MCID: CST005
MIFTS: 39

Castleman Disease

Categories: Rare diseases, Immune diseases

Aliases & Classifications for Castleman Disease

MalaCards integrated aliases for Castleman Disease:

Name: Castleman Disease 50 52
Angiofollicular Ganglionic Hyperplasia 50 69
Angiofollicular Lymph Hyperplasia 50
Angiolymphoid Hyperplasia 69

Classifications:



Summaries for Castleman Disease

NIH Rare Diseases : 50 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. last updated: 6/25/2015

MalaCards based summary : Castleman Disease, also known as angiofollicular ganglionic hyperplasia, is related to systemic lupus erythematosus and multicentric castleman disease. An important gene associated with Castleman Disease is IL6 (Interleukin 6), and among its related pathways/superpathways are Complement and coagulation cascades and Immune response Lectin induced complement pathway. The drugs Ganciclovir and Valganciclovir have been mentioned in the context of this disorder. Affiliated tissues include lymph node, b cells and bone.

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 170)
id Related Disease Score Top Affiliating Genes
1 systemic lupus erythematosus 28.6 CR1 CR2 CRP IL6
2 multicentric castleman disease 12.3
3 unicentric castleman disease 12.3
4 pediatric castleman disease 12.0
5 kaposi sarcoma 11.7
6 human herpesvirus 8 11.1
7 angiofollicular lymph hyperplasia 10.8
8 collagenopathy type 2 alpha 1 10.6 CRP IL6
9 crystal arthropathies 10.6 CRP IL6
10 shoulder impingement syndrome 10.6 CRP IL6
11 brachydactyly-distal symphalangism syndrome 10.6 CRP IL6
12 epstein-barr virus hepatitis 10.6 CRP IL6
13 x-linked dominant intellectual disability-epilepsy syndrome 10.6 CRP IL6
14 erythema elevatum diutinum 10.6 CRP IL6
15 chronic diarrhea due to guanylate cyclase 2c overactivity 10.6 CRP IL6
16 nodular nonsuppurative panniculitis 10.6 CRP IL6
17 hemangioma of spleen 10.6 CRP IL6
18 central nervous system leiomyosarcoma 10.6 CRP IL6
19 lung occult large cell carcinoma 10.6 CRP IL6
20 cicatricial lagophthalmos 10.6 CRP IL6
21 ariboflavinosis 10.6 CRP IL6
22 islet cell tumor 10.6 CRP IL6
23 queensland tick typhus 10.6 CRP IL6
24 placental abruption 10.6 CRP IL6
25 luxation of globe 10.6 CRP IL6
26 parasitic helminthiasis infectious disease 10.6 CRP IL6
27 allergic bronchopulmonary aspergillosis 10.6 CRP IL6
28 benign neonatal seizures 10.6 CRP IL6
29 hyperlipoproteinemia type iv 10.6 CRP IL6
30 partial central choroid dystrophy 10.6 CRP IL6
31 breast papillary carcinoma 10.6 CRP IL6
32 myeloid sarcoma 10.5 CRP IL6
33 pneumoconiosis 10.5 CRP IL6
34 early congenital syphilis 10.5 CRP IL6
35 heterotaxy, visceral, 4, autosomal 10.5 IL6 IL6R
36 plummer's disease 10.5 CRP IL6
37 residual stage corticosteroid-induced glaucoma 10.5 CRP IL6
38 diffuse glomerulonephritis 10.5 CR1 IL6
39 osteogenesis imperfecta, type x 10.5 IL6 IL6R
40 c1 inhibitor deficiency 10.5 CRP IL6
41 breast disease 10.5 CRP IL6
42 bardet-biedl syndrome 8 10.5 CRP IL6
43 brain glioblastoma multiforme 10.5 CRP IL6
44 focal dystonia 10.5 CRP IL6
45 ischemic heart disease 10.5 CRP IL6
46 urinary bladder posterior wall cancer 10.5 CRP IL6
47 keloids 10.5 IL6 IL6R
48 atrophy of prostate 10.5 CRP IL6
49 chondroma 10.5 CRP IL6
50 colonic disease 10.5 CRP IL6

