MCID: LGH004
MIFTS: 47

Light Chain Deposition Disease

Categories: Rare diseases, Nephrological diseases, Blood diseases

Aliases & Classifications for Light Chain Deposition Disease

MalaCards integrated aliases for Light Chain Deposition Disease:

Name: Light Chain Deposition Disease 49 55
Light-Chain Deposition Disease 49
Light Chain Disease 69
Lcdd 55

Classifications:



External Ids:

Orphanet 55 ORPHA93558
UMLS via Orphanet 70 C0238239
ICD10 via Orphanet 33 D89.8
UMLS 69 C0238239

Summaries for Light Chain Deposition Disease

NIH Rare Diseases : 49 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. Last updated: 8/2/2016

MalaCards based summary : Light Chain Deposition Disease, also known as light-chain deposition disease, is related to lacrimal duct defect and multicentric osteolysis, nodulosis, and arthropathy. An important gene associated with Light Chain Deposition Disease is MMP7 (Matrix Metallopeptidase 7), and among its related pathways/superpathways are Integrin Pathway and Degradation of the extracellular matrix. The drugs Dexamethasone and Melphalan have been mentioned in the context of this disorder. Affiliated tissues include kidney, liver and heart, and related phenotype is homeostasis/metabolism.

Wikipedia : 72 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 112)
# Related Disease Score Top Affiliating Genes
1 lacrimal duct defect 11.6
2 multicentric osteolysis, nodulosis, and arthropathy 10.4 MMP14 MMP2
3 winchester syndrome 10.4 MMP14 MMP2
4 extracranial arteriovenous malformation 10.4 MMP2 MMP9
5 lentigo maligna melanoma 10.4 MMP2 MMP9
6 villous adenocarcinoma 10.4 MMP2 MMP9
7 amyloidosis 10.4
8 psammomatous meningioma 10.4 MMP2 MMP9
9 acute transverse myelitis 10.4 MMP2 MMP9
10 brain glioblastoma multiforme 10.4 MMP7 MMP9
11 mid-dermal elastolysis 10.4 MMP12 MMP7
12 parotitis 10.4 MMP2 MMP9
13 pediatric multiple sclerosis 10.3 MMP7 MMP9
14 conjunctival nevus 10.3 MMP2 MMP9
15 microcystic meningioma 10.3 MMP2 MMP9
16 hypercementosis 10.3 MMP2 MMP9
17 corneal ulcer 10.3 MMP2 MMP9
18 myeloma, multiple 10.3
19 sorsby fundus dystrophy 10.3 MMP2 MMP9
20 nasal cavity squamous cell carcinoma 10.3 MMP2 MMP9
21 transverse myelitis 10.3 MMP2 MMP9
22 lung giant cell carcinoma 10.2 MMP2 MMP9
23 focal myositis 10.2 MMP2 MMP7 MMP9
24 senile ectropion 10.2 MMP2 MMP7 MMP9
25 salivary gland cancer 10.2 MMP2 MMP7 MMP9
26 septic arthritis 10.2 MMP2 MMP7
27 gastrointestinal system cancer 10.2 MMP2 MMP7 MMP9
28 klatskin's tumor 10.2 MMP14 MMP2 MMP9
29 cryptogenic organizing pneumonia 10.2 MMP9 TNC
30 fibromatosis, gingival, 1 10.2 MMP1 MMP2
31 gliosarcoma 10.2 ACTC1 MMP2 MMP9
32 endometrial stromal sarcoma 10.1 ACTC1 MMP2 MMP9
33 chronic actinic dermatitis 10.1 MMP1 MMP9
34 conjunctivochalasis 10.1 MMP1 MMP2
35 sialadenitis 10.1 MMP2 TNC
36 parotid gland cancer 10.1 MMP14 MMP7
37 squamous cell carcinoma of the hypopharynx 10.1 MMP1 MMP9
38 malignant glioma 10.1 MMP14 MMP2 MMP9
39 chromoblastomycosis 10.1 MMP1 MMP9
40 lymphoma 10.1
41 actinic keratosis 10.1 MMP14 MMP2 TNC
42 hepatitis 10.1
43 retinitis 10.1
44 malignant giant cell tumor of soft parts 10.0 ACTC1 CD68
45 nodular malignant melanoma 10.0 MMP1 MMP14 MMP2
46 al amyloidosis 10.0
47 lymphangioleiomyomatosis 10.0 MMP1 MMP14 MMP2
48 adult astrocytic tumour 10.0 MMP1 MMP7 MMP9
49 blepharochalasis 10.0 CD68 MMP9
50 adenomyosis 10.0 MMP1 MMP2 MMP9

