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 71
Giant Lymph Node Hyperplasia 12 20 17
Angiofollicular Lymph Hyperplasia 12 20
Castleman's Disease 74 15
Angiofollicular Lymph Node Hyperplasia 12
Angiolymphoid Hyperplasia 71
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 kaposi sarcoma 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 : 74 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 797)
# Related Disease Score Top Affiliating Genes
1 kaposi sarcoma 32.6 MTOR IL6ST IL6R IL6
2 multicentric castleman disease 32.5 MTOR KRT15 IL6 IL10 IFNG H2AC18
3 human herpesvirus 8 32.5 IL6ST IL6R IL6
4 polyclonal hypergammaglobulinemia 31.2 IL6 CXCL13 CRP
5 bronchiolitis obliterans 31.0 IL6 IL10 IFNG CRP
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 pneumonia 30.6 IL6 IL10 IFNG CRP
15 stomatitis 30.6 MTOR IL6 IL10 IFNG
16 intestinal obstruction 30.6 MTOR IL6 CRP
17 plasmacytoma 30.6 IL6ST IL6R IL6
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 crohn's disease 30.5 IL6 IL10 IFNG CRP
25 brucellosis 30.5 IL6 IL10 IFNG CRP
26 thyroiditis 30.5 IL6 IL10 IFNG CRP
27 lymphoid interstitial pneumonia 30.5 IL6 H2AC18 CRP
28 lymphadenitis 30.5 IL10 IFNG CRP
29 lymphoma, non-hodgkin, familial 30.5 IL6R IL6 H2AC18 FCER2 CR2
30 human immunodeficiency virus type 1 30.4 IL10 IFNG CR2 CR1 CCL4
31 retinal detachment 30.4 IL6 IL10 IFNG CCL4
32 follicular lymphoma 30.4 MIR210 FCER2 CR2 CR1
33 celiac disease 1 30.4 IL6 IL10 IFNG H2AC18 CRP
34 vascular disease 30.4 IL6 IL10 IFNG CRP ACVRL1
35 systemic autoimmune disease 30.4 IL6 CRP
36 lung abscess 30.4 IL6 IFNG CRP
37 bronchopneumonia 30.4 IL6 IL10 CRP
38 enterocolitis 30.4 IL6 IL10 CRP
39 b-cell growth factor 30.4 IL6 IFNG
40 conjunctivitis 30.4 IL6 IL10 IFNG
41 autoimmune disease 30.4 IL6 IL10 IFNG DSG3 CRP CR2
42 brain edema 30.4 IL6 IFNG CRP
43 bacterial sepsis 30.3 IL6 IL10 CRP
44 acute pyelonephritis 30.3 IL6 IL10 CRP
45 radiculopathy 30.3 IL6 IL10 CCL4
46 pericarditis 30.3 IL6 IFNG CRP
47 urticaria 30.3 IL6 FCER2 CRP
48 chronic inflammatory demyelinating polyradiculoneuropathy 30.3 IL10 IFNG CCL4
49 immune deficiency disease 30.3 IL6 IL10 IFNG H2AC18 CR2 CCL4
50 appendicitis 30.3 IL6 IL10 IFNG CRP

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

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

26 (show all 16)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased shRNA abundance (Z-score < -2) GR00366-A-128 9.55 IL10
2 Decreased shRNA abundance (Z-score < -2) GR00366-A-130 9.55 ACVRL1
3 Decreased shRNA abundance (Z-score < -2) GR00366-A-132 9.55 IL10
4 Decreased shRNA abundance (Z-score < -2) GR00366-A-14 9.55 IL6R
5 Decreased shRNA abundance (Z-score < -2) GR00366-A-168 9.55 ACVRL1
6 Decreased shRNA abundance (Z-score < -2) GR00366-A-173 9.55 IL10
7 Decreased shRNA abundance (Z-score < -2) GR00366-A-179 9.55 IL10
8 Decreased shRNA abundance (Z-score < -2) GR00366-A-180 9.55 CRP
9 Decreased shRNA abundance (Z-score < -2) GR00366-A-19 9.55 IL6R
10 Decreased shRNA abundance (Z-score < -2) GR00366-A-25 9.55 IL10
11 Decreased shRNA abundance (Z-score < -2) GR00366-A-34 9.55 IL10
12 Decreased shRNA abundance (Z-score < -2) GR00366-A-49 9.55 CRP
13 Decreased shRNA abundance (Z-score < -2) GR00366-A-65 9.55 IL6R
14 Decreased shRNA abundance (Z-score < -2) GR00366-A-68 9.55 CRP IL6R
15 Decreased shRNA abundance (Z-score < -2) GR00366-A-80 9.55 IL10
16 Decreased shRNA abundance (Z-score < -2) GR00366-A-89 9.55 CRP

