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MCID: CST005
MIFTS: 59

Castleman Disease

Categories: Blood diseases, Immune diseases, Rare diseases
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Aliases & Classifications for Castleman Disease

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MalaCards integrated aliases for Castleman Disease:

Name: Castleman Disease 11 19 58 53 43 14 75
Angiofollicular Ganglionic Hyperplasia 19 58 43 71
Angiofollicular Lymph Hyperplasia 11 19 58
Giant Lymph Node Hyperplasia 11 19 16
Castleman's Disease 75 14
Angiofollicular Lymph Node Hyperplasia 11
Angiolymphoid Hyperplasia 71
Benign Giant Lymphoma 19
Lymphoid Hamartoma 11

Characteristics:


Prevelance:

1-9/100000 (United States) 58

Age Of Onset:

All ages 58

Classifications:

MalaCards categories:
Global: Rare diseases
Anatomical: Blood diseases Immune diseases
See all MalaCards categories (disease lists)
ICD10: 32
Orphanet: 58  
Rare haematological diseases


External Ids:

Disease Ontology 11 DOID:0111157
NCIt 49 C3056
SNOMED-CT 68 207036003
MESH via Orphanet 44 C536362 D005871
ICD10 via Orphanet 32 D47.7
UMLS via Orphanet 72 C0017531 C2931179
Orphanet 58 ORPHA160
UMLS 71 C0017531 C2931179
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Summaries for Castleman Disease

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GARD: 19 Castleman disease (CD) is a rare condition that affects the lymph nodes and related tissues. There are two main forms: unicentric CD and multicentric CD. Unicentric CD is a "localized" condition that is generally confined to a single set of lymph nodes, while multicentric CD is a "systemic" disease that affects multiple sets of lymph nodes and other tissues throughout the body. The exact underlying cause of CD is currently unknown; however, it is thought to occur sporadically in people with no family history of the condition.

MalaCards based summary: Castleman Disease, also known as angiofollicular ganglionic hyperplasia, is related to multicentric castleman disease and igg4-related disease. An important gene associated with Castleman Disease is IL6 (Interleukin 6), and among its related pathways/superpathways are Innate Immune System and ERK Signaling. The drugs Thalidomide and Zanubrutinib have been mentioned in the context of this disorder. Affiliated tissues include lymph node, bone marrow and bone, and related phenotypes are fatigue and anemia

Orphanet: 58 A benign lymphoproliferative disorder that may present as a localized or multicentric form. The clinical manifestations are heterogeneous, ranging from asymptomatic discrete lymphadenopathy to recurrent episodes of diffuse lymphadenopathy with severe systemic symptoms.

Disease Ontology: 11 A lymphoproliferative syndrome characterized by one or more enlarged lymph nodes containing cells with hyaline-vascular, plasmacytic, or mixed appearance microscopically.

Wikipedia: 75 Castleman disease (CD) describes a group of rare lymphoproliferative disorders that involve enlarged... more...
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Related Diseases for Castleman Disease

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Diseases related to Castleman Disease via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 948)
# Related Disease Score Top Affiliating Genes
1 multicentric castleman disease 33.2 IL6R IL6 IL10 CRP CCR6
2 igg4-related disease 31.2 IL10 CRP CCR6
3 polyclonal hypergammaglobulinemia 31.2 IL6 CXCL13 CRP CCR6
4 bronchiolitis obliterans 31.1 IL6 IL10 IFNG CRP
5 anemia, autoimmune hemolytic 31.0 IL6 IL10 CRP CCR6
6 bronchiolitis 30.9 IL6 IL10 IFNG CRP CCL4
7 exanthem 30.9 MTOR IL6 IL10 IFNG CRP CCR6
8 deficiency anemia 30.9 IL6 IL10 CRP CR1 CCR6
9 inflammatory bowel disease 1 30.8 IL6 IL10 CCR6
10 plasmacytoma 30.8 MTOR IL6ST IL6R IL6
11 acquired immunodeficiency syndrome 30.8 IL6 IL10 IFNG CRP
12 glomerulonephritis 30.7 IL6 IL10 CRP CR1
13 back pain 30.7 IL6 IL10 CRP
14 autoimmune pancreatitis 30.7 IL10 CRP CCR6
15 myasthenia gravis 30.7 IL6 IL10 IFNG CR1
16 infective endocarditis 30.7 IL6 CRP CCR6
17 colitis 30.7 IL6 IL10 IFNG CRP CCR6
18 systemic scleroderma 30.7 IL6 IL10 CRP CCR6
19 brucellosis 30.7 IL6 IL10 IFNG CRP
20 autoimmune disease 30.7 IL6 IL10 IFNG DSG3 CRP CCR6
21 lymphoid interstitial pneumonia 30.7 MTOR IL6 IFNG CRP
22 ulcerative colitis 30.7 IL6 IL10 IFNG CRP CCR6
23 stomatitis 30.6 MTOR IL6 IL10 IFNG
24 hypersensitivity vasculitis 30.6 IL6 CRP CCR6
25 lymphadenitis 30.6 IL6 IL10 IFNG CRP CCR6
26 polyradiculoneuropathy 30.6 IL10 IFNG CCL4
27 aspergillosis 30.5 IL6 IL10 IFNG CCR6
28 spondyloarthropathy 1 30.5 IL6 IL10 CRP CCR6
29 peritonitis 30.5 IL6 IL10 IFNG CRP CR1
30 follicular lymphoma 30.5 MTOR MIR210 FCER2 CR1
31 crohn's disease 30.5 IL6 IL10 IFNG CRP CCR6
32 myeloma, multiple 30.5 MTOR IL6ST IL6R IL6 CCR6 CCL4
33 lichen planus 30.5 IL6 IL10 IFNG DSG3
34 cellulitis 30.5 IL6 CRP CCR6
35 henoch-schoenlein purpura 30.5 IL6 CRP CCR6
36 immune deficiency disease 30.5 MIR210 IL6R IL6 IL10 IFNG CRP
37 pneumonia 30.5 IL6 IL10 IFNG CRP
38 celiac disease 1 30.5 IL6 IL10 IFNG CRP CCR6
39 thyroiditis 30.4 IL6 IL10 IFNG CRP
40 uveitis 30.4 IL6 IL10 IFNG CCL4
41 temporal arteritis 30.4 IL6R IL6 IL10 IFNG CRP CCR6
42 familial mediterranean fever 30.4 IL6 IL10 CRP
43 optic nerve disease 30.4 IL6 IL10 CRP CCR6
44 lymphopenia 30.4 IL6 IL10 IFNG CCR6
45 sarcoidosis 1 30.3 IL6 IL10 IFNG CRP CCR6 CCL4
46 retinal detachment 30.3 IL6 IL10 IFNG CCL4
47 enterocolitis 30.3 IL6 IL10 CRP
48 interstitial lung disease 30.3 IL6 IL10 IFNG CRP CCR6
49 lung abscess 30.3 IL6 IFNG CRP
50 hemophagocytic lymphohistiocytosis 30.3 IL6 IL10 IFNG CRP CCR6