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



Diseases related to Castleman Disease

Symptoms & Phenotypes for Castleman Disease

Drugs & Therapeutics for Castleman Disease

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

(show top 50) (show all 61)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Ganciclovir Approved, Investigational Phase 4,Phase 2 82410-32-0 3454
2
Valganciclovir Approved, Investigational Phase 4,Phase 2 175865-60-8 64147
3 Anti-Infective Agents Phase 4,Phase 2,Phase 1,Early Phase 1
4 Antiviral Agents Phase 4,Phase 2,Early Phase 1
5 Ganciclovir triphosphate Phase 4,Phase 2
6 Siltuximab Approved Phase 2,Phase 1,Early Phase 1 541502-14-1
7
Suramin Approved Phase 2 145-63-1 5361
8
Zidovudine Approved Phase 2 30516-87-1 35370
9
Cyclophosphamide Approved, Investigational Phase 2,Phase 1 50-18-0, 6055-19-2 2907
10
Prednisone Approved, Vet_approved Phase 2,Phase 1 53-03-2 5865
11
Bortezomib Approved, Investigational Phase 2 179324-69-7 387447 93860
12
Doxorubicin Approved, Investigational Phase 2,Phase 1 23214-92-8 31703
13
Etoposide Approved Phase 2,Phase 1 33419-42-0 36462
14
Everolimus Approved Phase 2 159351-69-6 6442177
15
Lenograstim Approved Phase 2 135968-09-1
16
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
17
rituximab Approved Phase 2,Phase 1 174722-31-7 10201696
18
Sirolimus Approved, Investigational Phase 2 53123-88-9 5284616 6436030 46835353
19
Vincristine Approved, Investigational Phase 2,Phase 1 2068-78-2, 57-22-7 5978
20 Pomalidomide Approved Phase 1, Phase 2 19171-19-8
21
Doxil Approved June 1999 Phase 2,Phase 1 31703
22 Analgesics Phase 2
23 Antibodies Phase 2,Phase 1,Early Phase 1
24 Antibodies, Monoclonal Phase 2,Phase 1,Early Phase 1
25 Antipyretics Phase 2
26 Histamine Antagonists Phase 2
27 Histamine H1 Antagonists Phase 2
28 Immunoglobulins Phase 2,Phase 1,Early Phase 1
29 Anthelmintics Phase 2
30 Antiparasitic Agents Phase 2
31 Antiprotozoal Agents Phase 2
32 Pharmaceutical Solutions Phase 2,Phase 1
33 Anti-HIV Agents Phase 2,Early Phase 1
34 Antimetabolites Phase 2
35 Anti-Retroviral Agents Phase 2,Early Phase 1
36 Nucleic Acid Synthesis Inhibitors Phase 2
37 Reverse Transcriptase Inhibitors Phase 2
38 Alkylating Agents Phase 2,Phase 1
39 Angiogenesis Inhibitors Phase 2,Phase 1
40 Angiogenesis Modulating Agents Phase 2,Phase 1
41 Anti-Bacterial Agents Phase 2,Phase 1
42 Anti-Inflammatory Agents Phase 2,Phase 1
43 Antineoplastic Agents, Hormonal Phase 2,Phase 1
44 Antirheumatic Agents Phase 2,Phase 1
45 glucocorticoids Phase 2,Phase 1
46 Hormone Antagonists Phase 2,Phase 1
47 Hormones Phase 2,Phase 1
48 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 2,Phase 1
49 Immunosuppressive Agents Phase 2,Phase 1
50 Adjuvants, Immunologic Phase 2

Interventional clinical trials:

(show all 27)

id Name Status NCT ID Phase Drugs
1 Valganciclovir to Treat HHV-8 Associated Multicentric Castleman's Disease Withdrawn NCT00361933 Phase 4 Valganciclovir
2 A Study to Evaluate the Efficacy and Safety of CNTO328 Plus Best Supportive Care in Multicentric Castleman's Disease Completed NCT01024036 Phase 2 Siltuximab;Placebo;Best Supportive Care (BSC)
3 Suramin in Treating Patients With Refractory or Relapsed Multiple Myeloma or Castleman's Disease Completed NCT00002652 Phase 2 suramin
4 17-N-Allylamino-17-Demethoxygeldanamycin in Treating Patients With Systemic Mastocytosis Completed NCT00132015 Phase 2 tanespimycin
5 Tocilizumab for KSHV-Associated Multicentric Castleman Disease Recruiting NCT01441063 Phase 2 Zidovudine;Tocilizumab;Valganciclovir (VGC)
6 TCP Regimen in Newly Diagnosed MCD:a Prospective, Single-center, Single-arm, Phase-II Pilot Trial Recruiting NCT03043105 Phase 2 Thalidomide, cyclophosphamide and prednisone
7 Virotherapy and Natural History Study of KHSV-Associated Multricentric Castleman s Disease With 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
8 Siltuximab in Schizophrenia Recruiting NCT02796859 Phase 1, Phase 2 Siltuximab;normal saline
9 A Study to Evaluate the Safety of Long-term Treatment With Siltuximab in Patients With Multicentric Castleman's Disease Active, not recruiting NCT01400503 Phase 2 Siltuximab
10 Rituximab in the Treatment of HIV Associated Multicentric Castleman Disease Dependent on Chemotherapy Terminated NCT00127569 Phase 2 Rituximab
11 Study of Pomalidomide Combined With Modified DA-EPOCH and Rituximab in KSHV-Associated Lymphomas Withdrawn NCT02228512 Phase 1, Phase 2 Pomalidomide;Rituximab;Prednisone;Etoposide;Doxorubicin;Vincristine;Cyclophosphamide
12 Dose-escalation Study of Oral CX-4945 Unknown status NCT00891280 Phase 1 CX-4945 oral formulation
13 A Study to Provide RoActemra/Actemra (Tocilzumab) to Patients With Multicentric Castleman's Disease Who Demonstrated Benefit From Previous RoActemra/Actemra Treatment Completed NCT01183598 Phase 1 tocilizumab [RoActemra/Actemra]
14 A Safety and Efficacy Study of CNTO 328 in Patients With B-Cell Non-Hodgkin's Lymphoma, Multiple Myeloma, or Castleman's Disease Completed NCT00412321 Phase 1 CNTO 328
15 An Open-Label Trial of Tocilizumab in Schizophrenia Completed NCT01696929 Phase 1 Tocilizumab
16 Tocilizumab in Schizophrenia Recruiting NCT02874573 Phase 1 Tocilizumab;Normal saline
17 Pomalidomide in Combination With Liposomal Doxorubicin in People With Advanced or Refractory Kaposi Sarcoma Recruiting NCT02659930 Phase 1 liposomal doxorubicin;pomalidomide
18 Ibrutinib in Treating Relapsed or Refractory B-Cell Non-Hodgkin Lymphoma in Patients With HIV Infection Terminated NCT02109224 Phase 1 Ibrutinib
19 Epithelioid Hemangioma of Bone And Soft Tissue Completed NCT03169595
20 Detecting Anal and Genital Human Papillomavirus Infection and Squamous Intraepithelial Lesions in HIV-Positive Patients Enrolled in AIDS Cancer Clinical Trials Completed NCT00695422
21 Castleman Disease Collaborative Network Biobank Recruiting NCT02871050
22 Unlock the Cell: Castleman's Disease Flow Cytometry Study Recruiting NCT02853968
23 International Registry for Patients With Castleman Disease Recruiting NCT02817997
24 Specimen Collections From Patients With HIV Infection, KSHV Infection, Viral-Related Pre-malignant Lesions and Cancer Recruiting NCT00006518
25 Molecular Characterization of Viral-associated Tumors, Tumors Occurring in the Setting of HIV or Other Immune Disorders and Castleman Disease Not yet recruiting NCT03300830
26 Nelfinavir for the Treatment of Gammaherpesvirus-Related Tumors Terminated NCT02080416 Early Phase 1 Nelfinavir
27 Siltuximab in Treating Patients With Primary, Post-Polycythemia Vera, or Post-Essential Thrombocythemia Myelofibrosis Withdrawn NCT02805868 Early Phase 1

Search NIH Clinical Center for Castleman Disease

Genetic Tests for Castleman Disease

Anatomical Context for Castleman Disease

MalaCards organs/tissues related to Castleman Disease:

39
Lymph Node, B Cells, Bone, Bone Marrow, T Cells, Skin, Lung

Publications for Castleman Disease

Articles related to Castleman Disease:

(show top 50) (show all 261)
id Title Authors Year
1
International, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease. ( 28087540 )
2017
2
Salvage therapy with lenalidomide containing regimen for relapsed/refractory Castleman disease: a report of three cases. ( 28367597 )
2017
3
Hodgkin Lymphoma and Castleman Disease: When One Blood Disease Can Hide Another. ( 28197347 )
2017
4
Developmental Therapeutics Implications of Next-Generation Sequencing in Human Herpesvirus 8-Negative Castleman Disease. ( 28241212 )
2017
5
Late-onset skin involvement on the forehead in multicentric Castleman disease. ( 28244070 )
2017
6
Classical Hodgkin lymphoma and Castleman disease: a rare morphologic combination. ( 28729340 )
2017
7
Is tocilizumab a potential therapeutic option for refractory unicentric Castleman disease? ( 28401573 )
2017
8
Tentative diagnostic criteria and disease severity classification for Castleman disease: A report of the research group on Castleman disease in Japan. ( 28880697 )
2017
9
Follicular lymphoma with hyaline-vascular Castleman disease-like follicles and CD20 positive follicular dendritic cells. ( 28689633 )
2017
10
Paraneoplastic pemphigus associated with Castleman disease: progression from mucous to mucocutaneous lesions with epitope-spreading phenomena. ( 28213962 )
2017
11
JAK1 Genomic Alteration Associated With Exceptional Response to Siltuximab in Cutaneous Castleman Disease. ( 28241173 )
2017
12
Thin-section Computed Tomographic Findings of Multicentric Castleman Disease Changing Over 10 Years. ( 28914746 )
2017
13
Castleman Disease Presenting as an Abdominal Mass. ( 28584844 )
2017
14
Castleman disease-associated diffuse parenchymal lung disease: A STROBE-compliant retrospective observational analysis of 22 cases in a tertiary Chinese hospital. ( 28953671 )
2017
15
Multicentric Castleman disease: consensus at last? ( 28336727 )
2017
16
Corrigendum: Clinical manifestations of Kaposi sarcoma herpesvirus lytic activation: multicentric Castleman disease (KSHV-MCD) and the KSHV inflammatory cytokine syndrome. ( 28845155 )
2017
17
Multicentric Castleman disease of hyaline vascular variant presenting with unusual systemic manifestations: a case report. ( 28501028 )
2017
18
Coexistence of Mediastinal Castleman Disease with Spindle Cell Carcinoma of the Lung. ( 28661181 )
2017
19
Atypical laboratory presentation of paraneoplastic pemphigus associated with Castleman disease. ( 28367488 )
2017
20
Unusual Presentation of Castleman Disease in the Oral Cavity. ( 28468192 )
2017
21
A reference guide for management of Castleman disease. ( 28321096 )
2017
22
Relapse of HHV8-positive multicentric Castleman disease following rituximab-based therapy in HIV-positive patients. ( 28143881 )
2017
23
TAFRO syndrome: New subtype of idiopathic multicentric Castleman disease. ( 28135567 )
2017
24
Emerging treatments in Castleman disease - a critical appraisal of siltuximab. ( 26869762 )
2016
25
TAFRO syndrome: An atypical variant of KSHV-negative multicentric Castleman disease. ( 26663467 )
2016
26
A Multidisciplinary Approach to Castleman Disease of the Neck. ( 26840960 )
2016
27
An Unusual Presentation of Plasma Cells - Castleman Disease: A Case Report. ( 27352442 )
2016
28
Diagnostic Utility of Interleukin-6 Expression by Immunohistochemistry in Differentiating Castleman Disease Subtypes and Reactive Lymphadenopathies. ( 27650613 )
2016
29
Clinical spectrum of Castleman disease-associated neuropathy. ( 27807187 )
2016
30
A HHV-8 positive, HIV negative multicentric Castleman disease treated with R-CEOP chemotherapy and valganciclovir combination. ( 26948831 )
2016
31
Bone marrow manifestations in multicentric Castleman disease. ( 26817834 )
2016
32
A case of paraneoplastic pemphigus associated with Castleman disease reacting with multiple autoantigens, including the p200 protein. ( 27115591 )
2016
33
Clinicopathologic analysis of TAFRO syndrome demonstrates a distinct subtype of HHV-8-negative multicentric Castleman disease. ( 26805758 )
2016
34
iNKT and memory B cells alterations in HHV-8 multicentric Castleman disease. ( 28060720 )
2016
35
Case report and literature review: Glomerular and neurologic thrombotic microangiopathy as a primary manifestation of multicentric castleman disease. ( 27741115 )
2016
36
Successful treatment of TAFRO syndrome, a variant type of multicentric Castleman disease with thrombotic microangiopathy, with anti-IL-6 receptor antibody and steroids. ( 26980221 )
2016
37
Autoimmune thrombotic thrombocytopenic purpura associated with HHV8-related Multicentric Castleman disease. ( 27221364 )
2016
38
Castleman Disease of the Thorax: Clinical, Radiologic, and Pathologic Correlation: From the Radiologic Pathology Archives. ( 27618318 )
2016
39
Multicentric Castleman disease: Where are we now? ( 27296355 )
2016
40
Intra-abdominal localized hyaline-vascular Castleman disease: imaging characteristics and management of a rare condition. ( 26925142 )
2016
41
Rare Submandibular Presentation of Pediatric Castleman Disease: Case Report. ( 27870926 )
2016
42
Immunohistochemical analysis of IgA expression differentiates IgG4-related disease from plasma cell-type Castleman disease. ( 27438709 )
2016
43
Dysplastic follicular dendritic cells in hyaline-vascular Castleman disease: a rare occurrence creating diagnostic difficulty. ( 27593552 )
2016
44
Update and new approaches in the treatment of Castleman disease. ( 27536166 )
2016
45
Unicentric versus Multicentric Castleman Disease. ( 27618317 )
2016
46
A pediatric patient with glycogen storage disease type IA and Castleman disease. ( 28035790 )
2016
47
Human Herpesvirus Type 8-positive Multicentric Castleman Disease. ( 27133959 )
2016
48
Atypical presentation of multicentric Castleman disease in a pediatric patient: pleural and pericardial effusion. ( 26614115 )
2016
49
Complete resection of unicentric Castleman disease in the superior mediastinum: A case report. ( 27318017 )
2016
50
Castleman Disease of Parotid Gland. ( 27391505 )
2016

