MCID: CST005
MIFTS: 44

Castleman Disease

Categories: Rare diseases, Immune diseases

Aliases & Classifications for Castleman Disease

MalaCards integrated aliases for Castleman Disease:

Name: Castleman Disease 49 36 51
Angiofollicular Ganglionic Hyperplasia 49 69
Angiofollicular Lymph Hyperplasia 49
Angiolymphoid Hyperplasia 69

Classifications:



External Ids:

KEGG 36 H01479

Summaries for Castleman Disease

NIH Rare Diseases : 49 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 multicentric castleman disease and human herpesvirus 8. 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 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)
# Related Disease Score Top Affiliating Genes
1 multicentric castleman disease 33.7 CRP IL6 KRT15
2 human herpesvirus 8 31.8 IL6 IL6R KRT15
3 iron deficiency anemia 29.9 CRP IL6
4 dendritic cell tumor 29.9 CR1 CR2
5 pericardial effusion 29.8 CRP IL6
6 rosai-dorfman disease 29.8 CR2 CRP
7 plasmacytoma 29.5 IL6 IL6R
8 bronchiolitis obliterans 29.2 EVPL IL6
9 follicular dendritic cell sarcoma 29.2 CR1 CR2 EVPL
10 systemic lupus erythematosus 28.9 CR1 CR2 CRP IL6
11 unicentric castleman disease 12.4
12 angiofollicular lymph hyperplasia 12.3
13 pediatric castleman disease 12.1
14 kaposi sarcoma 11.8
15 cold agglutinin disease 10.4 CRP IL6
16 diabetic foot ulcers 10.4 CRP IL6
17 cryopyrin-associated periodic syndrome 10.4 CRP IL6
18 neutrophilia, hereditary 10.4 CRP IL6
19 acute cholangitis 10.4 CRP IL6
20 cardiogenic shock 10.4 CRP IL6
21 uremic pruritus 10.4 CRP IL6
22 spinal disease 10.4 CRP IL6
23 angina pectoris 10.4 CRP IL6
24 cecal disease 10.4 CRP IL6
25 relapsing polychondritis 10.4 CRP IL6
26 central nervous system vasculitis 10.4 CRP IL6
27 subacute thyroiditis 10.4 CRP IL6
28 intussusception 10.4 CRP IL6
29 louse-borne relapsing fever 10.4 CRP IL6
30 hypersensitivity reaction type iii disease 10.4 CRP IL6
31 ischemic heart disease 10.4 CRP IL6
32 mesenteric lymphadenitis 10.4 CRP IL6
33 systolic heart failure 10.4 CRP IL6
34 intermittent explosive disorder 10.4 CRP IL6
35 intermediate coronary syndrome 10.4 CRP IL6
36 prediabetes syndrome 10.4 CRP IL6
37 acute cystitis 10.4 CRP IL6
38 polyarteritis nodosa 10.4 CRP IL6
39 acute pyelonephritis 10.4 CRP IL6
40 apnea, obstructive sleep 10.4 CRP IL6
41 adult-onset still's disease 10.4 CRP IL6
42 perinatal necrotizing enterocolitis 10.4 CRP IL6
43 viral meningitis 10.4 CRP IL6
44 lymph node disease 10.4 CRP IL6
45 infective endocarditis 10.3 CRP IL6
46 proliferative glomerulonephritis 10.3 CR1 IL6
47 hemorrhagic fever with renal syndrome 10.3 CRP IL6
48 uterine anomalies 10.3 CRP IL6
49 peripheral artery disease 10.3 CRP IL6
50 bladder disease 10.3 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)
# 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 Ganciclovir triphosphate Phase 4,Phase 2
5 Antiviral Agents Phase 4,Phase 2,Early Phase 1
6
Suramin Approved, Investigational Phase 2 145-63-1 5361
7 Siltuximab Approved, Investigational Phase 2,Phase 1,Early Phase 1 541502-14-1
8
Doxorubicin Approved, Investigational Phase 2,Phase 1 23214-92-8 31703
9
Vincristine Approved, Investigational Phase 2,Phase 1 2068-78-2, 57-22-7 5978
10
Etoposide Approved Phase 2,Phase 1 33419-42-0 36462
11
Cyclophosphamide Approved, Investigational Phase 2,Phase 1 50-18-0, 6055-19-2 2907
12
Zidovudine Approved Phase 2 30516-87-1 35370
13
rituximab Approved Phase 2,Phase 1 174722-31-7 10201696
14
Bortezomib Approved, Investigational Phase 2 179324-69-7 387447 93860
15
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
16
Everolimus Approved Phase 2 159351-69-6 6442177
17
Lenograstim Approved, Investigational Phase 2 135968-09-1
18
Prednisone Approved, Vet_approved Phase 2,Phase 1 53-03-2 5865
19
Sirolimus Approved, Investigational Phase 2 53123-88-9 46835353 6436030 5284616
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 Histamine Antagonists Phase 2
24 Histamine H1 Antagonists Phase 2
25 Anthelmintics Phase 2
26 Antibodies, Monoclonal Phase 2,Phase 1,Early Phase 1
27 Pharmaceutical Solutions Phase 2,Phase 1
28 Immunoglobulins Phase 2,Phase 1,Early Phase 1
29 Antibodies Phase 2,Phase 1,Early Phase 1
30 Antiprotozoal Agents Phase 2
31 Antipyretics Phase 2
32 Antiparasitic Agents Phase 2
33 interferons Phase 2
34 Alkylating Agents Phase 2,Phase 1
35 glucocorticoids Phase 2,Phase 1
36 Reverse Transcriptase Inhibitors Phase 2
37 Adjuvants, Immunologic Phase 2
38 Topoisomerase Inhibitors Phase 2,Phase 1
39 Nucleic Acid Synthesis Inhibitors Phase 2
40 Hormone Antagonists Phase 2,Phase 1
41 Angiogenesis Inhibitors Phase 2,Phase 1
42 Hormones Phase 2,Phase 1
43 Angiogenesis Modulating Agents Phase 2,Phase 1
44 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 2,Phase 1
45 Anti-Bacterial Agents Phase 2,Phase 1
46 Anti-HIV Agents Phase 2,Early Phase 1
47 Antifungal Agents Phase 2
48 Etoposide phosphate Phase 2,Phase 1
49 Immunosuppressive Agents Phase 2,Phase 1
50 Antimetabolites Phase 2

