LCDD
MCID: LGH004
MIFTS: 43

Light Chain Deposition Disease (LCDD)

Categories: Blood diseases, Immune diseases, Nephrological diseases, Rare diseases

Aliases & Classifications for Light Chain Deposition Disease

MalaCards integrated aliases for Light Chain Deposition Disease:

Name: Light Chain Deposition Disease 53 59
Light-Chain Deposition Disease 53
Light Chain Disease 72
Lcdd 59

Classifications:



External Ids:

ICD10 via Orphanet 34 D89.8
UMLS via Orphanet 73 C0238239
Orphanet 59 ORPHA93558
UMLS 72 C0238239

Summaries for Light Chain Deposition Disease

NIH Rare Diseases : 53 Light chain deposition disease (LCDD) is a rare condition characterized by the deposition of specific proteins (monoclonal light chains) in the kidneys and other organs. Light chains are used to make antibodies that the body needs to fight infection. People with LCDD make too many light chains, which get deposited in many body tissues. While LCDD can occur in any organ, the kidneys are always involved. Signs and symptoms of LCDD may include protein in the urine; decreased kidney function; and/or nephrotic syndrome. Rarely, a person with LCDD may have symptoms from cardiac (heart) or liver involvement. The underlying cause of LCDD is unknown. It is often associated with multiple myeloma. LCDD may progress to multiple myeloma, or it may be present with multiple myeloma when it is first diagnosed. The goal of treating LCDD is to slow the production of light chains and their damage to organs. Treatment may include chemotherapy with a drug called Bortezomib; autologous stem cell transplantation; immunomodulatory drugs; and/or kidney transplant. If untreated, end-stage renal disease occurs in 70% of cases.

MalaCards based summary : Light Chain Deposition Disease, also known as light-chain deposition disease, is related to lymphangioleiomyomatosis and lacrimal duct defect. An important gene associated with Light Chain Deposition Disease is ACTC1 (Actin Alpha Cardiac Muscle 1), and among its related pathways/superpathways are Integrin Pathway and Degradation of the extracellular matrix. The drugs Dexamethasone acetate and Dexamethasone have been mentioned in the context of this disorder. Affiliated tissues include kidney, liver and heart, and related phenotypes are homeostasis/metabolism and immune system

Wikipedia : 75 Light chain deposition disease (LCDD) is a rare blood cell disease which is characterized by deposition... more...

Related Diseases for Light Chain Deposition Disease

Diseases related to Light Chain Deposition Disease via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 179)
# Related Disease Score Top Affiliating Genes
1 lymphangioleiomyomatosis 30.0 MMP2 MMP14 MMP1
2 lacrimal duct defect 12.0
3 amyloidosis 10.7
4 myeloma, multiple 10.7
5 nephrotic syndrome 10.6
6 fibromatosis, gingival, 1 10.4 MMP2 MMP1
7 kidney disease 10.4
8 end stage renal failure 10.4
9 al amyloidosis 10.4
10 multicentric osteolysis, nodulosis, and arthropathy 10.4 MMP2 MMP14
11 winchester syndrome 10.4 MMP2 MMP14
12 conjunctivochalasis 10.4 MMP2 MMP1
13 mid-dermal elastolysis 10.4 MMP7 MMP12
14 chronic actinic dermatitis 10.3 MMP9 MMP1
15 chromoblastomycosis 10.3 MMP9 MMP1
16 cholestasis 10.3
17 extracranial arteriovenous malformation 10.3 MMP9 MMP2
18 villous adenocarcinoma 10.3 MMP9 MMP2
19 diabetes and deafness, maternally inherited 10.3
20 glomerulonephritis 10.3
21 lentigo maligna melanoma 10.3 MMP9 MMP2
22 psammomatous meningioma 10.2 MMP9 MMP2
23 acute transverse myelitis 10.2 MMP9 MMP2
24 transverse myelitis 10.2 MMP9 MMP2
25 acute kidney failure 10.2
26 monoclonal gammopathy of uncertain significance 10.2
27 ischemic colitis 10.2 MMP9 MMP2
28 parotitis 10.2 MMP9 MMP2
29 conjunctival nevus 10.2 MMP9 MMP2
30 collagenous colitis 10.2 MMP9 MMP1
31 pelvic organ prolapse 10.2 MMP9 MMP1
32 aortic aneurysm, familial thoracic 1 10.2 MMP2 MMP1
33 lymph node disease 10.2 MMP14 MMP1
34 brain glioblastoma multiforme 10.2 MMP9 MMP7
35 corneal ulcer 10.2 MMP9 MMP2
36 lymphoproliferative syndrome 10.2
37 portal hypertension 10.2
38 plasmacytoma 10.2
39 restrictive cardiomyopathy 10.2
40 neuropathy 10.2
41 light and heavy chain deposition disease 10.2
42 heavy chain deposition disease 10.2
43 immunotactoid glomerulopathy 10.2
44 pediatric multiple sclerosis 10.2 MMP9 MMP7
45 nasal cavity squamous cell carcinoma 10.2 MMP9 MMP2
46 chronic venous insufficiency 10.1 MMP9 MMP1
47 venous insufficiency 10.1 MMP9 MMP1
48 atrial standstill 1 10.1
49 polyneuropathy 10.1
50 peripheral nervous system disease 10.1