Variations for Castleman Disease

Expression for Castleman Disease

Search GEO for disease gene expression data for Castleman Disease.

Pathways for Castleman Disease

GO Terms for Castleman Disease

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

id Name GO ID Score Top Affiliating Genes
1 interleukin-6 receptor complex GO:0005896 8.62 IL6 IL6R

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

(show all 12)
id Name GO ID Score Top Affiliating Genes
1 positive regulation of smooth muscle cell proliferation GO:0048661 9.51 IL6 IL6R
2 positive regulation of osteoblast differentiation GO:0045669 9.49 IL6 IL6R
3 positive regulation of interleukin-6 production GO:0032755 9.48 IL6 IL6R
4 monocyte chemotaxis GO:0002548 9.46 IL6 IL6R
5 endocrine pancreas development GO:0031018 9.43 IL6 IL6R
6 positive regulation of chemokine production GO:0032722 9.4 IL6 IL6R
7 positive regulation of leukocyte chemotaxis GO:0002690 9.37 IL6 IL6R
8 complement receptor mediated signaling pathway GO:0002430 9.32 CR1 CR2
9 interleukin-6-mediated signaling pathway GO:0070102 9.26 IL6 IL6R
10 negative regulation of lipid storage GO:0010888 9.16 CRP IL6
11 hepatic immune response GO:0002384 8.96 IL6 IL6R
12 acute-phase response GO:0006953 8.8 CRP IL6 IL6R

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

id Name GO ID Score Top Affiliating Genes
1 interleukin-6 receptor binding GO:0005138 8.62 IL6 IL6R

Sources for Castleman Disease

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