Interventional clinical trials:

(show all 27)

# 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 Molecular Characterization of Viral-associated Tumors, Tumors Occurring in the Setting of HIV or Other Immune Disorders and Castleman Disease Recruiting NCT03300830
25 Specimen Collections From Patients With HIV Infection, KSHV Infection, Viral-Related Pre-malignant Lesions and Cancer Recruiting NCT00006518
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

Inferred drug relations via UMLS 69 / NDF-RT 47 :


Genetic Tests for Castleman Disease

Anatomical Context for Castleman Disease

MalaCards organs/tissues related to Castleman Disease:

38
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 279)
# Title Authors Year
1
The full spectrum of Castleman disease: 273 patients studied over 20A years. ( 29143319 )
2018
2
Castleman Disease Pathogenesis. ( 29157613 )
2018
3
Clinical characteristics and outcomes of Castleman disease: A multicenter study of 185 Chinese patients. ( 29124835 )
2018
4
Treatment of Kaposi Sarcoma Herpesvirus-Associated Multicentric Castleman Disease. ( 29157621 )
2018
5
Diagnosis of Castleman Disease. ( 29157619 )
2018
6
Tocilizumab Cannot Prevent the Development of Bronchiolitis Obliterans in Patients With Castleman Disease-Associated Paraneoplastic Pemphigus. ( 29389687 )
2018
7
Unicentric Castleman Disease. ( 29157620 )
2018
8
Castleman Disease. ( 29157623 )
2018
9
The Peripheral Neuropathies of POEMS Syndrome and Castleman Disease. ( 29157616 )
2018
10
Castleman disease: delineating the spectrum. ( 29143317 )
2018
11
Pathology of Castleman Disease. ( 29157618 )
2018
12
The Role of Interleukin-6 in Castleman Disease. ( 29157617 )
2018
13
Castleman Disease and Rosai-Dorfman Disease. ( 29217303 )
2018
14
Treatment of Idiopathic Castleman Disease. ( 29157622 )
2018
15
Plasma Cell Variant Multicentric Castleman Disease and Kaposi's Sarcoma in a Treatment-Naive HIV-Infected Patient. ( 29345958 )
2018
16
Epidemiology of Castleman Disease. ( 29157611 )
2018
17
Castleman disease-associated diffuse parenchymal lung disease: A STROBE-compliant retrospective observational analysis of 22 cases in a tertiary Chinese hospital. ( 28953671 )
2017
18
Relapse of HHV8-positive multicentric Castleman disease following rituximab-based therapy in HIV-positive patients. ( 28143881 )
2017
19
Coexistence of Mediastinal Castleman Disease with Spindle Cell Carcinoma of the Lung. ( 28661181 )
2017
20
Salvage therapy with lenalidomide containing regimen for relapsed/refractory Castleman disease: a report of three cases. ( 28367597 )
2017
21
A reference guide for management of Castleman disease. ( 28321096 )
2017
22
Follicular lymphoma with hyaline-vascular Castleman disease-like follicles and CD20 positive follicular dendritic cells. ( 28689633 )
2017
23
Resection of unicentric interlobar Castleman disease with following adjuvant radiotherapy. ( 29225882 )
2017
24
Atypical laboratory presentation of paraneoplastic pemphigus associated with Castleman disease. ( 28367488 )
2017
25
Late-onset skin involvement on the forehead in multicentric Castleman disease. ( 28244070 )
2017
26
Developmental Therapeutics Implications of Next-Generation Sequencing in Human Herpesvirus 8-Negative Castleman Disease. ( 28241212 )
2017
27