Graphical network of the top 20 diseases related to Castleman Disease:



Diseases related to Castleman Disease
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Symptoms & Phenotypes for Castleman Disease

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Human phenotypes related to Castleman Disease:

58 30 (show all 31)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 fatigue 58 30 Frequent (33%) Frequent (79-30%)
 HP:0012378
2 anemia 58 30 Frequent (33%) Frequent (79-30%)
 HP:0001903
3 abdominal pain 58 30 Frequent (33%) Frequent (79-30%)
 HP:0002027
4 weight loss 58 30 Frequent (33%) Frequent (79-30%)
 HP:0001824
5 mediastinal lymphadenopathy 58 30 Frequent (33%) Frequent (79-30%)
 HP:0100721
6 elevated erythrocyte sedimentation rate 58 30 Frequent (33%) Frequent (79-30%)
 HP:0003565
7 follicular hyperplasia 58 30 Frequent (33%) Frequent (79-30%)
 HP:0002729
8 elevated circulating c-reactive protein concentration 30 Frequent (33%) HP:0011227
9 increased circulating interleukin 6 concentration 30 Frequent (33%) HP:0030783
10 nausea and vomiting 58 30 Occasional (7.5%) Occasional (29-5%)
 HP:0002017
11 jaundice 58 30 Occasional (7.5%) Occasional (29-5%)
 HP:0000952
12 cough 58 30 Occasional (7.5%) Occasional (29-5%)
 HP:0012735
13 abdominal distention 58 30 Occasional (7.5%) Occasional (29-5%)
 HP:0003270
14 generalized lymphadenopathy 58 30 Occasional (7.5%) Occasional (29-5%)
 HP:0008940
15 decreased mean corpuscular volume 58 30 Occasional (7.5%) Occasional (29-5%)
 HP:0025066
16 abdominal mass 58 30 Occasional (7.5%) Occasional (29-5%)
 HP:0031500
17 flank pain 58 30 Occasional (7.5%) Occasional (29-5%)
 HP:0030157
18 renal insufficiency 58 30 Very rare (1%) Very rare (<4-1%)
 HP:0000083
19 thrombocytopenia 58 30 Very rare (1%) Very rare (<4-1%)
 HP:0001873
20 hematuria 58 30 Very rare (1%) Very rare (<4-1%)
 HP:0000790
21 dyspnea 58 30 Very rare (1%) Very rare (<4-1%)
 HP:0002094
22 intestinal obstruction 58 30 Very rare (1%) Very rare (<4-1%)
 HP:0005214
23 restrictive cardiomyopathy 58 30 Very rare (1%) Very rare (<4-1%)
 HP:0001723
24 myelofibrosis 58 30 Very rare (1%) Very rare (<4-1%)
 HP:0011974
25 ureteral obstruction 58 30 Very rare (1%) Very rare (<4-1%)
 HP:0006000
26 anasarca 58 30 Very rare (1%) Very rare (<4-1%)
 HP:0012050
27 abnormality of the gastrointestinal tract 58 Occasional (29-5%)
28 lymphadenopathy 58 Very frequent (99-80%)
29 elevated c-reactive protein level 58 Frequent (79-30%)
30 constitutional symptom 58 Frequent (79-30%)
31 increased serum interleukin-6 58 Frequent (79-30%)

GenomeRNAi Phenotypes related to Castleman Disease according to GeneCards Suite gene sharing:

25 (show all 25)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased shRNA abundance (Z-score > 2) GR00366-A-100 9.74 IL10
2 Increased shRNA abundance (Z-score > 2) GR00366-A-113 9.74 IL10
3 Increased shRNA abundance (Z-score > 2) GR00366-A-120 9.74 CRP
4 Increased shRNA abundance (Z-score > 2) GR00366-A-124 9.74 IL6R
5 Increased shRNA abundance (Z-score > 2) GR00366-A-125 9.74 IL10
6 Increased shRNA abundance (Z-score > 2) GR00366-A-147 9.74 CRP
7 Increased shRNA abundance (Z-score > 2) GR00366-A-148 9.74 CRP
8 Increased shRNA abundance (Z-score > 2) GR00366-A-152 9.74 IL10
9 Increased shRNA abundance (Z-score > 2) GR00366-A-160 9.74 IL6R
10 Increased shRNA abundance (Z-score > 2) GR00366-A-161 9.74 CRP IL10
11 Increased shRNA abundance (Z-score > 2) GR00366-A-198 9.74 IL10
12 Increased shRNA abundance (Z-score > 2) GR00366-A-210 9.74 IL10
13 Increased shRNA abundance (Z-score > 2) GR00366-A-214 9.74 IL10
14 Increased shRNA abundance (Z-score > 2) GR00366-A-27 9.74 IL6R
15 Increased shRNA abundance (Z-score > 2) GR00366-A-30 9.74 GSK3B
16 Increased shRNA abundance (Z-score > 2) GR00366-A-37 9.74 IL6R
17 Increased shRNA abundance (Z-score > 2) GR00366-A-40 9.74 IL6R
18 Increased shRNA abundance (Z-score > 2) GR00366-A-52 9.74 IL10
19 Increased shRNA abundance (Z-score > 2) GR00366-A-53 9.74 IL10
20 Increased shRNA abundance (Z-score > 2) GR00366-A-63 9.74 CRP
21 Increased shRNA abundance (Z-score > 2) GR00366-A-65 9.74 IL10
22 Increased shRNA abundance (Z-score > 2) GR00366-A-67 9.74 IL10
23 Increased shRNA abundance (Z-score > 2) GR00366-A-68 9.74 IL10
24 Increased shRNA abundance (Z-score > 2) GR00366-A-82 9.74 IL10
25 Increased shRNA abundance (Z-score > 2) GR00366-A-85 9.74 IL10

MGI Mouse Phenotypes related to Castleman Disease:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 homeostasis/metabolism MP:0005376 10.28 AR CCR6 CR1 CRP DSG3 FCER2
2 nervous system MP:0003631 10.23 AR CCR6 CR1 FSCN1 GSK3B IFNG
3 normal MP:0002873 10.13 AR CCR6 GSK3B IFNG IL10 IL6R
4 cellular MP:0005384 10.13 AR CXCL13 DSG3 FCER2 FSCN1 GSK3B
5 immune system MP:0005387 10.09 AR CCR6 CR1 CRP CXCL13 DSG3
6 digestive/alimentary MP:0005381 10.07 AR DSG3 FCER2 GSK3B IFNG IL10
7 renal/urinary system MP:0005367 10.03 AR CR1 GSK3B IFNG IL6 MIR210
8 cardiovascular system MP:0005385 10 AR CCR6 CR1 CRP GSK3B IFNG
9 hematopoietic system MP:0005397 9.83 AR CCR6 CR1 CXCL13 DSG3 FCER2
10 mortality/aging MP:0010768 9.47 AR CR1 DSG3 FCER2 FSCN1 GSK3B
Sources

Drugs & Therapeutics for Castleman Disease

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Drugs for Castleman Disease (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 61)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Thalidomide Approved, Investigational, Withdrawn Phase 2 50-35-1 5426
2
Zanubrutinib Approved, Investigational Phase 2 1691249-45-2 135565884
3
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 2 1177-87-3 3680
4
Dexamethasone Approved, Investigational, Vet_approved Phase 2 50-02-2 3003 5743
5
Zidovudine Approved Phase 2 30516-87-1 35370
6
Sirolimus Approved, Investigational Phase 2 53123-88-9 5284616 6436030
7
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
8
Clotrimazole Approved, Vet_approved Phase 2 23593-75-1 2812
9
Lenograstim Approved, Investigational Phase 2 135968-09-1
10
Bortezomib Approved, Investigational Phase 2 179324-69-7 387447 93860
11
Ganciclovir Approved, Investigational Phase 2 82410-32-0 3454
12
Siltuximab Approved, Investigational Phase 2 541502-14-1
13
Suramin Investigational Phase 2 129-46-4, 145-63-1 5361
14 Analgesics Phase 2
15 Antipyretics Phase 2
16 Histamine H1 Antagonists Phase 2
17 Histamine Antagonists Phase 2
18 Antiprotozoal Agents Phase 2
19 Anthelmintics Phase 2
20 Antiparasitic Agents Phase 2
21 Pharmaceutical Solutions Phase 2
22 Chrysarobin Phase 2
23 Immunoglobulins, Intravenous Phase 2
24 Protein Kinase Inhibitors Phase 2
25 Anti-Retroviral Agents Phase 2
26 Anti-HIV Agents Phase 2
27 Antimetabolites Phase 2
28 Reverse Transcriptase Inhibitors Phase 2
29 Antifungal Agents Phase 2
30 interferons Phase 2
31 Interferon-alpha Phase 2
32 Ganciclovir triphosphate Phase 2
33 Immunoglobulins Phase 2
34 Antibodies, Monoclonal Phase 2
35 Antibodies Phase 2
36
Picropodophyllin Approved, Investigational Phase 1 518-28-5, 477-47-4 10607 72435
37
Levoleucovorin Approved, Experimental, Investigational Phase 1 68538-85-2, 58-05-9, 73951-54-9 149436 6006
38
Methotrexate Approved Phase 1 1959-05-2, 59-05-2 4112 126941
39
Daunorubicin Approved Phase 1 20830-81-3 30323
40
Cetuximab Approved Phase 1 205923-56-4
41
Valproic acid Approved, Investigational Phase 1 99-66-1 3121
42
Bevacizumab Approved, Investigational Phase 1 216974-75-3 135329020
43
Folic acid Approved, Nutraceutical, Vet_approved Phase 1 59-30-3 6037
44 Folic Acid Antagonists Phase 1
45 Folate Phase 1
46 Vitamin B9 Phase 1
47 Keratolytic Agents Phase 1
48 Vitamin B Complex Phase 1
49 Dermatologic Agents Phase 1
50 Neurotransmitter Agents Phase 1