MGI Mouse Phenotypes related to Castleman Disease:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 digestive/alimentary MP:0005381 9.81 ACVRL1 DSG3 FCER2 IFNG IL10 IL6
2 hematopoietic system MP:0005397 9.77 ACVRL1 CR2 CXCL13 DSG3 FCER2 FSCN1
3 immune system MP:0005387 9.44 CR2 CRP CXCL13 DSG3 FCER2 FSCN1

Drugs & Therapeutics for Castleman Disease

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

(show top 50) (show all 56)
# 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 acetate Approved, Investigational, Vet_approved Phase 2 1177-87-3
4
Dexamethasone Approved, Investigational, Vet_approved Phase 2 50-02-2 5743
5
Bortezomib Approved, Investigational Phase 2 179324-69-7 387447 93860
6
Zidovudine Approved Phase 2 30516-87-1 35370
7
Clotrimazole Approved, Vet_approved Phase 2 23593-75-1 2812
8
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
9
Sirolimus Approved, Investigational Phase 2 53123-88-9 5284616 6436030
10
Lenograstim Approved, Investigational Phase 2 135968-09-1
11
Ganciclovir Approved, Investigational Phase 2 82410-32-0 3454
12
Suramin Investigational Phase 2 145-63-1 5361
13 Histamine Antagonists Phase 2
14 Analgesics Phase 2
15 Histamine H1 Antagonists Phase 2
16 Antipyretics Phase 2
17 Pharmaceutical Solutions Phase 2
18 Antiparasitic Agents Phase 2
19 Anthelmintics Phase 2
20 Antiprotozoal Agents Phase 2
21 Protein Kinase Inhibitors Phase 2
22 Reverse Transcriptase Inhibitors Phase 2
23 Antimetabolites Phase 2
24 Antifungal Agents Phase 2
25 Interferon-alpha Phase 2
26 Ganciclovir triphosphate Phase 2
27 interferons Phase 2
28 Immunoglobulins Phase 2
29 Antibodies Phase 2
30 Antibodies, Monoclonal Phase 2
31
Methotrexate Approved Phase 1 1959-05-2, 59-05-2 126941
32
Levoleucovorin Approved, Investigational Phase 1 68538-85-2 149436
33
Daunorubicin Approved Phase 1 20830-81-3 30323
34
Cetuximab Approved Phase 1 205923-56-4 56842117 2333
35
Bevacizumab Approved, Investigational Phase 1 216974-75-3
36
Valproic acid Approved, Investigational Phase 1 99-66-1 3121
37
Folic acid Approved, Nutraceutical, Vet_approved Phase 1 59-30-3 6037
38 Keratolytic Agents Phase 1
39 Folic Acid Antagonists Phase 1
40 Vitamin B Complex Phase 1
41 Folate Phase 1
42 Dermatologic Agents Phase 1
43 Vitamin B9 Phase 1
44 Podophyllotoxin Phase 1 518-28-5
45 Anticonvulsants Phase 1
46 Immunoglobulin G Phase 1
47 Neurotransmitter Agents Phase 1
48 Psychotropic Drugs Phase 1
49 Mitogens Phase 1
50 Endothelial Growth Factors 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 71 / 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 1400)
# 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. 54 61
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. 61 54
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]. 61 54
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. 54 61
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
Dysphagia and Dysphonia, a Pairing of Symptoms Caused by an Unusual Pair of Diseases: Castleman's Disease and Myasthenia Gravis. 61
32812444 2021
16
Angiomatoid fibrous histiocytoma with Castleman disease: A case report. 61
32672859 2021
17
Two cases of idiopathic multicentric Castleman disease with nephrotic syndrome treated with tocilizumab. 61
32715375 2021
18
Coexistence of disseminated Kaposi sarcoma and multicentric Castleman disease in an HIV-infected patient under viral suppression. 61
33555231 2021
19
Systemic lupus erythematosus complicated with Castleman disease: a case-based review. 61
32797278 2021
20
Clinical characteristics and immunological abnormalities of Castleman disease complicated with autoimmune diseases. 61
33544201 2021
21
HIV Lymphadenopathy: Differential Diagnosis and Important Imaging Features. 61
