MCID: CST005
MIFTS: 48

Castleman Disease

Categories: Rare diseases, Immune diseases

Aliases & Classifications for Castleman Disease

MalaCards integrated aliases for Castleman Disease:

Name: Castleman Disease 12 53 37 55 44
Angiofollicular Ganglionic Hyperplasia 53 44 73
Angiofollicular Lymph Node Hyperplasia 12 53
Angiofollicular Lymph Hyperplasia 12 53
Giant Lymph Node Hyperplasia 12
Angiolymphoid Hyperplasia 73
Castleman's Disease 76
Lymphoid Hamartoma 12
Alnh 53

Classifications:



External Ids:

Disease Ontology 12 DOID:0111157
ICD10 33 D47.4
KEGG 37 H01479

Summaries for Castleman Disease

NIH Rare Diseases : 53 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.

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.

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

Wikipedia : 76 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 185)
# Related Disease Score Top Affiliating Genes
1 multicentric castleman disease 33.8 CRP IL6 KRT15
2 human herpesvirus 8 32.7 IL6 IL6R
3 iron deficiency anemia 30.3 CRP IL6
4 pericardial effusion 30.2 CRP IL6
5 dendritic cell tumor 30.1 CR1 CR2
6 rosai-dorfman disease 30.0 CR2 CRP
7 plasmacytoma 29.8 IL6 IL6R
8 myeloma, multiple 29.5 CRP IL6 IL6R
9 bronchiolitis obliterans 29.2 EVPL IL6
10 follicular dendritic cell sarcoma 29.1 CR1 CR2 EVPL
11 systemic lupus erythematosus 28.9 CR1 CR2 CRP IL6
12 unicentric castleman disease 12.4
13 angiomatous lymphoid hamartoma 12.2
14 pediatric castleman disease 12.1
15 kaposi sarcoma 11.8
16 cold agglutinin disease 10.7 CRP IL6
17 cryopyrin-associated periodic syndrome 10.7 CRP IL6
18 cardiogenic shock 10.7 CRP IL6
19 acute cholangitis 10.7 CRP IL6
20 neutrophilia, hereditary 10.7 CRP IL6
21 uremic pruritus 10.7 CRP IL6
22 spinal disease 10.7 CRP IL6
23 angina pectoris 10.7 CRP IL6
24 cecal disease 10.7 CRP IL6
25 relapsing polychondritis 10.7 CRP IL6
26 central nervous system vasculitis 10.7 CRP IL6
27 subacute thyroiditis 10.7 CRP IL6
28 intussusception 10.7 CRP IL6
29 louse-borne relapsing fever 10.7 CRP IL6
30 hypersensitivity reaction type iii disease 10.6 CRP IL6
31 ischemic heart disease 10.6 CRP IL6
32 mesenteric lymphadenitis 10.6 CRP IL6
33 alexithymia 10.6 CRP IL6
34 intermittent explosive disorder 10.6 CRP IL6
35 intermediate coronary syndrome 10.6 CRP IL6
36 acute cystitis 10.6 CRP IL6
37 systolic heart failure 10.6 CRP IL6
38 polyarteritis nodosa 10.6 CRP IL6
39 prediabetes syndrome 10.6 CRP IL6
40 acute pyelonephritis 10.6 CRP IL6
41 apnea, obstructive sleep 10.6 CRP IL6
42 adult-onset still's disease 10.6 CRP IL6
43 perinatal necrotizing enterocolitis 10.6 CRP IL6
44 viral meningitis 10.6 CRP IL6
45 infective endocarditis 10.6 CRP IL6
46 hemorrhagic fever with renal syndrome 10.6 CRP IL6
47 lymph node disease 10.6 CRP IL6
48 exanthem 10.6 CRP IL6
49 uterine anomalies 10.6 CRP IL6
50 proliferative glomerulonephritis 10.6 CR1 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 62)
# 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, Investigational Phase 2,Phase 1,Early Phase 1 541502-14-1
7
Suramin Approved, Investigational 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, Investigational 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 Pharmaceutical Solutions Phase 2,Phase 1
30 Anthelmintics Phase 2
31 Antiparasitic Agents Phase 2
32 Antiprotozoal Agents Phase 2
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, Alkylating Phase 2,Phase 1
44 Antineoplastic Agents, Hormonal Phase 2,Phase 1
45 Antirheumatic Agents Phase 2,Phase 1
46 glucocorticoids Phase 2,Phase 1
47 Hormone Antagonists Phase 2,Phase 1
48 Hormones Phase 2,Phase 1
49 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 2,Phase 1
50 Immunosuppressive Agents Phase 2,Phase 1