Graphical network of the top 20 diseases related to Light Chain Deposition Disease:



Diseases related to Light Chain Deposition Disease

Symptoms & Phenotypes for Light Chain Deposition Disease

MGI Mouse Phenotypes related to Light Chain Deposition Disease:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 homeostasis/metabolism MP:0005376 9.56 ACTC1 MMP1 MMP12 MMP14 MMP2 MMP7
2 immune system MP:0005387 9.17 CD68 MMP1 MMP12 MMP14 MMP2 MMP7

Drugs & Therapeutics for Light Chain Deposition Disease

Drugs for Light Chain Deposition Disease (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 42)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 3 1177-87-3
2
Dexamethasone Approved, Investigational, Vet_approved Phase 3 50-02-2 5743
3
Bortezomib Approved, Investigational Phase 3 179324-69-7 93860 387447
4
Melphalan Approved Phase 3 148-82-3 460612 4053
5
Mechlorethamine Approved, Investigational Phase 3 51-75-2 4033
6
Lenalidomide Approved Phase 3 191732-72-6 216326
7 Hormone Antagonists Phase 3
8 Antiemetics Phase 3
9 Anti-Inflammatory Agents Phase 3
10 Peripheral Nervous System Agents Phase 3
11 Gastrointestinal Agents Phase 3
12 HIV Protease Inhibitors Phase 3
13 glucocorticoids Phase 3
14 Hormones Phase 3
15
protease inhibitors Phase 3
16 Immunosuppressive Agents Phase 3
17 Antineoplastic Agents, Hormonal Phase 3
18 BB 1101 Phase 3
19 Autonomic Agents Phase 3
20 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 3
21 Alkylating Agents Phase 3
22 Antineoplastic Agents, Alkylating Phase 3
23 phenylalanine Phase 3
24 Angiogenesis Inhibitors Phase 3
25 Angiogenesis Modulating Agents Phase 3
26 Immunologic Factors Phase 3
27
Cyclophosphamide Approved, Investigational Phase 2 50-18-0, 6055-19-2 2907
28
Mesna Approved, Investigational Phase 2 3375-50-6 598
29
Prednisone Approved, Vet_approved Phase 2 53-03-2 5865
30
Doxorubicin Approved, Investigational Phase 2 23214-92-8 31703
31
Lenograstim Approved, Investigational Phase 2 135968-09-1
32 Anti-Infective Agents Phase 2
33 Anti-Bacterial Agents Phase 2
34 Antirheumatic Agents Phase 2
35
Liposomal doxorubicin Phase 2 31703
36 Dexamethasone 21-phosphate Phase 2
37 Antibodies Phase 2
38 Myeloma Proteins Phase 2
39 Paraproteins Phase 2
40 Adjuvants, Immunologic Phase 2
41 Immunoglobulins Phase 2
42
Pomalidomide Approved Phase 1 19171-19-8