33325733 2021
22
Comment on: HHV-8-negative multicentric Castleman disease patients with serological, histopathological and imaging features of IgG4-related disease. 61
33249497 2021
23
Comment on: HHV-8-negative multicentric Castleman disease patients with serological, histopathological and imaging features of IgG4-related disease: reply. 61
33253362 2021
24
Paraneoplastic pemphigus and fatal bronchiolitis obliterans associated with Castleman disease: Report of an autopsy case. 61
33382501 2021
25
Pathologically confirmed oesophageal involvement in idiopathic multicentric Castleman disease mimicking early oesophageal cancer. 61
32885232 2021
26
Predictive Model for Idiopathic Multicentric Castleman Disease Supporting Treatment Decisions. 61
33215784 2021
27
Newly diagnosed and previously treated multicentric Castleman disease respond equally to siltuximab. 61
33128769 2021
28
Sequential change in serum VEGF levels in a case of tocilizumab-resistant TAFRO syndrome treated effectively with rituximab. 61
33269653 2021
29
Castleman Disease: An Unusual Cause of an Isolated Neck Mass. 61
32796313 2021
30
HHV-8-negative multicentric Castleman disease patients with serological, histopathological and imaging features of IgG4-related disease. 61
32772106 2021
31
UCD with MCD-like inflammatory state: surgical excision is highly effective. 61
33570636 2021
32
Elevated IL-13 in effusions of patients with HIV and primary effusion lymphoma as compared with other Kaposi sarcoma herpesvirus-associated disorders. 61
33273183 2021
33
Updates on the diagnosis and management of multicentric Castleman disease. 61
33505874 2021
34
Retroperitoneal castleman disease mimicking lymph node spread from clear renal cell carcinoma. A case report. 61
33304821 2021
35
Adrenal Castleman Disease on 99mTc-HYNIC-TOC Scan and FDG PET/CT. 61
32956111 2021
36
Cutaneous Involvement as Initial Presentation of Multicentric Plasmacytic Castleman Disease. 61
33469928 2021
37
Human herpesvirus-8-positive primary effusion lymphoma in HIV-negative patients: Single institution case series with a multidisciplinary characterization. 61
32975904 2021
38
Membranous nephropathy with thrombotic microangiopathy-like lesions successfully treated with tocilizumab in a patient with idiopathic multicentric Castleman disease. 61
33389670 2021
39
Autoimmunity Due to Unicentric Castleman Disease Cured by Resection of a Hepatic Mass. 61
31609813 2021
40
Immune-mediated ataxia with cerebellar hemiatrophy and dystonia associated with Castleman disease. 61
33338826 2021
41
Idiopathic multicentric Castleman disease with novel heterozygous Ile729Met mutation in exon 10 of familial Mediterranean fever gene. 61
32830263 2021
42
Weathering the COVID-19 storm: Lessons from hematologic cytokine syndromes. 61
32425294 2021
43
Weathering the COVID-19 storm: Lessons from hematologic cytokine syndromes. 61
33558024 2021
44
Adult Langerhans cell histiocytosis of skull in a patient with synchronous papillary thyroid carcinoma and Castleman disease. 61
33495189 2021
45
[A Case of Venous Malformation that Occurred in the Retroperitoneum]. 61
33535291 2021
46
POEMS syndrome: A multisystem clonal disorder. 61
32889731 2021
47
Multicentric Plasma-Cell Type Castleman Disease Masquerading As Hodgkin Lymphoma: A Case Report. 61
33268942 2020
48
Bartonella henselae Neuroretinitis: A Rare Coinfection in POEMS Syndrome. 61
33389938 2020
49
Idiopathic multicentric Castleman disease with Sjögren's syndrome and secondary membranous nephropathy: a case report and review of the literature. 61
33276741 2020
50
International evidence-based consensus diagnostic and treatment guidelines for unicentric Castleman disease. 61
33284946 2020