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 A Study to Evaluate the Safety of Long-term Treatment With Siltuximab in Patients With Multicentric Castleman's Disease Completed NCT01400503 Phase 2 Siltuximab
4 Suramin in Treating Patients With Refractory or Relapsed Multiple Myeloma or Castleman's Disease Completed NCT00002652 Phase 2 suramin
5 17-N-Allylamino-17-Demethoxygeldanamycin in Treating Patients With Systemic Mastocytosis Completed NCT00132015 Phase 2 tanespimycin
6 Tocilizumab for KSHV-Associated Multicentric Castleman Disease Recruiting NCT01441063 Phase 2 Zidovudine;Tocilizumab;Valganciclovir (VGC)
7 TCP Regimen in Newly Diagnosed MCD:a Prospective, Single-center, Single-arm, Phase-II Pilot Trial Recruiting NCT03043105 Phase 2 Thalidomide, cyclophosphamide and prednisone
8 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
9 Siltuximab in Schizophrenia Recruiting NCT02796859 Phase 1, Phase 2 Siltuximab;normal saline
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 International Registry for Patients With Castleman Disease Recruiting NCT02817997
23 Unlock the Cell: Castleman's Disease Flow Cytometry Study Recruiting NCT02853968
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 73 / NDF-RT 51 :


Cochrane evidence based reviews: castleman disease

Genetic Tests for Castleman Disease

Anatomical Context for Castleman Disease

MalaCards organs/tissues related to Castleman Disease:

41
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 300)
# Title Authors Year
1
Effectiveness of rituximab-containing treatment regimens in idiopathic multicentric Castleman disease. ( 29732477 )
2018
2
Paraneoplastic Pemphigus Associated With Castleman Disease Detected by 18F-FDG PET/CT. ( 29561527 )
2018
3
The clinicopathological comparison among nodal cases of idiopathic multicentric Castleman disease with and without TAFRO syndrome. ( 29684500 )
2018
4
Renal failure in pediatric Castleman disease: Four French cases with thrombotic microangiopathy. ( 29603588 )
2018
5
Castleman Disease and Rosai-Dorfman Disease. ( 29217303 )
2018
6
Hepatobiliary and Pancreatic: Obliterative portal venopathy found in Castleman disease variant of POEMS syndrome. ( 29691905 )
2018
7
Role of Direct-Acting Antivirals in Hepatitis C Virus-Positive Castleman Disease. ( 29557805 )
2018
8
Castleman disease: delineating the spectrum. ( 29143317 )
2018
9
Castleman Disease Pathogenesis. ( 29157613 )
2018
10
Next-generation sequencing of idiopathic multicentric and unicentric Castleman disease and follicular dendritic cell sarcomas. ( 29496669 )
2018
11
The full spectrum of Castleman disease: 273 patients studied over 20A years. ( 29143319 )
2018
12
Castleman disease in the hilum liver mimicking the lymph node metastasis of hepatocellular carcinoma on 18F-FDG PET/CT: A case report. ( 29561464 )
2018
13
Castleman Disease. ( 29157623 )
2018
14
Treatment of Kaposi Sarcoma Herpesvirus-Associated Multicentric Castleman Disease. ( 29157621 )
2018
15
Clinical and molecular characteristics of unicentric mediastinal Castleman disease. ( 29850111 )
2018
16
Unicentric Castleman Disease. ( 29157620 )
2018
17
Pathology of Castleman Disease. ( 29157618 )
2018
18
Rare Forms of Castleman Disease Mimicking Malignancy: Mesenteric and Pancreatic Involvement. ( 29755906 )
2018
19
Plasma Cell Variant Multicentric Castleman Disease and Kaposi's Sarcoma in a Treatment-Naive HIV-Infected Patient. ( 29345958 )
2018
20
Treatment of Idiopathic Castleman Disease. ( 29157622 )
2018
21
The Peripheral Neuropathies of POEMS Syndrome and Castleman Disease. ( 29157616 )
2018
22
The Role of Interleukin-6 in Castleman Disease. ( 29157617 )
2018
23
Clinical characteristics and outcomes of Castleman disease: A multicenter study of 185 Chinese patients. ( 29124835 )
2018
24
(18F)-FDG-PET/MRI of unicentric retroperitoneal Castleman disease in a pediatric patient. ( 29604602 )
2018
25
Diagnosis of Castleman Disease. ( 29157619 )
2018
26
Plasma proteomics identifies a 'chemokine storm' in idiopathic multicentric Castleman disease. ( 29675946 )
2018
27
Chronic Myelomonocytic Leukemia following Multicentric Castleman Disease. ( 29607228 )
2018
28
Indolent T-lymphoblastic proliferation associated with low grade follicular dendritic cell sarcoma and Castleman disease. ( 29486963 )
2018
29
Epidemiology of Castleman Disease. ( 29157611 )
2018
30
Tocilizumab histologically improved AA renal amyloidosis in a patient with multicentric Castleman disease: A case reporta8c. ( 29701172 )
2018
31
Tocilizumab Cannot Prevent the Development of Bronchiolitis Obliterans in Patients With Castleman Disease-Associated Paraneoplastic Pemphigus. ( 29389687 )
2018
32
The Clinical and Immunologic Features of Patients With Combined Anti-GBM Disease and Castleman Disease. ( 29510921 )
2018
33
Is tocilizumab a potential therapeutic option for refractory unicentric Castleman disease? ( 28401573 )
2017
34
Salvage therapy with lenalidomide containing regimen for relapsed/refractory Castleman disease: a report of three cases. ( 28367597 )
2017
35
Hodgkin Lymphoma and Castleman Disease: When One Blood Disease Can Hide Another. ( 28197347 )
2017
36
Classical Hodgkin lymphoma and Castleman disease: a rare morphologic combination. ( 28729340 )
2017
37
Paraneoplastic pemphigus associated with Castleman disease: progression from mucous to mucocutaneous lesions with epitope-spreading phenomena. ( 28213962 )
2017
38
Late-onset skin involvement on the forehead in multicentric Castleman disease. ( 28244070 )
2017
39
Multicentric Castleman disease: consensus at last? ( 28336727 )
2017
40
EBV+ HHV-8+ Multicentric Castleman Disease With Plasmablastic Aggregates in an HIV+ Man: An Evolving Clinicopathologic Entity. ( 29198143 )
2017
41
A possible new morphological variant of mantle cell lymphoma with plasma-cell type Castleman disease-like features. ( 28974340 )
2017
42
A reference guide for management of Castleman disease. ( 28321096 )
2017
43
Castleman Disease Presenting as an Abdominal Mass. ( 28584844 )
2017
44
International, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease. ( 28087540 )
2017
45
Resection of unicentric interlobar Castleman disease with following adjuvant radiotherapy. ( 29225882 )
2017
46
TAFRO syndrome: New subtype of idiopathic multicentric Castleman disease. ( 28135567 )
2017
47
Corrigendum: Clinical manifestations of Kaposi sarcoma herpesvirus lytic activation: multicentric Castleman disease (KSHV-MCD) and the KSHV inflammatory cytokine syndrome. ( 28845155 )
2017
48
Multicentric Castleman disease of hyaline vascular variant presenting with unusual systemic manifestations: a case report. ( 28501028 )
2017
49
JAK1 Genomic Alteration Associated With Exceptional Response to Siltuximab in Cutaneous Castleman Disease. ( 28241173 )
2017
50
Thin-section Computed Tomographic Findings of Multicentric Castleman Disease Changing Over 10 Years. ( 28914746 )
2017

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:

37
# 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 13)
# Name GO ID Score Top Affiliating Genes
1 complement activation, classical pathway GO:0006958 9.54 CR1 CR2 CRP
2 positive regulation of osteoblast differentiation GO:0045669 9.52 IL6 IL6R
3 positive regulation of smooth muscle cell proliferation GO:0048661 9.51 IL6 IL6R
4 positive regulation of interleukin-6 production GO:0032755 9.49 IL6 IL6R
5 monocyte chemotaxis GO:0002548 9.48 IL6 IL6R
6 endocrine pancreas development GO:0031018 9.46 IL6 IL6R
7 positive regulation of leukocyte chemotaxis GO:0002690 9.43 IL6 IL6R
8 positive regulation of chemokine production GO:0032722 9.4 IL6 IL6R
9 interleukin-6-mediated signaling pathway GO:0070102 9.32 IL6 IL6R
10 complement receptor mediated signaling pathway GO:0002430 9.26 CR1 CR2
11 negative regulation of lipid storage GO:0010888 9.16 CRP IL6
12 acute-phase response GO:0006953 9.13 CRP IL6 IL6R
13 hepatic immune response GO:0002384 8.62 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
17 ExPASy
19 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 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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