MCID: CST005
MIFTS: 56

Castleman Disease

Categories: Immune diseases, Rare diseases

Aliases & Classifications for Castleman Disease

MalaCards integrated aliases for Castleman Disease:

Name: Castleman Disease 12 20 36 54 44 15
Angiofollicular Ganglionic Hyperplasia 20 44 70
Giant Lymph Node Hyperplasia 12 20 17
Angiofollicular Lymph Hyperplasia 12 20
Castleman's Disease 73 15
Angiofollicular Lymph Node Hyperplasia 12
Angiolymphoid Hyperplasia 70
Benign Giant Lymphoma 20
Lymphoid Hamartoma 12

Classifications:



Summaries for Castleman Disease

KEGG : 36 Castleman disease is a rare lymphoproliferative disorder with two primary subtypes that vary in presentation and course. Unicentric Castleman disease is localized and carries an excellent prognosis. Multicentric Castleman disease can be associated with HIV and human herpesvirus-8 and is characterized by generalized lymphadenopathy and systemic symptoms, such as fever, fatigue, anorexia, anemia, and cachexia. Castleman disease is unique in that dysregulated secretion of interleukin-6 (IL-6) plays a central pathogenetic role, although the exact events precipitating the oversecretion of IL-6 are unknown. Recently, two antibody-based therapeutics targeting components of the IL-6/IL-6R complex have been approved for the treatment of Castleman disease. Tocilizumab is a monoclonal antibody directed against the IL-6 receptor, and siltuximab is a monoclonal antibody specific for IL-6.

MalaCards based summary : Castleman Disease, also known as angiofollicular ganglionic hyperplasia, is related to human herpesvirus 8 and multicentric castleman disease. An important gene associated with Castleman Disease is IL6 (Interleukin 6), and among its related pathways/superpathways are Cytokine-cytokine receptor interaction and JAK-STAT signaling pathway. The drugs Siltuximab and Zanubrutinib have been mentioned in the context of this disorder. Affiliated tissues include lymph node, bone and bone marrow, and related phenotypes are fatigue and anemia

Disease Ontology : 12 A lymphoproliferative syndrome characterized by one or more enlarged lymph nodes containing cells with hyaline-vascular, plasmacytic, or mixed appearance microscopically.

GARD : 20 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. Treatment varies based on the form of the condition, the severity of symptoms and whether or not the affected person also has an HIV and/or human herpes virus type 8 (HHV-8) infection. For more specific information about each form of CD, please visit GARD's unicentric Castleman disease and multicentric Castleman disease pages.

Wikipedia : 73 Castleman disease is a group of uncommon lymphoproliferative disorders characterized by lymph node... more...

