MCID: CST005
MIFTS: 57

Castleman Disease

Categories: Immune diseases, Rare diseases

Aliases & Classifications for Castleman Disease

MalaCards integrated aliases for Castleman Disease:

Name: Castleman Disease 12 52 36 54 43 15
Angiofollicular Ganglionic Hyperplasia 52 43 71
Giant Lymph Node Hyperplasia 12 52 17
Angiofollicular Lymph Hyperplasia 12 52
Castleman's Disease 74 15
Angiofollicular Lymph Node Hyperplasia 12
Angiolymphoid Hyperplasia 71
Benign Giant Lymphoma 52
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 multicentric castleman disease and kaposi sarcoma. 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 Sirolimus and Everolimus have been mentioned in the context of this disorder. Affiliated tissues include lymph node, b cells and bone, 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.

NIH Rare Diseases : 52 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 867)
# Related Disease Score Top Affiliating Genes
1 multicentric castleman disease 34.4 PAX5 KRT15 IL6 IL5 IL10 CRP
2 kaposi sarcoma 33.3 VEGFA IL6ST IL6 CXCL8 CSF2
3 human herpesvirus 8 33.1 IL6ST IL6R IL6 BCL6
4 glomeruloid hemangioma 32.7 VEGFA IL6
5 angioimmunoblastic lymphadenopathy with dysproteinemia 31.2 IL6 IL5
6 paraneoplastic pemphigus 31.0 PPL EVPL DSP DSG3 DSG1
7 thrombocytosis 30.9 IL6 CSF2 CRP
8 lymphoid interstitial pneumonia 30.9 IL6 CSF2 CRP
9 sarcoma 30.8 VEGFA IL6ST IL6 CXCL8 CSF2 CR2
10 osteosclerotic myeloma 30.8 VEGFA IL6
11 hydronephrosis 30.8 VEGFA IL6 CXCL8 CRP
12 proliferative glomerulonephritis 30.8 IL6 IL4 CR1
13 pericardial effusion 30.7 VEGFA IL6 CRP
14 colitis 30.7 IL6 IL4 IL10 CXCL8
15 follicular dendritic cell sarcoma 30.7 PPL EVPL DSP DSG3 CR2 CR1
16 adult-onset still's disease 30.7 IL6 CXCL8 CRP
17 acquired immunodeficiency syndrome 30.7 IL6 IL10 CSF2 CRP CR2 BCL6
18 intussusception 30.7 VEGFA IL6 CRP
19 follicular lymphoma 30.7 PAX5 CSF2 CR2 CR1 BCL6
20 thyroiditis 30.7 IL6 IL4 IL10
21 exanthem 30.6 VEGFA IL6 IL10 CXCL8 CSF2 CRP
22 marginal zone b-cell lymphoma 30.6 PAX5 CR2 BCL6
23 mantle cell lymphoma 30.6 VEGFA PAX5 IL6 BCL6
24 endocarditis 30.6 IL6 IL10 CXCL8 CRP
25 plasma cell neoplasm 30.6 VEGFA PAX5 IL6 BCL6
26 ulcerative colitis 30.6 IL6 IL4 IL10 CXCL8 CRP
27 neutrophilia, hereditary 30.5 IL6 CSF2 CRP
28 bronchiolitis obliterans 30.5 PPL IL6 IL10 EVPL CXCL8 CRP
29 thrombocytopenia 30.5 PAX5 IL6 IL4 IL10 CXCL8 CSF2
30 pemphigus 30.5 PPL IL4 IL10 EVPL DSP DSG3
31 bronchiolitis 30.4 IL6 IL5 IL4 IL10 CXCL8 CRP
32 macular retinal edema 30.4 VEGFA IL6 CXCL8
33 pericarditis 30.4 IL6 CXCL8 CRP
34 obstructive jaundice 30.4 IL6 CXCL8 CRP
35 diarrhea 30.4 IL6 IL5 IL10 CXCL8
36 immune deficiency disease 30.4 IL6 IL4 IL10 CSF2 CR2 BCL6
37 radiculopathy 30.4 IL6 IL10 CXCL8
38 spondylitis 30.4 IL6 IL10 CRP
39 myelitis 30.4 IL6 IL10 CXCL8
40 b-cell growth factor 30.3 IL6 IL4
41 bronchopneumonia 30.3 IL6 IL10 CXCL8 CRP
42 pemphigus vulgaris, familial 30.3 PPL EVPL DSP DSG3 DSG1
43 urticaria 30.3 IL6 IL5 IL4 CRP
44 ecthyma 30.3 DSG1 CRP
45 bacterial sepsis 30.3 IL6 IL10 CXCL8 CRP
46 appendicitis 30.3 IL6 IL10 CXCL8 CRP
47 respiratory failure 30.3 IL6 IL5 IL10 CXCL8 CRP
48 retinal vascular disease 30.3 VEGFA IL6 CXCL8 CRP
49 plasmacytoma 30.3 VEGFA PAX5 IL6ST IL6R IL6 IL4
50 common variable immunodeficiency 30.3 IL6 IL5 IL4 IL10 CR2

