MCID: UNC014
MIFTS: 28

Unicentric Castleman Disease

Categories: Rare diseases, Blood diseases, Immune diseases

Aliases & Classifications for Unicentric Castleman Disease

MalaCards integrated aliases for Unicentric Castleman Disease:

Name: Unicentric Castleman Disease 50
Localized Castleman Disease 50 56
Unicentric Angiofollicular Ganglionic Hyperplasia 50
Localized Angiofollicular Lymphoid Hyperplasia 69
Unicentric Angiofollicular Lymph Hyperplasia 50
Angiolymphoid Hyperplasia 69

Classifications:



External Ids:

Orphanet 56 ORPHA93685
ICD10 via Orphanet 34 D36.0

Summaries for Unicentric Castleman Disease

NIH Rare Diseases : 50 unicentric castleman disease (ucd) is a rare condition that affects the lymph nodes and related tissues. it is a form of castleman disease that is "localized" to a single set of lymph nodes (as opposed to multicentric castleman disease which has more widespread effects). ucd often starts as an enlarged lymph node. depending on the location of this enlarged node, some people will have no additional features of the condition, while others will develop symptoms when it pushes on nearby organs and/or tissues. the exact underlying cause of ucd is currently unknown. treatment typically includes surgical removal of the affected lymph node. last updated: 6/24/2015

MalaCards based summary : Unicentric Castleman Disease, also known as localized castleman disease, is related to castleman disease and multicentric castleman disease. An important gene associated with Unicentric Castleman Disease is CRP (C-Reactive Protein). 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 Unicentric Castleman Disease

Diseases related to Unicentric Castleman Disease via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 12)
id Related Disease Score Top Affiliating Genes
1 castleman disease 11.5
2 multicentric castleman disease 11.1
3 microcytic anemia 10.0
4 prostate cancer 9.8
5 nephrotic syndrome 9.8
6 prostatitis 9.8
7 cervicitis 9.8
8 thymoma 9.8
9 pancreatitis 9.8
10 amyloidosis nodular localized cutaneous 9.5 CRP SAA4
11 uniparental disomy of chromosome 2 9.4 CRP SAA4
12 crystal arthropathies 9.2 CRP SAA4

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



Diseases related to Unicentric Castleman Disease

Symptoms & Phenotypes for Unicentric Castleman Disease

Drugs & Therapeutics for Unicentric Castleman Disease

Drugs for Unicentric 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 Unicentric Castleman Disease

Genetic Tests for Unicentric Castleman Disease

Anatomical Context for Unicentric Castleman Disease

MalaCards organs/tissues related to Unicentric Castleman Disease:

39
Lymph Node, B Cells, Bone, Prostate

Publications for Unicentric Castleman Disease

Articles related to Unicentric Castleman Disease:

(show all 19)
id Title Authors Year
1
Is tocilizumab a potential therapeutic option for refractory unicentric Castleman disease? ( 28401573 )
2017
2
Complete resection of unicentric Castleman disease in the superior mediastinum: A case report. ( 27318017 )
2016
3
Perivesical unicentric Castleman disease initially suspected to be metastatic prostate cancer. ( 27141204 )
2016
4
Unicentric Castleman Disease: An Unusual Cause of An Isolated Neck Mass. ( 27660550 )
2016
5
Case Report Unicentric Castleman disease located in the anterior mediastinum misdiagnosed as invasive thymoma: a case report. ( 26125875 )
2015
6
Endosonographic features of unicentric Castleman Disease. ( 26425506 )
2014
7
Intrapulmonary unicentric Castleman disease mimicking peripheral pulmonary malignancy. ( 26767055 )
2014
8
Unicentric Castleman disease: from screening mammography to PET/CT imaging. ( 25286148 )
2014
9
Parapharengeal unicentric castleman disease with nephrotic syndrome. ( 23880810 )
2013
10
Iron-Refractory Microcytic Anemia as the Presenting Feature of Unicentric Castleman Disease in Children. ( 24367988 )
2013
11
Unicentric Castleman disease relapsed after rituximab-CHOP chemotherapy or radiation therapy in an adolescent. ( 22258344 )
2012
12
Unicentric Castleman disease mimicking a pancreatic neoplasm. ( 22524754 )
2012
13
Treatment of unicentric Castleman disease with neoadjuvant rituximab. ( 21051400 )
2010
14
Cervical unicentric Castleman disease presenting as a neck mass: case report and review of the literature. ( 19444782 )
2009
15
Complete anemia reversal after surgical excision of mesenteric hyaline-vascular unicentric Castleman disease. ( 19865557 )
2009
16
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. ( 19730402 )
2009
17
Isolated microcytic anemia disclosing a unicentric Castleman disease: The interleukin-6/hepcidin pathway? ( 18549942 )
2008
18
Unicentric castleman disease is not clearly distinguished from multicentric type: a case report. ( 18765316 )
2008
19
Outcome and late complications of radiotherapy in patients with unicentric Castleman disease. ( 17118850 )
2006

Variations for Unicentric Castleman Disease

Expression for Unicentric Castleman Disease

Search GEO for disease gene expression data for Unicentric Castleman Disease.

Pathways for Unicentric Castleman Disease

GO Terms for Unicentric Castleman Disease

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

id Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 8.62 CRP SAA4

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

id Name GO ID Score Top Affiliating Genes
1 acute-phase response GO:0006953 8.62 CRP SAA4

Sources for Unicentric 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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