Multicentric Castleman disease of hyaline vascular variant presenting with unusual systemic manifestations: a case report. ( 28501028 )
2017
28
A possible new morphological variant of mantle cell lymphoma with plasma-cell type Castleman disease-like features. ( 28974340 )
2017
29
Tentative diagnostic criteria and disease severity classification for Castleman disease: A report of the research group on Castleman disease in Japan. ( 28880697 )
2017
30
JAK1 Genomic Alteration Associated With Exceptional Response to Siltuximab in Cutaneous Castleman Disease. ( 28241173 )
2017
31
Castleman Disease Presenting as an Abdominal Mass. ( 28584844 )
2017
32
Corrigendum: Clinical manifestations of Kaposi sarcoma herpesvirus lytic activation: multicentric Castleman disease (KSHV-MCD) and the KSHV inflammatory cytokine syndrome. ( 28845155 )
2017
33
TAFRO syndrome: New subtype of idiopathic multicentric Castleman disease. ( 28135567 )
2017
34
International, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease. ( 28087540 )
2017
35
Paraneoplastic pemphigus associated with Castleman disease: progression from mucous to mucocutaneous lesions with epitope-spreading phenomena. ( 28213962 )
2017
36
EBV+ HHV-8+ Multicentric Castleman Disease With Plasmablastic Aggregates in an HIV+ Man: An Evolving Clinicopathologic Entity. ( 29198143 )
2017
37
Unusual Presentation of Castleman Disease in the Oral Cavity. ( 28468192 )
2017
38
Multicentric Castleman disease: consensus at last? ( 28336727 )
2017
39
Hodgkin Lymphoma and Castleman Disease: When One Blood Disease Can Hide Another. ( 28197347 )
2017
40
Classical Hodgkin lymphoma and Castleman disease: a rare morphologic combination. ( 28729340 )
2017
41
Thin-section Computed Tomographic Findings of Multicentric Castleman Disease Changing Over 10 Years. ( 28914746 )
2017
42
Is tocilizumab a potential therapeutic option for refractory unicentric Castleman disease? ( 28401573 )
2017
43
Case report and literature review: Glomerular and neurologic thrombotic microangiopathy as a primary manifestation of multicentric castleman disease. ( 27741115 )
2016
44
Emerging treatments in Castleman disease - a critical appraisal of siltuximab. ( 26869762 )
2016
45
Multicentric Castleman Disease With Tubulointerstitial Nephritis Mimicking IgG4-related Disease: Two Case Reports. ( 26598921 )
2016
46
Unicentric Castleman Disease: An Unusual Cause of An Isolated Neck Mass. ( 27660550 )
2016
47
Autoimmune thrombotic thrombocytopenic purpura associated with HHV8-related Multicentric Castleman disease. ( 27221364 )
2016
48
Rare Submandibular Presentation of Pediatric Castleman Disease: Case Report. ( 27870926 )
2016
49
Human Herpesvirus Type 8-positive Multicentric Castleman Disease. ( 27133959 )
2016
50
iNKT and memory B cells alterations in HHV-8 multicentric Castleman disease. ( 28060720 )
2016

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

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 9.43 CR1 CR2 CRP DES EVPL KRT15
2 intermediate filament cytoskeleton GO:0045111 9.16 DES EVPL
3 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)
# 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:

# 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
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 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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