Interventional clinical trials:

(show all 35)
# Name Status NCT ID Phase Drugs
1 Clinical and Virologic Response to HHV-8 Associated Multicentric Castleman's Disease to Valganciclovir Withdrawn NCT00361933 Phase 4 Valganciclovir
2 Thalidomide, Cyclophosphamide and Prednisone in Newly Diagnosed Multicentric Castleman's Disease: a Prospective, Single-center, Single-arm, Phase-II Pilot Trial Unknown status NCT03043105 Phase 2 Thalidomide, cyclophosphamide and prednisone
3 Pilot Study of Tocilizumab in Patients With Symptomatic Kaposi Sarcoma Herpesvirus (KSHV) - Associated Multicentric Castleman Disease Completed NCT01441063 Phase 2 Zidovudine;Tocilizumab;Valganciclovir (VGC)
4 A Randomized, Double Blind, Placebo Controlled Study to Assess the Efficacy and Safety of CNTO 328 (Anti IL 6 Monoclonal Antibody) Plus Best Supportive Care Compared With Best Supportive Care in Subjects With Multicentric Castleman's Disease Completed NCT01024036 Phase 2 Siltuximab;Placebo;Best Supportive Care (BSC)
5 A PHASE II PILOT STUDY OF SURAMIN IN PREVIOUSLY TREATED PATIENTS WITH MULTIPLE MYELOMA AND PATIENTS WITH CASTLEMAN'S DISEASE Completed NCT00002652 Phase 2 suramin
6 An Open-label, Multicenter Study to Evaluate the Safety of Long-term Treatment With Siltuximab in Subjects With Multicentric Castleman's Disease Completed NCT01400503 Phase 2 Siltuximab
7 LCCC 1950 - Rituximab for Multicentric Castleman Disease in Malawi, A Single-Arm Phase II Safety/Efficacy Trial Recruiting NCT04585893 Phase 2 Rituximab;Etoposide
8 The Efficacy and Safety of Zanuburutinib in Relapsed and Refractory Idiopathic Multicentric Castleman Disease (iMCD): a Prospective, Single-center, Single-arm Trial Recruiting NCT04743687 Phase 2 Zanubrutinib
9 A Phase II, Single-arm Open-label Multi-center Study of Sirolimus in Previously Treated Idiopathic Multicentric Castleman Disease Recruiting NCT03933904 Phase 2 Sirolimus
10 A Single-arm, Open-label, Multi-center Phase â…¡a Clinical Study to Evaluate the Efficacy and Safety of Recombinant Humanized Anti-IL-6R mAb Injection in the Treatment of Patients With Idiopathic Multicentric Castleman's Disease Recruiting NCT05345522 Phase 2
11 Bortezomib, Cyclophosphamide and Dexamethasone (BCD) in Newly Diagnosed Idiopathic Multicentric Castleman's Disease (iMCD) : a Prospective, Single-center, Single-arm, Phase-II Pilot Trial Recruiting NCT03982771 Phase 2 Bortezomib;Cyclophosphamide;Dexamethason
12 Targeted Oncolytic Virotherapy and Natural History Study of KSHV-Associated Multicentric Castleman's Disease With Laboratory and Clinical Correlates of Disease Activity Active, not recruiting NCT00092222 Phase 2 Etoposide;Interferon-alpha;Rituximab;Zidovudine;Liposomal Doxorubicin;Bortezomib;Valganciclovir;Doxorubicin;Vincristine;Cyclophosphamide;Filgrastim (G-CSF);Prednisone;Sirolimus
13 A Phase 2 Study of Intrapatient Siltuximab Dose Escalation in Patients With Idiopathic Multicentric Castleman Disease That Has Progressed After Prior Siltuximab Treatment Terminated NCT04838860 Phase 2 Siltuximab
14 Multicenter, Phase II Trial Assessing the Efficacy of Rituximab in HIV Infected Patients With Multicentric Castleman Disease Dependent on Chemotherapy (ANRS 117 Study, CastlemaB) Terminated NCT00127569 Phase 2 Rituximab
15 Phase I/II Study of Pomalidomide Combined With Modified DA-EPOCH and Rituximab in KSHV-Associated Lymphomas (Primary Effusion Lymphoma and Large Cell Lymphoma Arising in KSHV-Associated Multicentric Castleman Disease) Withdrawn NCT02228512 Phase 1, Phase 2 Pomalidomide;Rituximab;Prednisone;Etoposide;Doxorubicin;Vincristine;Cyclophosphamide
16 A Phase 1, Multi-Center, Open-Label, Dose-Escalation, Safety, Pharmacokinetic, and Pharmacodynamic Study of CX-4945 Administered Orally to Patients With Advanced Solid Tumors, Castleman's Disease or Multiple Myeloma Unknown status NCT00891280 Phase 1 CX-4945 oral formulation