Related Diseases for Castleman Disease

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

(show top 50) (show all 807)
# Related Disease Score Top Affiliating Genes
1 human herpesvirus 8 32.5 IL6ST IL6R IL6
2 multicentric castleman disease 32.2 SDC1 MTOR KRT15 IL6 IL10 IFNG
3 polyclonal hypergammaglobulinemia 31.2 IL6 CXCL13 CRP
4 bronchiolitis obliterans 31.0 IL6 IL10 IFNG CRP
5 hemolytic anemia 30.9 IL10 IFNG CXCL13 CR1 CCL4
6 bronchiolitis 30.9 IL6 IL10 IFNG CRP CCL4
7 ulcerative colitis 30.8 IL6 IL10 IFNG CRP
8 infective endocarditis 30.7 IL6 CRP CCL4
9 exanthem 30.7 MTOR IL6 IL10 IFNG H2AC18 CRP
10 temporal arteritis 30.7 IL6 IFNG CRP
11 proliferative glomerulonephritis 30.7 IL6 IFNG CR1
12 adult-onset still's disease 30.7 IL6 IFNG CRP
13 peritonitis 30.7 IL6 IL10 CRP CR1
14 plasmacytoma 30.7 IL6ST IL6R IL6
15 pneumonia 30.7 IL6 IL10 IFNG CRP
16 stomatitis 30.6 MTOR IL6 IL10 IFNG
17 intestinal obstruction 30.6 MTOR IL6 CRP
18 follicular dendritic cell sarcoma 30.6 FCER2 DSG3 CXCL13 CR2 CR1
19 lichen planus 30.6 IL6 IL10 IFNG DSG3
20 rare tumor 30.6 FSCN1 FCER2 CR2
21 polyradiculoneuropathy 30.5 IL10 IFNG CCL4
22 pancytopenia 30.5 IL6 IFNG H2AC18 CRP
23 aphthous stomatitis 30.5 IL6 IL10 IFNG
24 brucellosis 30.5 IL6 IL10 IFNG CRP
25 crohn's disease 30.5 IL6 IL10 IFNG CRP
26 lymphoid interstitial pneumonia 30.5 IL6 H2AC18 CRP
27 thyroiditis 30.5 IL6 IL10 IFNG CRP
28 lymphadenitis 30.5 IL10 IFNG CRP
29 liver cirrhosis 30.5 IL6 IL10 IFNG CRP CR1
30 interstitial lung disease 30.5 IL6 IL10 IFNG CRP
31 uveitis 30.5 IL6 IL10 IFNG CCL4
32 human immunodeficiency virus type 1 30.4 IL10 IFNG CR2 CR1 CCL4
33 retinal detachment 30.4 IL6 IL10 IFNG CCL4
34 follicular lymphoma 30.4 MIR210 FCER2 CR2 CR1
35 rheumatoid arthritis 30.4 IL6ST IL6R IL6 IL10 IFNG CRP
36 celiac disease 1 30.4 IL6 IL10 IFNG H2AC18 CRP
37 syphilis 30.4 IL6 IFNG CRP
38 systemic autoimmune disease 30.4 IL6 CRP
39 lung abscess 30.4 IL6 IFNG CRP
40 bronchopneumonia 30.4 IL6 IL10 CRP
41 brain edema 30.4 IL6 IFNG CRP
42 allergic disease 30.4 IL6 IL10 IFNG FCER2
43 b-cell growth factor 30.4 IL6 IFNG
44 conjunctivitis 30.4 IL6 IL10 IFNG
45 bacterial sepsis 30.3 IL6 IL10 CRP
46 acute pyelonephritis 30.3 IL6 IL10 CRP
47 radiculopathy 30.3 IL6 IL10 CCL4
48 mixed connective tissue disease 30.3 IL6 IL10 IFNG
49 pericarditis 30.3 IL6 IFNG CRP
50 chronic inflammatory demyelinating polyradiculoneuropathy 30.3 IL10 IFNG CCL4

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



Diseases related to Castleman Disease

Symptoms & Phenotypes for Castleman Disease

Human phenotypes related to Castleman Disease:

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

MGI Mouse Phenotypes related to Castleman Disease:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 cellular MP:0005384 10.1 CXCL13 DSG3 FCER2 FSCN1 IFNG IL10
2 hematopoietic system MP:0005397 9.97 CR2 CXCL13 DSG3 FCER2 FSCN1 IFNG
3 digestive/alimentary MP:0005381 9.86 DSG3 FCER2 IFNG IL10 IL6 IL6ST
4 immune system MP:0005387 9.8 CR2 CRP CXCL13 DSG3 FCER2 FSCN1
5 integument MP:0010771 9.28 DSG3 FSCN1 IFNG IL10 IL6 IL6ST