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 A Randomized Phase III Trial of Melphalan and Dexamethasone (MDex) Versus Bortezomib, Melphalan and Dexamethasone (BMDex) for Untreated Patients With Systemic Light-Chain (AL) Amyloidosis Ineligible for Autologous Stem-Cell Transplantation Completed NCT01078454 Phase 3 melphalan;dexamethasone;bortezomib
2 Randomized Phase III Trial of Lenalidomide Versus Observation Alone in Patients With Asymptomatic High-Risk Smoldering Multiple Myeloma Active, not recruiting NCT01169337 Phase 3 Lenalidomide
3 S0115, A Phase II Trial Evaluating Modified High Dose Melphalan (100 mg/m) And Autologous Peripheral Blood Stem Cell Supported Transplant (SCT) For High Risk Patients With Multiple Myeloma And/Or Light Chain Amyloidosis (AL Amyloidosis) (A BMT Study) Completed NCT00064337 Phase 2 cyclophosphamide;dexamethasone;melphalan;thalidomide
4 An Open-label Phase II Study of the Efficacy of Combination Bortezomib-containing Regimens in the Treatment of Newly Diagnosed Patients With t (4; 14) Positive Multiple Myeloma Completed NCT00570180 Phase 2 Bortezomib
5 A Multicenter Phase II Trial of Bortezomib (Velcade), Melphalan, and Dexamethasone (V-MD) in Patients With Symptomatic AL-Amyloidosis or Light Chain Deposition Disease Active, not recruiting NCT00520767 Phase 2 bortezomib;dexamethasone;melphalan
6 High-Dose Melphalan and Autologous Stem Cell Transplantation (HDM/SCT) in Light-Chain Deposition Disease (LCDD) and Immunoglobulin Deposition Disease (IGDD) Terminated NCT00681044 Phase 2 melphalan
7 A Phase 1 Study of Reolysin Alone in Patients With Relapsed or Refractory Multiple Myeloma Completed NCT01533194 Phase 1
8 Phase I Study of Pomalidomide, Bortezomib, and Dexamethasone (PVD) as First-Line Treatment of AL Amyloidosis or Light Chain Deposition Disease Active, not recruiting NCT01728259 Phase 1 pomalidomide;bortezomib;dexamethasone
9 Pilot Study of Bortezomib/Dexamethasone (BD), Followed By Autologous Stem Cell Transplantation and Maintenance Bortezomib/Dexamethasone For the Initial Treatment of Monoclonal Immunoglobulin Deposition Disease (MIDD) Associated With Multiple Myeloma and AL Amyloidosis Completed NCT01383759 Bortezomib/Dexamethasone (BD), Followed By Autologous STC & Maintenance Bortezomib/Dexamethasone
10 Role of Cardiac MR (CMR) In Detecting Features of Early Myocardial Infiltration With Amyloid and Light Chain Deposition Disease in Subjects With Multiple Myeloma Terminated NCT01018927

Search NIH Clinical Center for Light Chain Deposition Disease

Genetic Tests for Light Chain Deposition Disease

Anatomical Context for Light Chain Deposition Disease

MalaCards organs/tissues related to Light Chain Deposition Disease:

41
Kidney, Liver, Heart, Lung, B Cells, Bone, Bone Marrow

Publications for Light Chain Deposition Disease

Articles related to Light Chain Deposition Disease:

(show top 50) (show all 361)
# Title Authors PMID Year
1
Bortezomib-Based Chemotherapy with Autologous Stem Cell Transplantation for Monoclonal Gammopathy of Renal Significance: A Case Report and Literature Review. 38
31352451 2019
2
Proteomic evidence of specific IGKV1-8 association with cystic lung light chain deposition disease. 38
30967366 2019
3
Coexistent Amyloid Fibrils in a Patient with Combined Light Chain Deposition Disease and Light Chain Cast Nephropathy. 38
31142970 2019
4
[A case of bronchiectasis due to light chain deposition disease]. 38
30686554 2019
5
Comparison of two serum free light chain assays for the diagnosis of primary plasma cell malignant proliferative disease. 38
31049418 2019
6
Primary idiopathic CNS non-amyloidogenic light chain deposition disease complicated by treatment-resistant focal seizure disorder. 38
30424969 2019
7
Histopathologic findings in lung biopsies from patients with primary biliary cholangitis. 38
30067952 2018
8
Treatment of multiple myeloma with renal involvement: the nephrologist's view. 38
30524711 2018
9
Lenalidomide as a Beneficial Treatment Option for Renal Impairment Caused by Light Chain Deposition Disease. 38
30101913 2018
10
Unmasking and successful management of light chain deposition disease of kidney in pregnancy: a complex case, mirroring the complex needs of pregnancy with kidney disease in India. 38
30187379 2018
11
Acquired Fanconi syndrome secondary to light chain deposition disease associated with monoclonal gammopathy of renal significance: A case report. 38
30200082 2018
12
Successful treatment of nephrotic syndrome induced by lambda light chain deposition disease using lenalidomide: A case report and review of the literature
. 38
29393843 2018
13
Primary Central Nervous System Lymphoma With Light Chain Deposition Disease (Aggregoma). 38
29280764 2018
14
Phenotypic plasticity of mesenchymal stem cells is crucial for mesangial repair in a model of immunoglobulin light chain-associated mesangial damage. 38
29668344 2018
15
Cutaneous Light Chain Deposition Disease: A Report of 2 Cases and Review of the Literature. 38
28953013 2018
16
Autologous Stem Cell Transplantation for Multiple Myeloma: Single Centre Experience from North India. 38
29622867 2018
17
Light Chain Deposition Disease Associated With Multiple Myeloma Developing in Late Pregnancy. 38
29507277 2018
18
Light Chain Deposition Disease with Bullous Skin Lesions Mimicking Atypical Bullous Pemphigoid. 38
29135016 2018
19
Light-Chain Deposition Disease Presenting with Spontaneous Splenic Rupture. 38
30756004 2018
20
Primary CNS Nonamyloidogenic Light Chain Deposition Disease: Case Report and Brief Review. 38
28669250 2017
21
A Case Report of Acute Renal Failure as a Result of Light-Chain-Mediated Acute Tubular Interstitial Nephritis in a 30-Year-Old Combat Veteran. 38
29087890 2017
22
OCULAR MANIFESTATIONS OF MONOCLONAL IMMUNOGLOBULIN LIGHT CHAIN DEPOSITION DISEASE. 38
27315323 2017
23
Detection and Treatment of Monoclonal Gammopathy of Renal Significance With Progression to Light Chain Deposition Disease. 38
28809601 2017
24
Clinical characteristics and prognostic factors in multiple myeloma patients with light chain deposition disease. 38
28383130 2017
25
[The value of serum free light chain in differential diagnosis of monoclonal gammopathy of renal significance]. 38
28822451 2017
26
Cardiac light-chain deposition disease relapsing in the transplanted heart. 38
28562083 2017
27
Intracystic hemorrhage in a patient with pulmonary cystic disorder related to light-chain deposition disease. 38
27756655 2017
28
[Clinical features and prognosis of patients with primary light chain deposition disease]. 38
28395454 2017
29
The Lung in Dysregulated States of Humoral Immunity. 38
28910817 2017
30
Light Chain Deposition Disease Diagnosed with Laser Micro-dissection, Liquid Chromatography, and Tandem Mass Spectrometry of Nodular Glomerular Lesions. 38
28050001 2017
31
Light-Chain Deposition Disease with Prominent Hepatic Involvement. 38
30755931 2017
32
Plasma cell neoplasia after kidney transplantation: French cohort series and review of the literature. 38
28636627 2017
33
ATYPICAL MACULOPATHY IN A PATIENT WITH LIGHT CHAIN DEPOSITION DISEASE MIMICKING ADVANCED GEOGRAPHIC ATROPHY. 38
26934302 2017
34
[Light-chain deposition disease is a hematologic problem]. 38
28252625 2017
35
Treatment of idiopathic light chain deposition disease: complete remission with bortezomib and dexamethasone. 38
28001187 2016
36
Renal disease related to Waldenström macroglobulinaemia: incidence, pathology and clinical outcomes. 38
27468978 2016
37
Cystic Lung Diseases: Algorithmic Approach. 38
27180915 2016
38
Bortezomib Based Chemotherapy for Light Chain Deposition Disease. 38
27762125 2016
39
Nodular pulmonary light chain deposition disease. 38
27306574 2016
40
Diffuse Cystic Lung Diseases: Diagnostic Considerations. 38
27231867 2016
41
Surgical Management of Pulmonary Mucosa-Associated Lymphoid Tissue Lymphoma Associated With Light-Chain Deposition Disease. 38
27211983 2016
42
Nodular pulmonary light chain deposition disease: an entity associated with Sjögren syndrome or marginal zone lymphoma. 38
26502747 2016
43
Spontaneous splenic rupture in a patient with light-chain deposition disease undergoing autologous peripheral blood stem cell transplantation. 38
27384856 2016
44
Can We Cure Light Chain Deposition Disease of the Kidneys?-A Review and Case Report of a Patient Treated With a Triple Transplant Approach. 38
27101986 2016
45
IgG Subclass Staining in Routine Renal Biopsy Material. 38
26848798 2016
46
Renal impairment in multiple myeloma: A single center experience. 38
27215238 2016
47
Clinicopathological characteristics and outcomes of light chain deposition disease: an analysis of 48 patients in a single Chinese center. 38
27056200 2016
48
Unusual Presentation of Light Chain Deposition Disease: A Case Report. 38
27437235 2016
49
A Case of Cardiac Light Chain Deposition Disease in a Patient with Solitary Plasmacytoma. 38
26988342 2016
50
Trends in Survival and Renal Recovery in Patients with Multiple Myeloma or Light-Chain Amyloidosis on Chronic Dialysis. 38
26728585 2016

Variations for Light Chain Deposition Disease

Expression for Light Chain Deposition Disease

Search GEO for disease gene expression data for Light Chain Deposition Disease.