17 A Phase 1 Study of Multiple Intravenous Administrations of a Chimeric Antibody Against Interleukin-6 (CNTO 328) in Subjects With B-Cell Non-Hodgkin's Lymphoma, Multiple Myeloma, or Castleman's Disease Completed NCT00412321 Phase 1 CNTO 328
18 Provision of Tocilizumab for Patients With Multicentric Castleman's Disease Who Have Demonstrated Benefit From Previous Tocilizumab Treatment Completed NCT01183598 Phase 1 tocilizumab [RoActemra/Actemra]
19 A Phase I Study of Subcutaneous Rituximab Hyaluronidase Combined With Local Standard-of-Care Chemotherapy for the Treatment of Burkitt Lymphoma, Diffuse Large B-Cell Lymphoma or as Monotherapy for Kaposi Sarcoma Herpesvirus Associated Multicentric Castleman Disease in Pediatrics and Adults in Uganda Recruiting NCT03864419 Phase 1 Cyclophosphamide;Vincristine;Methotrexate;Doxorubicin;Doxorubicin Hydrochloride;Prednisone;Etoposide
20 A Phase I Trial of Bevacizumab, Temsirolimus Alone and in Combination With Valproic Acid, or Cetuximab in Patients With Advanced Malignancy and Other Indications Active, not recruiting NCT01552434 Phase 1 Temsirolimus;Valproic Acid
21 Phase I and Pharmacokinetic Study of Ibrutinib in HIV-Infected Patients With Relapsed or Refractory B-Cell Non-Hodgkin Lymphoma or Multiple Myeloma Terminated NCT02109224 Phase 1 Ibrutinib
22 Castleman Disease - Diagnosis and Surgery Completed NCT05440305
23 Unlock the Cell: Intracellular Inflammatory Pathways and Flow Cytometry Study for Castleman's Disease Completed NCT02853968
24 Epithelioid Hemangioma of Bone And Soft Tissue: a Benign Tumor With Metastatic Potential Completed NCT03169595
25 Molecular Characterization of Viral-associated Tumors, Tumors Occurring in the Setting of HIV or Other Immune Disorders and Castleman Disease Recruiting NCT03300830
26 ACCELERATE (Advancing Castleman Care With an Electronic Longitudinal Registry, E-Repository, And Treatment/Effectiveness Research): An International Registry for Patients With Castleman Disease Recruiting NCT02817997
27 Exploratory Analysis of the Molecular Basis of Langerhans and Non-Langerhans Cell Histiocytic Neoplasms and Castleman Disease Recruiting NCT05028621
28 Screening for HHV8 Infection in Solid Organ Transplantation Donors, Candidates and Recipients Recruiting NCT05081141
29 Registry for Adults With Plasma Cell Disorders (PCD's) Recruiting NCT03717844
30 A Prospective Cohort Study of Patients With Plasma Cell Disorders (PCDs) in PKUFH Recruiting NCT05283993
31 Collection of Blood, Bone Marrow, Tumor, or Tissue Samples From Patients With HIV Infection, KSHV Infection, Viral-Related Pre-Malignant Lesions, and/or Cancer Recruiting NCT00006518
32 Natural History Study of KSHV-Associated Multicentric Castleman's Disease Recruiting NCT04968288
33 Assessment of Bone Mineral Density in People Living With HIV and KSHV Associated Malignancies: A Retrospective Longitudinal Analysis Active, not recruiting NCT04742387
34 A Pilot Trial of Nelfinavir for the Lytic Activation and Treatment of Gammaherpesvirus-Related Tumors Terminated NCT02080416 Early Phase 1 Nelfinavir
35 The Castleman Disease Collaborative Network Biobank: A Collection of Biospecimens and Clinical Data to Facilitate Research Withdrawn NCT02871050

Search NIH Clinical Center for Castleman Disease

Inferred drug relations via UMLS 71 / NDF-RT 50 :


siltuximab

Cochrane evidence based reviews: castleman disease

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Genetic Tests for Castleman Disease

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Anatomical Context for Castleman Disease

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Organs/tissues related to Castleman Disease:

MalaCards : Lymph Node, Bone Marrow, Bone, Endothelial, Liver, Lung, B Cells
Sources

Publications for Castleman Disease

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Articles related to Castleman Disease:

(show top 50) (show all 3914)
# Title Authors PMID Year
1
Membranoproliferative glomerulonephritis in the setting of multicentric angiofollicular lymph node hyperplasia (Castleman's disease) complicated by Evan's syndrome. 53 62
20439324 2010
2
IL-6: from its discovery to clinical applications. 53 62
20410258 2010
3
IgG4-related disease: historical overview and pathology of hematological disorders. 53 62
20403026 2010
4
Interleukin-6 as a therapeutic target in candidate inflammatory diseases. 53 62
20182422 2010
5
[What's new in internal medicine?]. 53 62
20110057 2009
6
A 5-year-old boy with unicentric Castleman disease affecting the mesentery: utility of serum IL-6 level and (18)F-FDG PET for diagnosis. 53 62
19730402 2009
7
The analysis of interleukin-6 in patients with systemic IgG4-related plasmacytic syndrome--expansion of SIPS to the territory of Castleman's disease. 53 62
19447768 2009
8
[New therapeutic strategy for autoimmune and chronic inflammatory disease based on clinical results using IL-6 blocking therapy with a humanized anti-IL-6 receptor antibody]. 53 62
19483409 2009
9
Systemic IgG4-related lymphadenopathy: a clinical and pathologic comparison to multicentric Castleman's disease. 53 62
19270642 2009
10
Synergistic effect of interleukin-6 and endoplasmic reticulum stress inducers on the high level of ABCG2 expression in plasma cells. 53 62
19139722 2009
11
IL-6R distribution in normal human and cynomolgus monkey tissues. 53 62
19010373 2009
12
[Castleman's disease, a fatal cause of lymph node enlargement and fever]. 53 62
19857312 2009
13
Mechanisms and pathologic significances in increase in serum interleukin-6 (IL-6) and soluble IL-6 receptor after administration of an anti-IL-6 receptor antibody, tocilizumab, in patients with rheumatoid arthritis and Castleman disease. 53 62
18784373 2008
14
[Immunotherapy using IL-6 receptor as the target]. 53 62
18974624 2008
15
Cutaneous castleman's disease responds to anti interleukin-6 treatment. 53 62
17766835 2007
16
Anti-interleukin 6 receptor antibody tocilizumab reduces the level of serum hepcidin in patients with multicentric Castleman's disease. 53 62
17550864 2007
17
Reversible cardiomyopathy associated with Multicentric Castleman disease: successful treatment with tocilizumab, an anti-interleukin 6 receptor antibody. 53 62
17483056 2007
18
Bone marrow findings in multicentric Castleman disease in HIV-negative patients. 53 62
17325481 2007
19
Interleukin-6-producing giant cell carcinoma of the lung with multicentric Castleman's disease-like presentation. 53 62
17527041 2007
20
Anti-interleukin-6 receptor antibody therapy in rheumatic diseases. 53 62
17214583 2006
21
Interleukin 6: from bench to bedside. 53 62
17075601 2006
22
[Anti-interleukin-6 receptor antibody therapy--from bedside to bench]. 53 62
17075187 2006
23
Tocilizumab: blockade of interleukin-6 signaling pathway as a therapeutic strategy for inflammatory disorders. 53 62
17028666 2006
24
[A case of HIV-1 and HHV-8-associated Castleman disease with a relapsing high fever and lymphoadenopathy]. 53 62
16922487 2006
25
Autoantibody production from a thymoma and a follicular dendritic cell sarcoma associated with paraneoplastic pemphigus. 53 62
16120143 2005
26
Clinical studies in patients with Castleman's disease, Crohn's disease, and rheumatoid arthritis in Japan. 53 62
16129906 2005
27
Intraventricular chordoid meningioma presenting with Castleman disease due to overproduction of interleukin-6. Case report. 53 62
15871519 2005
28
Nodular regenerative hyperplasia of the liver and Castleman's disease: potential role of interleukin-6. 53 62
15745091 2005
29
Resolution of AIDS-related Castleman's disease with anti-CD20 monoclonal antibodies is associated with declining IL-6 and TNF-alpha levels. 53 62
15223659 2004
30
Inhibition of IL-6 for the treatment of inflammatory diseases. 53 62
15251133 2004
31
Remission of nephrotic syndrome after thalidomide therapy in a patient with Castleman's disease. 53 62
15182131 2004
32
Systemic juvenile idiopathic arthritis mimics multicentric Castleman's disease. 53 62
14677035 2003
33
Acute myelogenous leukemia M5b developed during clinical remission of Castleman disease. 53 62
12731671 2003
34
Does IL-6 mediate renal manifestations of Castleman's disease? 53 62
12521189 2002
35
Anti-interleukin 6 (IL-6) receptor antibody suppresses Castleman's disease like symptoms emerged in IL-6 transgenic mice. 53 62
12633573 2002
36
Secretion of interleukin-6 and vascular endothelial growth factor by spindle cell sarcoma complicating Castleman's disease (so-called 'vascular neoplasia'). 53 62
12015752 2002
37
High incidence of Kaposi sarcoma-associated herpesvirus-related non-Hodgkin lymphoma in patients with HIV infection and multicentric Castleman disease. 53 62
11895764 2002
38
The Kaposi sarcoma-associated herpesvirus (KSHV) induces cellular interleukin 6 expression: role of the KSHV latency-associated nuclear antigen and the AP1 response element. 53 62
11781250 2002
39
K15 protein of Kaposi's sarcoma-associated herpesvirus is latently expressed and binds to HAX-1, a protein with antiapoptotic function. 53 62
11752170 2002
40
Anti-interleukin 6 receptor antibody treatment in rheumatic disease. 53 62
11053081 2000
41
High levels of human herpesvirus 8 viral load, human interleukin-6, interleukin-10, and C reactive protein correlate with exacerbation of multicentric castleman disease in HIV-infected patients. 53 62
10979949 2000
42
Increased expression of vascular endothelial growth factor (VEGF) in Castleman's disease: proposed pathomechanism of vascular proliferation in the affected lymph node. 53 62
10830746 2000
43
Human herpesvirus 8-encoded interleukin-6 homologue (viral IL-6) induces endogenous human IL-6 secretion. 53 62
10861641 2000
44
Human herpes virus 8 interleukin-6 homologue triggers gp130 on neuronal and hematopoietic cells. 53 62
10848977 2000
45
[Is there a place for interferon-alpha in the treatment strategy of multicentric Castleman's disease?]. 53 62
10874763 2000
46
Characterization of signaling cascades triggered by human interleukin-6 versus Kaposi's sarcoma-associated herpes virus-encoded viral interleukin 6. 53 62
10741750 2000
47
Successful treatment of Castleman's disease with HAART in two HIV-infected patients. 53 62
10762119 2000
48
Improvement in Castleman's disease by humanized anti-interleukin-6 receptor antibody therapy. 53 62
10607684 2000
49
Expression of cell-homologous genes of human herpesvirus-8 in human immunodeficiency virus-negative lymphoproliferative diseases. 53 62
10515899 1999
50
Primary gastric plasmacytoma: a rare cause of hypertrophic gastritis in an adolescent. 53 62
10512402 1999
Sources