Drugs & Therapeutics for Castleman Disease

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

(show top 50) (show all 57)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Siltuximab Approved, Investigational Phase 2 541502-14-1
2
Zanubrutinib Approved, Investigational Phase 2 1691249-45-2
3
Dexamethasone Approved, Investigational, Vet_approved Phase 2 50-02-2 5743
4
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 2 1177-87-3
5
Bortezomib Approved, Investigational Phase 2 179324-69-7 93860 387447
6
Clotrimazole Approved, Vet_approved Phase 2 23593-75-1 2812
7
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
8
Sirolimus Approved, Investigational Phase 2 53123-88-9 6436030 5284616
9
Zidovudine Approved Phase 2 30516-87-1 35370
10
Ganciclovir Approved, Investigational Phase 2 82410-32-0 3454
11
Lenograstim Approved, Investigational Phase 2 135968-09-1
12
Thalidomide Approved, Investigational, Withdrawn Phase 2 50-35-1 5426
13
Suramin Investigational Phase 2 145-63-1 5361
14 Analgesics Phase 2
15 Histamine H1 Antagonists Phase 2
16 Antipyretics Phase 2
17 Histamine Antagonists Phase 2
18 Pharmaceutical Solutions Phase 2
19 Antiparasitic Agents Phase 2
20 Antiprotozoal Agents Phase 2
21 Anthelmintics Phase 2
22 Protein Kinase Inhibitors Phase 2
23 Antifungal Agents Phase 2
24 Antimetabolites Phase 2
25 Reverse Transcriptase Inhibitors Phase 2
26 interferons Phase 2
27 Interferon-alpha Phase 2
28 Ganciclovir triphosphate Phase 2
29 Antibodies Phase 2
30 Immunoglobulins Phase 2
31 Antibodies, Monoclonal Phase 2
32
Methotrexate Approved Phase 1 1959-05-2, 59-05-2 126941
33
Levoleucovorin Approved, Investigational Phase 1 68538-85-2 149436
34
Daunorubicin Approved Phase 1 20830-81-3 30323
35
Cetuximab Approved Phase 1 205923-56-4 56842117 2333
36
Bevacizumab Approved, Investigational Phase 1 216974-75-3
37
Valproic acid Approved, Investigational Phase 1 99-66-1 3121
38
Folic acid Approved, Nutraceutical, Vet_approved Phase 1 59-30-3 6037
39 Vitamin B9 Phase 1
40 Folic Acid Antagonists Phase 1
41 Immunoglobulins, Intravenous Phase 1
42 Keratolytic Agents Phase 1
43 Dermatologic Agents Phase 1
44 Vitamin B Complex Phase 1
45 Folate Phase 1
46 Podophyllotoxin Phase 1 518-28-5
47 Neurotransmitter Agents Phase 1
48 Psychotropic Drugs Phase 1
49 Immunoglobulin G Phase 1
50 Mitogens Phase 1

Interventional clinical trials:

(show all 28)
# 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 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)
3 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
4 Pilot Study of Tocilizumab in Patients With Symptomatic Kaposi Sarcoma Herpesvirus (KSHV) - Associated Multicentric Castleman Disease Completed NCT01441063 Phase 2 Zidovudine;Tocilizumab;Valganciclovir (VGC)
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 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
7 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
8 A Phase II, Single-arm Open-label Multi-center Study of Sirolimus in Previously Treated Idiopathic Multicentric Castleman Disease Recruiting NCT03933904 Phase 2 Sirolimus
9 Targeted Oncolytic Virotherapy and Natural History Study of KSHV-Associated Multicentric Castleman's Disease With Laboratory and Clinical Correlates of Disease Activity Recruiting NCT00092222 Phase 2 Etoposide;Interferon-alpha;Rituximab;Zidovudine;Liposomal Doxorubicin;Bortezomib;Valganciclovir;Doxorubicin;Vincristine;Cyclophosphamide;Filgrastim (G-CSF);Prednisone;Sirolimus
10 Thalidomide, Cyclophosphamide and Prednisone in Newly Diagnosed Multicentric Castleman's Disease: a Prospective, Single-center, Single-arm, Phase-II Pilot Trial Active, not recruiting NCT03043105 Phase 2 Thalidomide, cyclophosphamide and prednisone
11 LCCC 1950 - Rituximab for Multicentric Castleman Disease in Malawi, A Single-Arm Phase II Safety/Efficacy Trial Not yet recruiting NCT04585893 Phase 2 Rituximab;Etoposide
12 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
13 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
14 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
15 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]
16 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
17 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
18 A Phase I Trial of Bevacizumab, Temsirolimus Alone and in Combination With Valproic Acid, or Cetuximab in Patients With Advanced Malignancy and Other Indications Recruiting NCT01552434 Phase 1 Temsirolimus;Valproic Acid
19 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
20 Unlock the Cell: Intracellular Inflammatory Pathways and Flow Cytometry Study for Castleman's Disease Completed NCT02853968
21 Epithelioid Hemangioma of Bone And Soft Tissue: a Benign Tumor With Metastatic Potential Completed NCT03169595
22 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
23 Molecular Characterization of Viral-associated Tumors, Tumors Occurring in the Setting of HIV or Other Immune Disorders and Castleman Disease Recruiting NCT03300830
24 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
25 Registry for Adults With Plasma Cell Disorders (PCD's) Recruiting NCT03717844
26 Assessment of Bone Mineral Density in People Living With HIV and KSHV Associated Malignancies: A Retrospective Longitudinal Analysis Active, not recruiting NCT04742387
27 A Pilot Trial of Nelfinavir for the Lytic Activation and Treatment of Gammaherpesvirus-Related Tumors Terminated NCT02080416 Early Phase 1 Nelfinavir
28 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 70 / NDF-RT 51 :


siltuximab

Cochrane evidence based reviews: castleman disease

Genetic Tests for Castleman Disease

Anatomical Context for Castleman Disease

MalaCards organs/tissues related to Castleman Disease:

40
Lymph Node, Bone, Bone Marrow, Endothelial, Skin, Kidney, Pancreas

Publications for Castleman Disease

Articles related to Castleman Disease:

(show top 50) (show all 1413)
# Title Authors PMID Year
1
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. 61 54
19730402 2009
2
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. 61 54
18784373 2008
3
Reversible cardiomyopathy associated with Multicentric Castleman disease: successful treatment with tocilizumab, an anti-interleukin 6 receptor antibody. 54 61
17483056 2007
4
Bone marrow findings in multicentric Castleman disease in HIV-negative patients. 54 61
17325481 2007
5
Interleukin 6: from bench to bedside. 61 54
17075601 2006
6
[A case of HIV-1 and HHV-8-associated Castleman disease with a relapsing high fever and lymphoadenopathy]. 54 61
16922487 2006
7
Autoantibody production from a thymoma and a follicular dendritic cell sarcoma associated with paraneoplastic pemphigus. 54 61
16120143 2005
8
Intraventricular chordoid meningioma presenting with Castleman disease due to overproduction of interleukin-6. Case report. 61 54
15871519 2005
9
Acute myelogenous leukemia M5b developed during clinical remission of Castleman disease. 54 61
12731671 2003
10
High incidence of Kaposi sarcoma-associated herpesvirus-related non-Hodgkin lymphoma in patients with HIV infection and multicentric Castleman disease. 54 61
11895764 2002
11
The Kaposi sarcoma-associated herpesvirus (KSHV) induces cellular interleukin 6 expression: role of the KSHV latency-associated nuclear antigen and the AP1 response element. 61 54
11781250 2002
12
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. 54 61
10979949 2000
13
[Advances in interleukin-6 therapy]. 54 61
10340005 1999
14
Castleman disease in POEMS syndrome with elevated interleukin-6. 54 61
8499003 1993
15
Membranous nephropathy with thrombotic microangiopathy-like lesions successfully treated with tocilizumab in a patient with idiopathic multicentric Castleman disease. 61
33389670 2021
16
Autoimmune hypoglycemia expands the biological spectrum of HHV8+ multicentric Castleman disease. 61
33787862 2021
17
Response assessment and salvage indication for idiopathic multicentric Castleman disease. 61
33215565 2021
18
Castleman disease and paraneoplastic pemphigus in a pregnant woman: A case report. 61
33787583 2021
19
A Review of Genetic Abnormalities in Unicentric and Multicentric Castleman Disease. 61
33804823 2021
20
Dichotomic response to interleukin-6 blockade in idiopathic multicentric Castleman disease: two case reports. 61
33676575 2021
21
The clinical, laboratory, and radiologic improvement due to siltuximab treatment in idiopathic multicentric Castleman's disease. 61
32088937 2021
22
18F-FDG PET/CT imaging features of patients with multicentric Castleman disease. 61
33741858 2021
23
A 10-year observational single-center study of retroperitoneal unicentric Castleman disease. 61
33725899 2021
24
Castleman disease: A single-center case series. 61
33631648 2021
25
Dysphagia and Dysphonia, a Pairing of Symptoms Caused by an Unusual Pair of Diseases: Castleman's Disease and Myasthenia Gravis. 61
32812444 2021
26
Emerging role of 18F-FDG PET/CT in Castleman disease: a review. 61
33709329 2021
27
Coexistence of disseminated Kaposi sarcoma and multicentric Castleman disease in an HIV-infected patient under viral suppression. 61
33555231 2021
28
Characteristics and outcomes of KSHV-associated multicentric Castleman disease with or without other KSHV diseases. 61
33720337 2021
29
Evidence for altered host genetic factors in KSHV infection and KSHV-related disease development. 61
33043529 2021
30
HIV Lymphadenopathy: Differential Diagnosis and Important Imaging Features. 61
33325733 2021
31
Systemic lupus erythematosus complicated with Castleman disease: a case-based review. 61
32797278 2021
32
Clinical characteristics and immunological abnormalities of Castleman disease complicated with autoimmune diseases. 61
33544201 2021
33
Two cases of idiopathic multicentric Castleman disease with nephrotic syndrome treated with tocilizumab. 61
32715375 2021
34
Angiomatoid fibrous histiocytoma with Castleman disease: A case report. 61
32672859 2021
35
Unicentric Castleman Disease; the laparoscopic en bloc resection of a hypervascular Giant Lymph Node in the aortacaval zone. 61
33631872 2021
36
Pathologically confirmed oesophageal involvement in idiopathic multicentric Castleman disease mimicking early oesophageal cancer. 61
32885232 2021
37
Paraneoplastic pemphigus and fatal bronchiolitis obliterans associated with Castleman disease: Report of an autopsy case. 61
33382501 2021
38
Author Correction: The value of multiparameter 18F-FDG PET/CT imaging in differentiating retroperitoneal paragangliomas from unicentric Castleman disease. 61
33623136 2021
39
Comment on: HHV-8-negative multicentric Castleman disease patients with serological, histopathological and imaging features of IgG4-related disease. 61
33249497 2021
40
Comment on: HHV-8-negative multicentric Castleman disease patients with serological, histopathological and imaging features of IgG4-related disease: reply. 61
33253362 2021
41
Skin-associated lymphoid tissue could be a sign of systemic disease: reply from authors. 61
33615442 2021
42
HHV-8-negative multicentric Castleman disease patients with serological, histopathological and imaging features of IgG4-related disease. 61
32772106 2021
43
UCD with MCD-like inflammatory state: surgical excision is highly effective. 61
33570636 2021
44
Newly diagnosed and previously treated multicentric Castleman disease respond equally to siltuximab. 61
33128769 2021
45
Predictive Model for Idiopathic Multicentric Castleman Disease Supporting Treatment Decisions. 61
33215784 2021
46
Sequential change in serum VEGF levels in a case of tocilizumab-resistant TAFRO syndrome treated effectively with rituximab. 61
33269653 2021
47
Castleman Disease: An Unusual Cause of an Isolated Neck Mass. 61
32796313 2021
48
Autoimmunity Due to Unicentric Castleman Disease Cured by Resection of a Hepatic Mass. 61
31609813 2021
49
Immune-mediated ataxia with cerebellar hemiatrophy and dystonia associated with Castleman disease. 61
33338826 2021
50
Idiopathic multicentric Castleman disease with novel heterozygous Ile729Met mutation in exon 10 of familial Mediterranean fever gene. 61
32830263 2021