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 serum interleukin-6 31 frequent (33%) HP:0030783
10 nausea and vomiting 31 occasional (7.5%) HP:0002017
11 cough 31 occasional (7.5%) HP:0012735
12 jaundice 31 occasional (7.5%) HP:0000952
13 abdominal distention 31 occasional (7.5%) HP:0003270
14 abdominal mass 31 occasional (7.5%) HP:0031500
15 generalized lymphadenopathy 31 occasional (7.5%) HP:0008940
16 decreased mean corpuscular volume 31 occasional (7.5%) HP:0025066
17 flank pain 31 occasional (7.5%) HP:0030157
18 renal insufficiency 31 very rare (1%) HP:0000083
19 dyspnea 31 very rare (1%) HP:0002094
20 hematuria 31 very rare (1%) HP:0000790
21 thrombocytopenia 31 very rare (1%) HP:0001873
22 intestinal obstruction 31 very rare (1%) HP:0005214
23 anasarca 31 very rare (1%) HP:0012050
24 restrictive cardiomyopathy 31 very rare (1%) HP:0001723
25 myelofibrosis 31 very rare (1%) HP:0011974
26 ureteral obstruction 31 very rare (1%) HP:0006000

MGI Mouse Phenotypes related to Castleman Disease:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 growth/size/body region MP:0005378 10.25 BCL6 CSF2 DSG1 DSG3 DSP IL10
2 homeostasis/metabolism MP:0005376 10.21 BCL6 CR2 CRP CSF2 DSG3 IL10
3 hematopoietic system MP:0005397 10.18 BCL6 CR2 CSF2 DSG3 IL10 IL4
4 immune system MP:0005387 10.17 BCL6 CR2 CRP CSF2 DSG3 IL10
5 digestive/alimentary MP:0005381 10.11 DSG3 DSP IL10 IL4 IL5 IL6
6 integument MP:0010771 10.03 BCL6 CSF2 DSG1 DSG3 DSP EVPL
7 craniofacial MP:0005382 10.02 CSF2 DSG3 DSP IL10 IL4 PAX5
8 liver/biliary system MP:0005370 9.76 BCL6 IL10 IL4 IL5 IL6 IL6R
9 neoplasm MP:0002006 9.56 CSF2 IL10 IL5 IL6 IL6R IL6ST
10 respiratory system MP:0005388 9.23 BCL6 CSF2 DSG3 IL10 IL4 IL5