Variations for Castleman Disease

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Expression for Castleman Disease

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Search GEO for disease gene expression data for Castleman Disease.
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Pathways for Castleman Disease

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Pathways related to Castleman Disease according to GeneCards Suite gene sharing:

(show top 50) (show all 52)
# Super pathways Score Top Affiliating Genes
1
Innate Immune System 66
Show member pathways
 13.94 CCL4 CCR6 CR1 CRP FCER2 FSCN1
2
ERK Signaling 64
Show member pathways
 13.8 PDGFRB MTOR IL6R IL6 IL10 GSK3B
3
Signal Transduction 66
13.72 AR CCL4 CCR6 CXCL13 FCER2 GSK3B
4
MIF Mediated Glucocorticoid Regulation 64
Show member pathways
 13.48 PDGFRB MTOR IL6ST IL6R IL6 IL10
5
Cytokine Signaling in Immune system 66
Show member pathways
 13.3 IL6ST IL6R IL6 IL10 IFNG FSCN1
6
PAK Pathway 64
Show member pathways
 13.27 PDGFRB IL6R IL6 IL10 CXCL13 CCR6
7
Akt Signaling 64
Show member pathways
 13.21 AR CCL4 CCR6 CXCL13 GSK3B IL10
8
NFAT and Cardiac Hypertrophy 64
Show member pathways
 12.84 PDGFRB MTOR IL6ST IL6 GSK3B
9
CCR5 Pathway in Macrophages 64
Show member pathways
 12.83 GSK3B CXCL13 CCR6 CCL4 AR
10
Malignant pleural mesothelioma 74
12.66 PDGFRB MTOR IL6 IL10 GSK3B CCL4
11
Overview of interferons-mediated signaling pathway 74
Show member pathways
 12.57 PDGFRB MTOR IL6ST IL6R IL6 IL10
12
Breast cancer pathway 74
Show member pathways
 12.44 PDGFRB MTOR IL6ST GSK3B
13
IL-17 Family Signaling Pathways 65
Show member pathways
 12.25 IL6ST IL6R IL6 GSK3B
14
MAPK Signaling: Mitogens 65
Show member pathways
 12.22 PDGFRB MTOR IL6ST IL6R GSK3B
15
Colorectal Cancer Metastasis 64
12.17 IL6R IL6 IFNG GSK3B
16
T-cell activation SARS-CoV-2 74
Show member pathways
 12.13 MTOR IL10 IFNG GSK3B
17
Sudden infant death syndrome (SIDS) susceptibility pathways 74
12.11 MIR210 IL6R IL6 IL10 AR
18
Neuroinflammation and glutamatergic signaling 74
12.04 IFNG IL10 IL6 IL6R IL6ST
19
IL27-mediated signaling events 61
Show member pathways
 11.91 IL6ST IL6 IL10 IFNG
20
Cytokine production by Th17 cells in CF (Mouse model) 22
Show member pathways
 11.86 IL6ST IL6R IL6
21
Allograft rejection 74
11.85 IL10 IFNG CXCL13
22
Th17 Differentiation 65
Show member pathways
 11.85 IL6ST IL6R IL6 IFNG
23
Interferon Pathway 64
11.84 MTOR IL6 IFNG
24
Interleukin-4 and Interleukin-13 signaling 66
11.84 IL6R IL6 IL10 FSCN1 FCER2
25
Transcription Androgen Receptor nuclear signaling 22
Show member pathways
 11.8 MTOR IL6ST IL6R IL6 GSK3B AR
26
Innate Lymphoid Cells Differentiation 65
11.77 CCR6 IFNG IL10
27
Glucocorticoid receptor regulatory network 61
11.75 IL6 IFNG GSK3B
28
Dendritic Cells Developmental Lineage Pathway 65
11.73 IL6 IL10 IFNG
29
Acute viral myocarditis 74
11.73 IL6 IL10 IFNG GSK3B
30
Interleukin-6 family signaling 66
Show member pathways
 11.71 IL6ST IL6R IL6
31
AP-1 transcription factor network 61
11.7 IL6 IL10 IFNG
32
CCL18 signaling pathway 74
11.7 MTOR IL6 IL10 GSK3B CCR6
33
Th17 cell differentiation pathway 74
11.69 MTOR IL6R IL6 IFNG
34
miRNAs involvement in the immune response in sepsis 74
11.64 IL6 IL10 CCL4
35
IL4-mediated signaling events 61
11.63 MTOR IL10 FCER2
36
IL 10 signaling pathway 67
11.63 IL6ST IL6R IL6 IL10
37
Inflammatory bowel disease signaling 74
Show member pathways
 11.62 IFNG IL10 IL6
38
RAF-independent MAPK1/3 activation 66
Show member pathways
 11.52 IL6ST IL6R IL6
39
Extrafollicular B cell activation by SARS-CoV-2 74
11.51 IL6 IL10 IFNG CRP CR1
40
Transcription_Role of VDR in regulation of genes involved in osteoporosis 22
11.48 IL6 IFNG AR
41
Interleukin-10 signaling 66
11.45 IL6 IL10 FCER2 CCL4
42
IL6-mediated signaling events 61
11.41 IL6ST IL6R IL6 CRP
43
IL-15 Signaling and its Primary Biological Effects in Different Immune Cell Types 65
Show member pathways
 11.37 IL10 IFNG CCL4
44
Immune response_IL-6 signaling pathway 22
11.33 IL6 IL6R IL6ST
45
Cytokines and inflammatory response 74
11.27 IL6 IL10 IFNG
46
Immune infiltration in pancreatic cancer 74
11.23 IL6 IL10 IFNG
47
PI3K/AKT/mTOR - vitamin D3 signaling 74
11.12 MTOR IL10 GSK3B
48
Senescence and autophagy in cancer 74
11.12 MTOR IL6ST IL6R IL6 IFNG GSK3B
49
SHP2 signaling 61
11.09 PDGFRB IL6ST IL6R IL6 IFNG
50
ncRNAs involved in STAT3 signaling in hepatocellular carcinoma 74
11.02 IL6ST IL6R IL6
Sources