Variations for Castleman Disease

Expression for Castleman Disease

Search GEO for disease gene expression data for Castleman Disease.

Pathways for Castleman Disease

Pathways related to Castleman Disease according to KEGG:

36
# Name Kegg Source Accession
1 Cytokine-cytokine receptor interaction hsa04060
2 JAK-STAT signaling pathway hsa04630
3 Hematopoietic cell lineage hsa04640

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

(show all 31)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.98 PDGFRB MTOR IL6ST IL6R IL6 IL10
2
Show member pathways
13.59 PDGFRB MTOR IL6ST IL6R IL6 IL10
3
Show member pathways
13.22 PDGFRB IL6ST IL6R IL6 IL10 IFNG
4 12.79 PDGFRB MTOR IL6ST IL6R IL6 IFNG
5
Show member pathways
12.49 MTOR IL6ST IL6R IL6 IL10 IFNG
6
Show member pathways
12.37 MTOR IL10 FCER2 CR2 CCL4
7
Show member pathways
12.28 IL6ST IL6R IL6 IFNG
8
Show member pathways
12.26 MTOR IL6ST IL6R IL6
9 12.18 IL6 IL10 IFNG CR1
10 12.13 MIR210 IL6R IL6 IL10
11
Show member pathways
12.07 IL6ST IL6R IL6 IL10 CXCL13 CCL4
12 11.93 SDC1 IL6 IL10 IFNG
13
Show member pathways
11.9 PDGFRB MTOR IL6ST IL6R IL6 IL10
14 11.87 MTOR IL6R IL6 IFNG
15 11.86 IL6R IL6 IL10 FSCN1 FCER2
16 11.79 MTOR IL6ST IL6R IL6 IFNG
17
Show member pathways
11.78 IL6ST IL6R IL6 IFNG
18
Show member pathways
11.75 IL6ST IL6R IL6 CRP
19 11.72 IL6R IL6 FCER2 CR2 CR1
20 11.7 IL6 IL10 IFNG
21
Show member pathways
11.68 IL6ST IL6 IL10 IFNG
22 11.61 MTOR IL10 FCER2
23
Show member pathways
11.57 IL6ST IL6R IL6
24
Show member pathways
11.42 IL6ST IL6R IL6
25 11.42 IL6 IL10 FCER2 CCL4
26 11.37 IL6 IL10 CXCL13 CR2 CR1
27 11.35 IL6 IL10 IFNG
28
Show member pathways
11.33 IL10 IFNG CCL4
29 11.29 IL6ST IL6R IL6
30 11.2 IL6 IL10 IFNG
31 10.72 SDC1 IL6 IL10 IFNG CR1