Drugs & Therapeutics for Castleman Disease

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
Sirolimus Approved, Investigational Phase 2 53123-88-9 5284616 6436030 46835353
2
Everolimus Approved Phase 2 159351-69-6 6442177 70789204
3
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
4
Dexamethasone Approved, Investigational, Vet_approved Phase 2 50-02-2 5743
5
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 2 1177-87-3
6
Bortezomib Approved, Investigational Phase 2 179324-69-7 387447 93860
7 Siltuximab Approved, Investigational Phase 1, Phase 2 541502-14-1
8
Zidovudine Approved Phase 2 30516-87-1 35370
9
Lenograstim Approved, Investigational Phase 2 135968-09-1
10
Ganciclovir Approved, Investigational Phase 2 82410-32-0 3454
11
Suramin Investigational Phase 2 145-63-1 5361
12 Antiparasitic Agents Phase 2
13 Antiprotozoal Agents Phase 2
14 Anthelmintics Phase 2
15 Antipyretics Phase 2
16 Histamine H1 Antagonists Phase 2
17 Histamine Antagonists Phase 2
18 Analgesics Phase 2
19 Pharmaceutical Solutions Phase 2
20 Antibodies, Monoclonal Phase 1, Phase 2
21 Antibodies Phase 1, Phase 2
22 Immunoglobulins Phase 1, Phase 2
23 Antifungal Agents Phase 2
24 Antiemetics Phase 2
25 Gastrointestinal Agents Phase 2
26 Autonomic Agents Phase 2
27 BB 1101 Phase 2
28 Anti-Retroviral Agents Phase 2
29 Reverse Transcriptase Inhibitors Phase 2
30 Antimetabolites Phase 2
31 Ganciclovir triphosphate Phase 2
32 interferons Phase 2
33 Adjuvants, Immunologic Phase 2
34 Interferon-alpha Phase 2
35 Anti-HIV Agents Phase 2
36
Methotrexate Approved Phase 1 1959-05-2, 59-05-2 126941
37
Daunorubicin Approved Phase 1 20830-81-3 30323
38
leucovorin Approved Phase 1 58-05-9 6006 143
39
Cetuximab Approved Phase 1 205923-56-4 56842117 2333
40
Bevacizumab Approved, Investigational Phase 1 216974-75-3
41
Valproic acid Approved, Investigational Phase 1 99-66-1 3121
42
Folic acid Approved, Nutraceutical, Vet_approved Phase 1 59-30-3 6037
43 Folic Acid Antagonists Phase 1
44 Vitamin B Complex Phase 1
45 Vitamin B9 Phase 1
46 Podophyllotoxin Phase 1 518-28-5
47 Dermatologic Agents Phase 1
48 Folate Phase 1
49 Tranquilizing Agents Phase 1
50 Endothelial Growth Factors Phase 1

Interventional clinical trials:

(show all 37)
# 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 PHASE II PILOT STUDY OF SURAMIN IN PREVIOUSLY TREATED PATIENTS WITH MULTIPLE MYELOMA AND PATIENTS WITH CASTLEMAN'S DISEASE Completed NCT00002652 Phase 2 suramin
3 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)
4 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
5 A Phase II Clinical Trial of 17-(Allylamino)-17- Demethoxygeldanamycin (17-AAG, NSC 330507 and EPL Diluent, NSC 704057) in Adults With Systemic Mastocytosis Completed NCT00132015 Phase 2 tanespimycin
6 Phase IIa of Tocilizumab In the Treatment of Polymyalgia Rheumatica Completed NCT01396317 Phase 2 Tocilizumab
7 A Phase II, Single-arm Open-label Multi-center Study of Sirolimus in Previously Treated Idiopathic Multicentric Castleman Disease Recruiting NCT03933904 Phase 2 Sirolimus
8 Thalidomide, Cyclophosphamide and Prednisone in Newly Diagnosed Multicentric Castleman's Disease: a Prospective, Single-center, Single-arm, Phase-II Pilot Trial Recruiting NCT03043105 Phase 2 Thalidomide, cyclophosphamide and prednisone
9 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
10 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
11 Pilot Study of Tocilizumab in Patients With Symptomatic Kaposi Sarcoma Herpesvirus (KSHV) - Associated Multicentric Castleman Disease Recruiting NCT01441063 Phase 2 Zidovudine;Tocilizumab;Valganciclovir (VGC)
12 A Randomized Controlled Trial of Adjunctive Siltuximab in Schizophrenia Recruiting NCT02796859 Phase 1, Phase 2 Siltuximab;normal saline
13 Natural History Study of the KSHV Inflammatory Cytokine Syndrome (KICS) Incorporating Pilot Evaluation of KSHV Targeted Therapies Recruiting NCT01419561 Phase 2 Zidovudine;Liposomal Doxorubicin;Valganiclovir;Rituximab
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 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]
18 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
19 A Study of Siltuximab (Anti-IL-6 Monoclonal Antibody) Effects on the QT Interval in Subjects With Monoclonal Gammopathy of Undetermined Significance, Smoldering Multiple Myeloma, or Indolent Multiple Myeloma Completed NCT01219010 Phase 1
20 An Open-Label Trial of Tocilizumab in Schizophrenia Completed NCT01696929 Phase 1 Tocilizumab
21 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
22 A Phase 1 Trial of Pomalidomide in Combination With Liposomal Doxorubicin in Patients With Advanced or Refractory Kaposi Sarcoma Recruiting NCT02659930 Phase 1 liposomal doxorubicin;pomalidomide
23 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
24 A Randomized Controlled Trial of Adjunctive Tocilizumab in Schizophrenia Recruiting NCT02874573 Phase 1 Tocilizumab;Normal saline
25 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
26 Epithelioid Hemangioma of Bone And Soft Tissue: a Benign Tumor With Metastatic Potential Completed NCT03169595
27 UARK 91-004 Therapeutic Research in Multiple Myeloma Completed NCT00572338
28 A Companion Protocol to Evaluate Anogenital Human Papillomavirus (HPV) Infection and Anogenital Squamous Intraepithelial Lesions (ASIL) in Subjects Participating in AMC Clinical Trials Completed NCT00695422
29 KSHV Seroprevalence in Hospital Patients From San Antonio Completed NCT00006310
30 Unlock the Cell: Intracellular Inflammatory Pathways and Flow Cytometry Study for Castleman's Disease Recruiting NCT02853968
31 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
32 Molecular Characterization of Viral-associated Tumors, Tumors Occurring in the Setting of HIV or Other Immune Disorders and Castleman Disease Recruiting NCT03300830
33 Registry for Adults With Plasma Cell Disorders (PCD's) Recruiting NCT03717844
34 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
35 A Pilot Trial of Nelfinavir for the Lytic Activation and Treatment of Gammaherpesvirus-Related Tumors Terminated NCT02080416 Early Phase 1 Nelfinavir
36 The Castleman Disease Collaborative Network Biobank: A Collection of Biospecimens and Clinical Data to Facilitate Research Withdrawn NCT02871050
37 A Single-arm, Single Center, Pilot Study of Siltuximab, an Anti-IL6 Therapy, for Patients With Myelofibrosis Withdrawn NCT02805868 Early Phase 1

Search NIH Clinical Center for Castleman Disease

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


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, B Cells, Bone, T Cells, Lung, Skin, Thyroid

Publications for Castleman Disease

Articles related to Castleman Disease:

(show top 50) (show all 1262)
# 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. 54 61
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. 54 61
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. 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. 54 61
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
AA amyloidosis associated with Castleman disease: A case report and review of the literature. 61
32028407 2020
16
Comparison of the clinical characteristics of TAFRO syndrome and idiopathic multicentric Castleman disease in general internal medicine: a 6-year retrospective study. 61
31211492 2020
17
Long-term safety of siltuximab in patients with idiopathic multicentric Castleman disease: a prespecified, open-label, extension analysis of two trials. 61
32027862 2020
18
Hemosiderin deposition in lymph nodes of patients with plasma cell-type Castleman disease. 61
32037354 2020
19
Idiopathic multicentric Castleman disease: bringing order to chaos. 61
32027863 2020
20
Intrapericardial pneumonectomy for unicentric hilar castleman disease. 61
32024436 2020
21
Castleman Disease: An Uncommon Mass in the Retroperitoneum. 61
31812578 2020
22
Clinical features and treatment of 7 Chinese TAFRO syndromes from 96 de novo Castleman diseases: a 10-year retrospective study. 61
31938902 2020
23
Morphologic variant of follicular lymphoma reminiscent of hyaline-vascular Castleman disease. 61
32013324 2020
24
POEMS Syndrome: an Atypical Presentation with Chronic Diarrhoea and Asthenia. 61
32015961 2020
25
Peripheral T cell lymphoma coexisting with Castleman's disease: A case report and literature review. 61
31914048 2020
26
HHV-8-negative multicentric Castleman disease presenting as a crescentic immune complexes membranoproliferative glomerulonephritis. 61
31911409 2020
27
Retroperitoneal Castleman Disease Invading Iliac Vein and Inferior Vena Cava Treated by Tumorectomy with Vascular Repair: A Case Report. 61
31917225 2020
28
KSHV/HHV8-positive large B-cell lymphomas and associated diseases: a heterogeneous group of lymphoproliferative processes with significant clinicopathological overlap. 61
31527708 2020
29
Multicentric Castleman Disease as a Rare Cause of Diffuse Lung Cysts. 61
31914318 2020
30
Clinicopathologic characteristics of 342 patients with multicentric Castleman disease in Japan. 61
31829070 2020
31
A case of idiopathic multicentric Castleman disease in an alemtuzumab-treated patient with MS. 61
31806686 2020
32
A case of idiopathic multicentric Castleman disease in an alemtuzumab-treated patient with MS. 61
31704887 2020
33
18F-FDG PET/CT in lung B lymphoid hyperplasia-type Castleman disease. 61
31494704 2020
34
[Successful multidisciplinary management of tetraplegia with a thoracic operation. Unicentric, mediastinal Castleman disease]. 61
31884815 2020
35
Polyneuropathy, organomegaly, endocrinopathy, monoclonal band, and skin (POEMS) changes syndrome presenting with a pseudosensory level: a case report. 61
31878962 2019
36
Epidemiological analysis of multicentric and unicentric Castleman disease and TAFRO syndrome in Japan. 61
31708515 2019
37
Reactive lymphadenopathy with concurrent idiopathic plasma cell variant Castleman disease, amyloid deposition and non-caseating granulomas. 61
31662201 2019
38
Epidemiology of Castleman disease associated with AA amyloidosis: description of 2 new cases and literature review. 61
31364863 2019
39
Unusual presentation of an intra-abdominal mass: a rare case of Castleman disease of the greater omentum. 61
30230198 2019
40
Unicentric Castleman disease, hyaline vascular variant, stromal rich, with increased plasma cells and a high level of serum IL-6: Raising the diagnostic and therapeutic issues. 61
31630065 2019
41
Whole-exome sequencing identifies novel somatic alterations associated with outcomes in idiopathic multicentric Castleman disease. 61
31863597 2019
42
Multicentric Castleman disease with TAFRO syndrome and Sjögren's. 61
31893065 2019
43
Castleman disease in the hepatic-gastric space: A case report. 61
31911923 2019
44
Clinical and pathological characteristics of Castleman disease: an observational study in a Spanish tertiary hospital. 61
31305183 2019
45
A rare case of Castleman disease mimicking a pancreatic tumor. 61
31894755 2019
46
Induction of Kaposi Sarcoma Associated Herpesvirus-encoded Thymidine Kinase (ORF21) by X-Box Binding Protein-1. 61
31801863 2019
47
Calcified or ossified benign soft tissue lesions that may simulate malignancy. 61
31297550 2019
48
Severe warm-antibody autoimmune hemolytic anemia due to multicentric Castleman disease: Responding to rituximab. 61
30486744 2019
49
Idiopathic Multicentric Castleman Disease with Autoimmune Hemolytic Anemia and Production of Anti-drug Antibody against Tocilizumab. 61
31292389 2019
50
The life and death of the germinal center. 61
31751845 2019