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 32)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.98 PDGFRB MTOR IL6ST IL6R IL6 IL10
2
Show member pathways
13.54 PDGFRB MTOR IL6ST IL6R IL6 IL10
3
Show member pathways
13.36 PDGFRB MTOR IL6R IL6 IL10 CXCL13
4
Show member pathways
13.25 PDGFRB IL6R IL6 IL10 CXCL13 CCL4
5
Show member pathways
13.24 PDGFRB IL6ST IL6R IL6 IL10 IFNG
6 12.78 PDGFRB MTOR IL6ST IL6R IL6 IFNG
7
Show member pathways
12.51 MTOR IL6ST IL6R IL6 IL10 IFNG
8
Show member pathways
12.36 MTOR IL10 FCER2 CR2 CCL4
9
Show member pathways
12.29 IL6ST IL6R IL6 IFNG
10
Show member pathways
12.27 MTOR IL6ST IL6R IL6
11 12.18 IL6 IL10 IFNG CR1
12 12.13 MIR210 IL6R IL6 IL10
13
Show member pathways
11.97 IL6ST IL6R IL6 IL10 CXCL13 CCL4
14 11.89 MTOR IL6R IL6 IFNG
15 11.82 IL6R IL6 IL10 FSCN1 FCER2
16
Show member pathways
11.8 IL6ST IL6R IL6 IFNG
17
Show member pathways
11.76 IL6ST IL6R IL6 CRP
18 11.74 MTOR IL6ST IL6R IL6 IFNG
19 11.7 IL6 IL10 IFNG
20
Show member pathways
11.69 IL6ST IL6 IL10 IFNG
21 11.65 IL6R IL6 FCER2 CR2 CR1
22 11.62 MTOR IL10 FCER2
23
Show member pathways
11.57 IL6ST IL6R IL6
24
Show member pathways
11.57 PDGFRB MTOR IL6ST IL6R IL6 IL10
25 11.43 IL6 IL10 IFNG CR1
26
Show member pathways
11.42 IL6ST IL6R IL6
27 11.4 IL6 IL10 FCER2 CCL4
28 11.34 IL6 IL10 IFNG
29
Show member pathways
11.32 IL10 IFNG CCL4
30 11.28 IL6ST IL6R IL6
31 11.2 IL6 IL10 IFNG
32 11.17 IL6 IL10 CXCL13 CR2 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 region GO:0005576 9.97 IL6ST IL6R IL6 IL10 IFNG FCER2
2 extracellular space GO:0005615 9.91 MIR210 IL6ST IL6R IL6 IL10 IFNG
3 receptor complex GO:0043235 9.35 PDGFRB IL6ST IL6R CR2 ACVRL1
4 ciliary neurotrophic factor receptor complex GO:0070110 9.16 IL6ST IL6R
5 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 positive regulation of cell proliferation GO:0008284 9.95 PDGFRB IL6ST IL6R IL6 IFNG CR1
2 immune response GO:0006955 9.88 IL6 IL10 IFNG CXCL13 CR2 CCL4
3 regulation of actin cytoskeleton organization GO:0032956 9.79 PDGFRB MTOR FSCN1
4 positive regulation of endothelial cell proliferation GO:0001938 9.78 MTOR IL10 ACVRL1
5 response to activity GO:0014823 9.72 MTOR IL6 IL10
6 monocyte chemotaxis GO:0002548 9.7 IL6R IL6 CCL4
7 positive regulation of chemokine production GO:0032722 9.69 IL6R IL6 IFNG
8 acute-phase response GO:0006953 9.67 IL6R IL6 CRP
9 positive regulation of MHC class II biosynthetic process GO:0045348 9.62 IL10 IFNG
10 neutrophil mediated immunity GO:0002446 9.62 IL6R IL6
11 negative regulation of lipid storage GO:0010888 9.6 IL6 CRP
12 positive regulation of extracellular matrix disassembly GO:0090091 9.59 IL6 FSCN1
13 retina vasculature development in camera-type eye GO:0061298 9.58 PDGFRB ACVRL1
14 T-helper 17 cell lineage commitment GO:0072540 9.58 IL6R IL6
15 vascular endothelial growth factor production GO:0010573 9.57 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 9.17 IL6ST IL6R IL6 IL10 FSCN1 FCER2

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-Mar-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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