GO Terms for Castleman Disease

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

# Name GO ID Score Top Affiliating Genes
1 extracellular exosome GO:0070062 9.97 SDC1 KRT15 IL6ST H2AC18 FSCN1 FCER2
2 extracellular space GO:0005615 9.91 MIR210 IL6ST IL6R IL6 IL10 IFNG
3 extracellular region GO:0005576 9.85 SDC1 IL6ST IL6R IL6 IL10 IFNG
4 receptor complex GO:0043235 9.67 PDGFRB IL6ST IL6R CR2
5 ciliary neurotrophic factor receptor complex GO:0070110 8.96 IL6ST IL6R
6 interleukin-6 receptor complex GO:0005896 8.8 IL6ST IL6R IL6

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

(show all 25)
# Name GO ID Score Top Affiliating Genes
1 inflammatory response GO:0006954 9.97 SDC1 IL6 CXCL13 CRP CCL4
2 positive regulation of cell proliferation GO:0008284 9.95 PDGFRB IL6ST IL6R IL6 IFNG CR1
3 immune response GO:0006955 9.88 IL6 IL10 IFNG CXCL13 CR2 CCL4
4 regulation of actin cytoskeleton organization GO:0032956 9.79 PDGFRB MTOR FSCN1
5 response to glucocorticoid GO:0051384 9.77 SDC1 IL6 IL10
6 response to activity GO:0014823 9.72 MTOR IL6 IL10
7 monocyte chemotaxis GO:0002548 9.7 IL6R IL6 CCL4
8 positive regulation of chemokine production GO:0032722 9.69 IL6R IL6 IFNG
9 acute-phase response GO:0006953 9.67 IL6R IL6 CRP
10 positive regulation of MHC class II biosynthetic process GO:0045348 9.62 IL10 IFNG
11 neutrophil mediated immunity GO:0002446 9.62 IL6R IL6
12 negative regulation of lipid storage GO:0010888 9.61 IL6 CRP
13 positive regulation of extracellular matrix disassembly GO:0090091 9.59 IL6 FSCN1
14 T-helper 17 cell lineage commitment GO:0072540 9.58 IL6R IL6
15 vascular endothelial growth factor production GO:0010573 9.58 IL6R IL6
16 positive regulation of killing of cells of other organism GO:0051712 9.56 IFNG FCER2
17 positive regulation of tyrosine phosphorylation of STAT protein GO:0042531 9.56 IL6ST IL6R IL6 IFNG
18 ciliary neurotrophic factor-mediated signaling pathway GO:0070120 9.54 IL6ST IL6R
19 interleukin-6-mediated signaling pathway GO:0070102 9.5 IL6ST IL6R IL6
20 positive regulation of ferrous iron import across plasma membrane GO:1904440 9.46 MIR210 IFNG
21 positive regulation of osteoblast differentiation GO:0045669 9.46 MIR210 IL6ST IL6R IL6
22 interleukin-11-mediated signaling pathway GO:0038154 9.43 IL6ST IL6R
23 hepatic immune response GO:0002384 9.37 IL6R IL6
24 positive regulation of smooth muscle cell proliferation GO:0048661 9.26 PDGFRB MTOR IL6R IL6
25 cytokine-mediated signaling pathway GO:0019221 9.23 SDC1 IL6ST IL6R IL6 IL10 FSCN1

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

# Name GO ID Score Top Affiliating Genes
1 ciliary neurotrophic factor receptor activity GO:0004897 9.4 IL6ST IL6R
2 interleukin-11 binding GO:0019970 9.37 IL6ST IL6R
3 cytokine activity GO:0005125 9.35 IL6 IL10 IFNG CXCL13 CCL4
4 interleukin-11 receptor activity GO:0004921 9.32 IL6ST IL6R
5 interleukin-6 binding GO:0019981 9.26 IL6ST IL6R
6 interleukin-6 receptor activity GO:0004915 9.16 IL6ST IL6R
7 interleukin-6 receptor binding GO:0005138 8.8 IL6ST IL6R IL6

Sources for Castleman Disease

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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