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:

43
# Description MGI Source Accession Score Top Affiliating Genes
1 homeostasis/metabolism MP:0005376 9.23 MMP12 MMP14 MMP2 MMP7 MMP9 TNC

Drugs & Therapeutics for Light Chain Deposition Disease

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

(show all 45)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Dexamethasone Approved, Investigational, Vet_approved Phase 3,Phase 2,Phase 1 50-02-2 5743
2
Melphalan Approved Phase 3,Phase 2 148-82-3 460612 4053
3
Bortezomib Approved, Investigational Phase 3,Phase 2,Phase 1 179324-69-7 387447 93860
4
Mechlorethamine Approved, Investigational Phase 3,Phase 2 51-75-2 4033
5
Lenalidomide Approved Phase 3 191732-72-6 216326
6 Dexamethasone acetate Phase 3,Phase 2,Phase 1 1177-87-3
7 Alkylating Agents Phase 3,Phase 2
8 glucocorticoids Phase 3,Phase 2,Phase 1
9
protease inhibitors Phase 3,Phase 2,Phase 1
10 Gastrointestinal Agents Phase 3,Phase 2,Phase 1
11 HIV Protease Inhibitors Phase 3,Phase 2,Phase 1
12 Hormone Antagonists Phase 3,Phase 2,Phase 1
13 Angiogenesis Inhibitors Phase 3,Phase 2,Phase 1
14 Hormones Phase 3,Phase 2,Phase 1
15 Angiogenesis Modulating Agents Phase 3,Phase 2,Phase 1
16 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 3,Phase 2,Phase 1
17 Peripheral Nervous System Agents Phase 3,Phase 2,Phase 1
18 Antiemetics Phase 3,Phase 2,Phase 1
19 Immunosuppressive Agents Phase 3,Phase 2,Phase 1
20 Anti-Inflammatory Agents Phase 3,Phase 2,Phase 1
21 Antineoplastic Agents, Hormonal Phase 3,Phase 2,Phase 1
22 BB 1101 Phase 3,Phase 2,Phase 1
23 Autonomic Agents Phase 3,Phase 2,Phase 1
24 Anti-Bacterial Agents Phase 3,Phase 2,Phase 1
25 Anti-Infective Agents Phase 3,Phase 2,Phase 1
26 phenylalanine Nutraceutical Phase 3,Phase 2
27
Doxorubicin Approved, Investigational Phase 2 23214-92-8 31703
28
Etoposide Approved Phase 2 33419-42-0 36462
29
Cisplatin Approved Phase 2 15663-27-1 2767 441203 84093
30
Cyclophosphamide Approved, Investigational Phase 2 50-18-0, 6055-19-2 2907
31
Lenograstim Approved, Investigational Phase 2 135968-09-1
32
Doxil Approved June 1999 Phase 2 31703
33 Myeloma Proteins Phase 2
34 Topoisomerase Inhibitors Phase 2
35 Dexamethasone 21-phosphate Phase 2
36 Paraproteins Phase 2
37 Immunoglobulins Phase 2
38 Etoposide phosphate Phase 2
39 Antibiotics, Antitubercular Phase 2
40 Antibodies Phase 2
41 Antineoplastic Agents, Phytogenic Phase 2
42 Antirheumatic Agents Phase 2
43 Adjuvants, Immunologic Phase 2
44
Sunitinib Approved, Investigational Phase 1 341031-54-7, 557795-19-4 5329102
45 Pomalidomide Approved Phase 1 19171-19-8