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 top 50) (show all 55)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.92 VEGFA PPL IL6ST IL6R IL6 IL5
2
Show member pathways
13.84 VEGFA IL6R IL6 IL5 IL4 IL10
3
Show member pathways
13.52 VEGFA PPL KRT15 IL5 EVPL DSP
4
Show member pathways
13.32 IL6R IL6 IL5 IL4 IL10 CXCL8
5
Show member pathways
13.32 VEGFA IL6ST IL6R IL6 IL5 IL4
6
Show member pathways
12.96 VEGFA IL6ST IL6R IL6 CXCL8 CSF2
7
Show member pathways
12.88 VEGFA IL6 IL5 IL4 IL10 CXCL8
8 12.84 VEGFA IL6ST IL6R IL6 IL5 IL4
9
Show member pathways
12.63 IL6 IL5 IL4 IL10 CSF2
10
Show member pathways
12.62 IL6ST IL6R IL6 IL5 IL4 IL10
11
Show member pathways
12.61 VEGFA IL5 IL4 CSF2 CR2 BCL6
12 12.59 PAX5 IL6ST IL4 IL10 BCL6
13
Show member pathways
12.47 PPL KRT15 EVPL DSP DSG3 DSG1
14
Show member pathways
12.37 IL6ST IL6R IL6 IL10 CXCL8
15
Show member pathways
12.29 IL6 IL5 IL4 IL10 CXCL8
16 12.28 PAX5 IL6 CXCL8 CSF2 BCL6
17
Show member pathways
12.25 IL6ST IL6R IL6 IL5 IL4 CXCL8
18
Show member pathways
12.23 IL6ST IL6R IL6 IL5 IL4 IL10
19 12.18 VEGFA IL6R IL6 IL10 CXCL8
20
Show member pathways
12.09 IL6ST IL6R IL6 IL10
21 11.99 IL6 IL5 IL4 IL10 CSF2
22 11.98 IL6ST IL6R IL6 CXCL8
23 11.96 IL6 IL10 CXCL8 CSF2
24 11.93 IL6ST IL6R IL6 IL10
25 11.91 VEGFA IL6 CXCL8 CSF2
26
Show member pathways
11.87 IL6ST IL6R IL6 CRP
27
Show member pathways
11.86 IL6ST IL6R IL6 CXCL8 CSF2
28
Show member pathways
11.8 IL5 IL4 CXCL8 CSF2
29 11.79 IL6 CXCL8 CSF2
30 11.79 IL5 IL4 IL10
31 11.79 IL6 IL5 IL4 CXCL8 CSF2
32 11.77 IL6 IL10 CXCL8
33 11.76 IL6 IL4 IL10 CSF2
34 11.75 IL6 IL10 CSF2
35
Show member pathways
11.69 VEGFA IL6 CXCL8 CSF2
36 11.68 IL5 IL4 IL10 BCL6
37
Show member pathways
11.64 IL6ST IL6R IL6
38 11.63 IL6 CXCL8 CR1
39 11.57 VEGFA IL6R IL6 CXCL8
40 11.56 IL6 IL5 CSF2
41 11.56 VEGFA IL6R IL6
42 11.56 PAX5 IL6R IL10 CR2 BCL6
43 11.56 VEGFA IL6R IL6 IL4 IL10 CXCL8
44 11.55 IL6 IL10 CXCL8 CR1
45 11.53 IL5 IL4 IL10
46 11.49 IL6 IL4 IL10 CXCL8 CSF2
47
Show member pathways
11.48 IL6ST IL6R IL6
48 11.43 IL6 IL5 IL4 DSP CXCL8 CSF2
49 11.36 IL6ST IL6R IL6
50 11.35 IL6 IL5 IL4

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 10.02 VEGFA IL6ST IL6R IL6 IL5 IL4
2 intermediate filament GO:0005882 9.71 PPL KRT15 EVPL DSP
3 ficolin-1-rich granule membrane GO:0101003 9.54 DSP DSG1 CR1
4 cornified envelope GO:0001533 9.35 PPL EVPL DSP DSG3 DSG1
5 interleukin-6 receptor complex GO:0005896 9.33 IL6ST IL6R IL6
6 ciliary neurotrophic factor receptor complex GO:0070110 9.26 IL6ST IL6R
7 desmosome GO:0030057 9.02 PPL EVPL DSP DSG3 DSG1

