades, measuring the amount of free light chains (FLCs) in the blood has become a practical clinical test. FLC tests can be used to diagnose and monitor diseases like multiple myeloma and amyloidosis.

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<span class="mw-page-title-main">LECT2 amyloidosis</span> Medical condition

LECT2 Amyloidosis (ALECT2) is a form of amyloidosis caused by the LECT2 protein. It was found to be the third most common cause of amyloidosis in a set of more than 4,000 individuals studied at the Mayo Clinic; the first and second most common forms the disorder were AL amyloidosis and AA amyloidosis, respectively. Amyloidosis is a disorder in which the abnormal deposition of a protein in organs and/or tissues gradually leads to organ failure and/or tissue injury.

Monoclonal immunoglobulin deposition disease, or MIDD, is a disease characterised by the deposition of monoclonal immunoglobulins on the basement membrane of the kidney. Monoclonal immunoglobulins are produced by monoclonal plasma cells, which are found in a variety of plasma cell dyscrasias. The deposition of monoclonal immunoglobulins on the basement membrane of the kidney causes renal impairment. As well as the kidney, MIDD may also affect the liver, heart, peripheral nerves, lung and skin.

Crystal-storing histiocytosis is a form of histiocytosis which mostly occurs in people with monoclonal gammopathies. Histiocytosis is an excessive number of histiocytes. In the vast majority of crystal-storing histiocytosis cases, immunoglobulins accumulate within the cytoplasm of histiocytes; in rare cases clofazimine, cystine, silica, or Charcot–Leyden crystals may be found in the histiocytes instead. Non-immunoglobulin crystal-storing histiocytosis is mostly associated with non-malignant disorders, such as chronic inflammation or autoimmune abnormality conditions such as rheumatoid arthritis, Crohn's disease, or Helicobacter pylori gastritis. It may be a localised or generalised disease. Examples of locations where histiocytosis may occur include the lungs, pleura, stomach, kidney, bone marrow, thyroid, thymus, and parotid gland. The disease is described as generalised if two or more unrelated sites are involved.

Monoclonal gammopathy of renal significance (MGRS) are a group of kidney disorders that present with kidney damage due to nephrotoxic monoclonal immunoglobulins secreted by clonal plasma cells or B cells. By definition, people with MGRS do not meet criteria for multiple myeloma or other hematologic malignancies. The term MGRS was introduced in 2012 by the International Kidney and Monoclonal Gammopathy Research Group (IKMG). MGRS is associated with monoclonal gammopathy of undetermined significance (MGUS). People with MGUS have a monoclonal gammopathy but does not meet the criteria for the clonal burden nor the presence of end organ damage seen in hematologic malignancies. In a population based study based on the NHANES III health survey; 6% of patients with MGUS were subsequently classified as having MGRS. The prevalence and incidence of MGRS in the general population or in specific populations is not known but it is more prevalent in those over the age of 50 as there is a monoclonal protein (M-protein) present in 3% of those 50 and years older and 5% of those 70 years and older, placing those 50 and older at increased risk of MGRS.

References

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  29. ↑ Dispenzieri, Angela; Lacy, Martha Q.; Katzmann, Jerry A.; Rajkumar, S. Vincent; Abraham, Roshini S.; Hayman, Suzanne R.; Kumar, Shaji K.; Clark, Raynell; Kyle, Robert A.; Litzow, Mark R.; Inwards, David J.; Ansell, Stephen M.; Micallef, Ivana M.; Porrata, Luis F.; Elliott, Michelle A.; Johnston, Patrick B.; Greipp, Philip R.; Witzig, Thomas E.; Zeldenrust, Steven R.; Russell, Stephen J.; Gastineau, Dennis; Gertz, Morie A. (April 15, 2006). "Absolute values of immunoglobulin free light chains are prognostic in patients with primary systemic amyloidosis undergoing peripheral blood stem cell transplantation". Blood. American Society of Hematology. 107 (8): 3378–3383. doi: 10.1182/blood-2005-07-2922 . ISSN   0006-4971. PMC   1895763 . PMID   16397135.
  30. ↑ Ganeval, Dominique; Noël, Laure-Hélène; Preud'homme, Jean-Louis; Droz, Dominique; Grünfeld, Jean-Pierre (1984). "Light-chain deposition disease: Its relation with AL-type amyloidosis". Kidney International. Elsevier BV. 26 (1): 1–9. doi: 10.1038/ki.1984.126 . ISSN   0085-2538. PMID   6434789.
  31. ↑ Fogo, Agnes B.; Lusco, Mark A.; Najafian, Behzad; Alpers, Charles E. (2015). "AJKD Atlas of Renal Pathology: Light Chain Deposition Disease". American Journal of Kidney Diseases. Elsevier BV. 66 (6): e47–e48. doi: 10.1053/j.ajkd.2015.10.007 . ISSN   0272-6386. PMID   26593321.
  32. ↑ "Light chain deposition disease | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program". rarediseases.info.nih.gov. Retrieved 2018-04-18.
  33. ↑ Kim, Hee-Jun; Park, Eunkyung; Lee, Tae Jin; Do, Jae Hyuk; Cha, Young Joo; Lee, Sang Jae (2012). "A Case of Isolated Light Chain Deposition Disease in the Duodenum". Journal of Korean Medical Science. Korean Academy of Medical Sciences. 27 (2): 207–210. doi: 10.3346/jkms.2012.27.2.207 . ISSN   1011-8934. PMC   3271296 . PMID   22323870.
  34. ↑ LIN, JULIE; MARKOWITZ, GLEN S.; VALERI, ANTHONY M.; KAMBHAM, NEERAJA; SHERMAN, WILLIAM H.; APPEL, GERALD B.; D'AGATI, VIVETTE D. (2001). "Renal Monoclonal Immunoglobulin Deposition Disease". Journal of the American Society of Nephrology. Ovid Technologies (Wolters Kluwer Health). 12 (7): 1482–1492. doi: 10.1681/asn.v1271482 . ISSN   1046-6673. PMID   11423577.
  35. ↑ Wang, Qi; Jiang, Fang; Xu, Gaosi (2019). "The pathogenesis of renal injury and treatment in light chain deposition disease". Journal of Translational Medicine. 17 (1): 387. doi: 10.1186/s12967-019-02147-4 . ISSN   1479-5876. PMC   6878616 . PMID   31767034.

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