Pathways for Light Chain Deposition Disease

Pathways related to Light Chain Deposition Disease according to GeneCards Suite gene sharing:

(show all 24)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.15 TNC MMP9 MMP7 MMP2 MMP12 MMP1
2
Show member pathways
12.45 TNC MMP9 MMP7 MMP2 MMP14 MMP12
3
Show member pathways
11.98 TNC MMP9 MMP7 MMP2 MMP12 MMP1
4 11.87 TNC MMP12 MMP1
5
Show member pathways
11.86 TNC MMP9 MMP7 MMP2
6 11.85 MMP9 MMP7 MMP2 MMP14 MMP12 MMP1
7 11.82 MMP9 MMP2 MMP14
8 11.78 MMP9 MMP2 MMP1
9 11.62 TNC MMP9 MMP7 MMP2
10 11.56 MMP9 MMP2 MMP1
11
Show member pathways
11.49 MMP9 MMP2 MMP1
12 11.44 MMP9 MMP7 MMP2 MMP14
13
Show member pathways
11.43 MMP9 MMP7 MMP2 MMP14 MMP12 MMP1
14 11.3 MMP9 MMP2
15
Show member pathways
11.26 MMP9 MMP2
16 11.26 TNC MMP9 MMP1
17 11.18 MMP9 MMP2 MMP1
18 11.11 MMP9 MMP2
19 11.09 TNC MMP9
20 11.01 MMP9 MMP2
21 10.95 MMP9 MMP2
22 10.9 MMP2 MMP1
23 10.89 MMP9 MMP7 MMP2 MMP14 MMP12 MMP1
24 10.84 MMP9 MMP7 MMP1

GO Terms for Light Chain Deposition Disease

Cellular components related to Light Chain Deposition Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.76 TNC MMP9 MMP7 MMP2 MMP12 MMP1
2 extracellular space GO:0005615 9.61 TNC MMP9 MMP7 MMP2 MMP14 MMP12
3 blood microparticle GO:0072562 9.43 IGKV1-5 IGKC ACTC1
4 extracellular matrix GO:0031012 9.17 TNC MMP9 MMP7 MMP2 MMP14 MMP12

Biological processes related to Light Chain Deposition Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 proteolysis GO:0006508 9.63 MMP9 MMP7 MMP2 MMP14 MMP12 MMP1
2 cellular protein metabolic process GO:0044267 9.58 TNC MMP2 MMP1
3 leukocyte migration GO:0050900 9.5 MMP1 IGKV1-5 IGKC
4 positive regulation of vascular smooth muscle cell proliferation GO:1904707 9.46 MMP9 MMP2
5 embryo implantation GO:0007566 9.43 MMP9 MMP2
6 endodermal cell differentiation GO:0035987 9.43 MMP9 MMP2 MMP14
7 extracellular matrix disassembly GO:0022617 9.43 MMP9 MMP7 MMP2 MMP14 MMP12 MMP1
8 response to hypoxia GO:0001666 9.37 MMP2 MMP14
9 collagen catabolic process GO:0030574 9.1 MMP9 MMP7 MMP2 MMP14 MMP12 MMP1

Molecular functions related to Light Chain Deposition Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 hydrolase activity GO:0016787 9.88 MMP9 MMP7 MMP2 MMP14 MMP12 MMP1
2 zinc ion binding GO:0008270 9.85 MMP9 MMP7 MMP2 MMP14 MMP12 MMP1
3 peptidase activity GO:0008233 9.73 MMP9 MMP7 MMP2 MMP14 MMP12 MMP1
4 metallopeptidase activity GO:0008237 9.63 MMP9 MMP7 MMP2 MMP14 MMP12 MMP1
5 endopeptidase activity GO:0004175 9.62 MMP9 MMP14 MMP12 MMP1
6 metalloendopeptidase activity GO:0004222 9.43 MMP9 MMP7 MMP2 MMP14 MMP12 MMP1
7 serine-type endopeptidase activity GO:0004252 8.92 MMP9 MMP7 MMP2 MMP1

Sources for Light Chain Deposition Disease

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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