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

(show all 30)
# Name GO ID Score Top Affiliating Genes
1 negative regulation of apoptotic process GO:0043066 10.02 VEGFA IL6ST IL6 IL4 IL10 BCL6
2 positive regulation of gene expression GO:0010628 10.01 VEGFA IL6 IL4 CSF2 CRP
3 positive regulation of cell proliferation GO:0008284 10.01 VEGFA IL6ST IL6R IL6 IL5 CSF2
4 inflammatory response GO:0006954 9.91 IL6 IL5 IL10 CXCL8 CRP BCL6
5 immune response GO:0006955 9.91 IL6 IL5 IL4 IL10 CXCL8 CSF2
6 cellular response to lipopolysaccharide GO:0071222 9.89 IL6 IL10 CXCL8 CSF2
7 positive regulation of T cell proliferation GO:0042102 9.78 IL6ST IL6 IL4
8 positive regulation of peptidyl-tyrosine phosphorylation GO:0050731 9.78 VEGFA IL6R IL6 IL5
9 positive regulation of osteoblast differentiation GO:0045669 9.77 IL6ST IL6R IL6
10 B cell differentiation GO:0030183 9.76 IL4 IL10 CR2 BCL6
11 positive regulation of B cell proliferation GO:0030890 9.74 IL5 IL4 BCL6
12 acute-phase response GO:0006953 9.73 IL6R IL6 CRP
13 keratinization GO:0031424 9.73 PPL KRT15 EVPL DSP DSG3 DSG1
14 positive regulation of JAK-STAT cascade GO:0046427 9.7 IL6 IL5 IL10
15 intermediate filament cytoskeleton organization GO:0045104 9.67 PPL EVPL DSP
16 positive regulation of podosome assembly GO:0071803 9.65 IL5 CSF2
17 positive regulation of immunoglobulin secretion GO:0051024 9.64 IL6 IL5
18 response to molecule of bacterial origin GO:0002237 9.64 IL10 CXCL8
19 positive regulation of cellular component movement GO:0051272 9.63 VEGFA BCL6
20 positive regulation of MHC class II biosynthetic process GO:0045348 9.63 IL4 IL10
21 cornification GO:0070268 9.63 PPL KRT15 EVPL DSP DSG3 DSG1
22 negative regulation of tumor necrosis factor biosynthetic process GO:0042536 9.62 IL4 IL10
23 negative regulation of lipid storage GO:0010888 9.62 IL6 CRP
24 ciliary neurotrophic factor-mediated signaling pathway GO:0070120 9.58 IL6ST IL6R
25 regulation of isotype switching GO:0045191 9.58 IL4 IL10
26 interleukin-6-mediated signaling pathway GO:0070102 9.58 IL6ST IL6R IL6
27 hepatic immune response GO:0002384 9.51 IL6R IL6
28 type 2 immune response GO:0042092 9.5 IL4 IL10 BCL6
29 positive regulation of tyrosine phosphorylation of STAT protein GO:0042531 9.43 VEGFA IL6ST IL6R IL6 IL4 CSF2
30 cytokine-mediated signaling pathway GO:0019221 9.32 VEGFA IL6ST IL6R IL6 IL5 IL4

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

# Name GO ID Score Top Affiliating Genes
1 protein binding GO:0005515 10.36 VEGFA PPL PAX5 KRT15 IL6ST IL6R
2 growth factor activity GO:0008083 9.43 VEGFA IL6 IL5 IL4 IL10 CSF2
3 ciliary neurotrophic factor receptor activity GO:0004897 9.37 IL6ST IL6R
4 interleukin-6 receptor binding GO:0005138 9.33 IL6ST IL6R IL6
5 interleukin-6 binding GO:0019981 9.32 IL6ST IL6R
6 interleukin-6 receptor activity GO:0004915 9.26 IL6ST IL6R
7 cytokine activity GO:0005125 9.17 VEGFA IL6 IL5 IL4 IL10 CXCL8

Sources for Castleman Disease

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
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
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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