GO Terms for Castleman Disease

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Cellular components related to Castleman Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 ciliary neurotrophic factor receptor complex GO:0070110 9.26 IL6ST IL6R
2 interleukin-6 receptor complex GO:0005896 9.1 IL6ST IL6R IL6

Biological processes related to Castleman Disease according to GeneCards Suite gene sharing:

(show all 15)
# Name GO ID Score Top Affiliating Genes
1 signal transduction GO:0007165 10.38 PDGFRB IL6ST IL6 IL10 IFNG GSK3B
2 positive regulation of cell population proliferation GO:0008284 10.17 AR CR1 IFNG IL6 IL6R IL6ST
3 immune response GO:0006955 10.15 CCL4 CCR6 CXCL13 IFNG IL10 IL6
4 monocyte chemotaxis GO:0002548 10.01 IL6R IL6 CCL4
5 positive regulation of chemokine production GO:0032722 9.99 IL6R IL6 IFNG
6 chemotaxis GO:0006935 9.95 PDGFRB CXCL13 CCR6 CCL4
7 acute-phase response GO:0006953 9.91 CRP IL6 IL6R
8 positive regulation of killing of cells of another organism GO:0051712 9.83 IFNG FCER2
9 positive regulation of osteoblast differentiation GO:0045669 9.8 MIR210 IL6ST IL6R IL6
10 ciliary neurotrophic factor-mediated signaling pathway GO:0070120 9.76 IL6ST IL6R
11 interleukin-11-mediated signaling pathway GO:0038154 9.71 IL6ST IL6R
12 hepatic immune response GO:0002384 9.67 IL6R IL6
13 positive regulation of tyrosine phosphorylation of STAT protein GO:0042531 9.56 IL6ST IL6R IL6 IFNG
14 positive regulation of iron ion import across plasma membrane GO:1904440 9.33 MIR210 IFNG
15 interleukin-6-mediated signaling pathway GO:0070102 9.1 IL6ST IL6R IL6

Molecular functions related to Castleman Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cytokine activity GO:0005125 10.02 IL6 IL10 IFNG CXCL13 CCL4
2 ciliary neurotrophic factor receptor activity GO:0004897 9.71 IL6ST IL6R
3 interleukin-11 binding GO:0019970 9.62 IL6ST IL6R
4 interleukin-11 receptor activity GO:0004921 9.56 IL6ST IL6R
5 interleukin-6 binding GO:0019981 9.46 IL6ST IL6R
6 interleukin-6 receptor activity GO:0004915 9.26 IL6ST IL6R
7 interleukin-6 receptor binding GO:0005138 9.1 IL6ST IL6R IL6
Sources

Sources for Castleman Disease

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2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
34 ICD9CM
35 IUPHAR
36 LifeMap
38 LOVD
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
52 NINDS
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 08-Dec-2022)
61 PubChem
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 UMLS via Orphanet
The MalaCards human disease database index: 1-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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