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Melphalan and Dexamethasone With or Without Bortezomib in Treating Patients With Previously Untreated Systemic Light-Chain Amyloidosis Completed NCT01078454 Phase 3 melphalan;dexamethasone;bortezomib
2 Lenalidomide or Observation in Treating Patients With Asymptomatic High-Risk Smoldering Multiple Myeloma Active, not recruiting NCT01169337 Phase 3 Lenalidomide
3 Bortezomib, Melphalan, and Dexamethasone in Treating Patients With Primary Amyloidosis or Light Chain Deposition Disease Active, not recruiting NCT00520767 Phase 2 bortezomib;dexamethasone;melphalan
4 Autologous Stem Cell Transplant Followed By Maintenance Therapy in Treating Elderly Patients With Multiple Myeloma Active, not recruiting NCT01849783 Phase 2 dexamethasone;cisplatin;doxorubicin;cyclophosphamide;etoposide;bortezomib;thalidomide;melphalan
5 HD Melphalan and SCT in Patients With IGDD or LCDD Terminated NCT00681044 Phase 2 melphalan
6 Viral Therapy in Treating Patients With Relapsed or Refractory Multiple Myeloma Completed NCT01533194 Phase 1
7 Sunitinib Malate in Treating HIV-Positive Patients With Cancer Receiving Antiretroviral Therapy Completed NCT00890747 Phase 1 sunitinib malate
8 First-line Pomalidomide, Bortezomib, and Dexamethasone For AL Amyloidosis or LCDD Suspended NCT01728259 Phase 1 pomalidomide;bortezomib;dexamethasone
9 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 Active, not recruiting NCT01383759 Bortezomib/Dexamethasone (BD), Followed By Autologous STC & Maintenance Bortezomib/Dexamethasone
10 Detecting Early Myocardial Infiltration w/Amyloid & Light Chain Deposition Disease in Multiple Myeloma Subjects 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:

38
Kidney, Liver, Heart, Lung, B Cells, Skin, Brain

Publications for Light Chain Deposition Disease

Articles related to Light Chain Deposition Disease:

(show top 50) (show all 173)
# Title Authors Year
1
Successful treatment of nephrotic syndrome induced by lambda light chain deposition disease using lenalidomide: A case report andA review of the literaturea8c. ( 29393843 )
2018
2
Light Chain Deposition Disease with Bullous Skin Lesions Mimicking Atypical Bullous Pemphigoid. ( 29135016 )
2018
3
Primary Central Nervous System Lymphoma With Light Chain Deposition Disease (Aggregoma). ( 29280764 )
2017
4
Cardiac light-chain deposition disease relapsing in the transplanted heart. ( 28562083 )
2017
5
Primary CNS Nonamyloidogenic Light Chain Deposition Disease: Case Report and Brief Review. ( 28669250 )
2017
6
Cutaneous Light Chain Deposition Disease: A Report of 2 Cases and Review of the Literature. ( 28953013 )
2017
7
Detection and Treatment of Monoclonal Gammopathy of Renal Significance With Progression to Light Chain Deposition Disease. ( 28809601 )
2017
8
Light Chain Deposition Disease Diagnosed with Laser Micro-dissection, Liquid Chromatography, and Tandem Mass Spectrometry of Nodular Glomerular Lesions. ( 28050001 )
2017
9
Clinical characteristics and prognostic factors in multiple myeloma patients with light chain deposition disease. ( 28383130 )
2017
10
Treatment of Light Chain Deposition Disease Using Bortezomib-Based Regimen Followed by Thalidomide-Based Regimen in a Saudi Male. ( 28083151 )
2016
11
Five Sequential Evaluations of Renal Histology in a Patient with Light Chain Deposition Disease. ( 27746438 )
2016
12
A case of light chain deposition disease in kidney in a known case of multiple myeloma. ( 27728501 )
2016
13
Bortezomib Based Chemotherapy for Light Chain Deposition Disease. ( 27762125 )
2016
14
Light Chain Deposition Disease in an Older Adult Patient Successfully Treated with Long-term Administration of Bortezomib, Melphalan and Prednisone. ( 27181540 )
2016
15
Treatment of idiopathic light chain deposition disease: complete remission with bortezomib and dexamethasone. ( 28001187 )
2016
16
Unusual case of light chain deposition disease kappa restricted in a case of multiple myeloma. ( 27728483 )
2016
17
Nodular pulmonary light chain deposition disease. ( 27306574 )
2016
18
Spontaneous splenic rupture in a patient with light-chain deposition disease undergoing autologous peripheral blood stem cell transplantation. ( 27384856 )
2016
19
A Case of Cardiac Light Chain Deposition Disease in a Patient with Solitary Plasmacytoma. ( 26988342 )
2016
20
ATYPICAL MACULOPATHY IN A PATIENT WITH LIGHT CHAIN DEPOSITION DISEASE MIMICKING ADVANCED GEOGRAPHIC ATROPHY. ( 26934302 )
2016
21
Surgical Management of Pulmonary Mucosa-Associated Lymphoid Tissue Lymphoma Associated With Light-Chain Deposition Disease. ( 27211983 )
2016
22
Unusual Presentation of Light Chain Deposition Disease: A Case Report. ( 27437235 )
2016
23
Intracystic hemorrhage in a patient with pulmonary cystic disorder related to light-chain deposition disease. ( 27756655 )
2016
24
Light Chain Deposition Disease After Kidney Transplantation With Long Graft Survival: Case Report. ( 26915878 )
2016
25
Animal Models of Light Chain Deposition Disease Provide a Better Understanding of Nodular Glomerulosclerosis. ( 26794829 )
2016
26
Nodular pulmonary light chain deposition disease: an entity associated with SjAPgren syndrome or marginal zone lymphoma. ( 26502747 )
2016
27
Clinicopathological characteristics and outcomes of light chain deposition disease: an analysis of 48 patients in a single Chinese center. ( 27056200 )
2016
28
OCULAR MANIFESTATIONS OF MONOCLONAL IMMUNOGLOBULIN LIGHT CHAIN DEPOSITION DISEASE. ( 27315323 )
2016
29
Can We Cure Light Chain Deposition Disease of the Kidneys?-A Review and Case Report of a Patient Treated With a Triple Transplant Approach. ( 27101986 )
2016
30
Nonamyloid tumoral light-chain-deposition disease (aggregoma) of the paraspinal region. ( 26253133 )
2015
31
Monoclonal gammopathy of renal significance with light-chain deposition disease diagnosed postrenal transplant: a diagnostic and therapeutic challenge. ( 25441103 )
2015
32
Natural history and outcome of light chain deposition disease. ( 26392598 )
2015
33
Blood stem cell transplantation to treat cystic lung light chain deposition disease. ( 26160867 )
2015
34
Light-Chain Deposition Disease Successfully Treated with Bortezomib in an Elderly Patient: A Case Report and Review of the Literature. ( 26568005 )
2015
35
Enlightening light chain deposition disease. ( 26705338 )
2015
36
AJKD Atlas of Renal Pathology: Light Chain Deposition Disease. ( 26593321 )
2015
37
ENHANCED DEPTH IMAGING SPECTRAL DOMAIN OPTICAL COHERENCE TOMOGRAPHY FEATURES OF LIGHT CHAIN DEPOSITION DISEASE. ( 26398301 )
2015
38
Clinical and Prognostic Differences among Patients with Light Chain Deposition Disease, Myeloma Cast Nephropathy and Both. ( 25860232 )
2015
39
Pulmonary light-chain deposition disease: CT and pathology findings in nine patients. ( 25659938 )
2015
40
Hypertension, renal failure, and edema in a 38-year-old man: light chain deposition disease; a case report and review of the literature. ( 24772399 )
2014
41
Light chain deposition disease without glomerular proteinuria: a diagnostic challenge for the nephrologist. ( 24619059 )
2014
42
Light chain deposition disease and proximal tubulopathy in two successive kidney allografts. ( 25492671 )
2014
43
Isolated intracerebral light chain deposition disease: novel imaging and pathologic findings. ( 25084689 )
2014
44
Late recurrence of light chain deposition disease after kidney transplantation treated with bortezomib: a case report. ( 25249814 )
2014
45
Osteolytic femoral lesion detected by sonography in a case of light chain deposition disease. ( 24449208 )
2014
46
Education and imaging. Hepatobiliary and Pancreatic: Portal hypertension due to hepatic light chain deposition disease. ( 24832781 )
2014
47
Hematologic response and stabilization of renal function in a patient with light chain deposition disease after lenalidomide treatment: a novel therapeutic approach? ( 25022598 )
2014
48
Retinal pigment epithelial detachments and tears, and progressive retinal degeneration in light chain deposition disease. ( 23385633 )
2013
49
Portal hypertension related to light chain deposition disease of liver: an enlightening experience. ( 23723105 )
2013
50
Light chain deposition disease; there are reasons for confusion. ( 25340150 )
2013

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 22)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.11 MMP1 MMP12 MMP2 MMP7 MMP9 TNC
2
Show member pathways
12.46 MMP1 MMP12 MMP14 MMP2 MMP7 MMP9
3
Show member pathways
11.95 MMP1 MMP12 MMP2 MMP7 MMP9 TNC
4 11.86 MMP1 MMP12 TNC
5 11.85 MMP1 MMP12 MMP14 MMP2 MMP7 MMP9
6 11.81 MMP14 MMP2 MMP9
7 11.77 MMP1 MMP2 MMP9
8 11.68 MMP2 MMP7 MMP9 TNC
9 11.56 MMP1 MMP2 MMP9
10
Show member pathways
11.49 MMP1 MMP2 MMP9
11 11.44 MMP14 MMP2 MMP7 MMP9
12
Show member pathways
11.43 MMP1 MMP12 MMP14 MMP2 MMP7 MMP9
13
Show member pathways
11.26 MMP2 MMP9
14 11.24 MMP1 MMP9 TNC
15 11.16 MMP1 MMP2 MMP9
16 11.1 MMP2 MMP9
17 11.08 MMP9 TNC
18 11 MMP2 MMP9
19 10.94 MMP2 MMP9
20 10.89 MMP1 MMP2
21 10.89 MMP1 MMP12 MMP14 MMP2 MMP7 MMP9
22 10.81 MMP1 MMP7 MMP9

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 9.86 IGKC IGKV1-5 MMP1 MMP12 MMP2 MMP7
2 extracellular space GO:0005615 9.76 ACTC1 IGKC MMP12 MMP14 MMP2 MMP7
3 blood microparticle GO:0072562 9.5 ACTC1 IGKC IGKV1-5
4 proteinaceous extracellular matrix GO:0005578 9.43 MMP1 MMP12 MMP2 MMP7 MMP9 TNC
5 extracellular matrix GO:0031012 9.17 MMP1 MMP12 MMP14 MMP2 MMP7 MMP9

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 9.76 IGKC IGKV1-5 MMP1 MMP12 MMP14 MMP2
2 leukocyte migration GO:0050900 9.62 IGKC IGKV1-5 MMP1 MMP9
3 cellular protein metabolic process GO:0044267 9.58 MMP1 MMP2 TNC
4 embryo implantation GO:0007566 9.43 MMP2 MMP9
5 endodermal cell differentiation GO:0035987 9.43 MMP14 MMP2 MMP9
6 extracellular matrix disassembly GO:0022617 9.43 MMP1 MMP12 MMP14 MMP2 MMP7 MMP9
7 positive regulation of vascular smooth muscle cell proliferation GO:1904707 9.37 MMP2 MMP9
8 collagen catabolic process GO:0030574 9.1 MMP1 MMP12 MMP14 MMP2 MMP7 MMP9

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 MMP1 MMP12 MMP14 MMP2 MMP7 MMP9
2 zinc ion binding GO:0008270 9.8 MMP1 MMP12 MMP14 MMP2 MMP7 MMP9
3 peptidase activity GO:0008233 9.73 MMP1 MMP12 MMP14 MMP2 MMP7 MMP9
4 metallopeptidase activity GO:0008237 9.63 MMP1 MMP12 MMP14 MMP2 MMP7 MMP9
5 endopeptidase activity GO:0004175 9.54 MMP1 MMP12 MMP9
6 metalloendopeptidase activity GO:0004222 9.43 MMP1 MMP12 MMP14 MMP2 MMP7 MMP9
7 serine-type endopeptidase activity GO:0004252 9.23 IGKC IGKV1-5 MMP1 MMP12 MMP14 MMP2

Sources for Light Chain Deposition Disease

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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