MCID: UNC014
MIFTS: 31

Unicentric Castleman Disease

Categories: Rare diseases, Immune diseases, Blood diseases

Aliases & Classifications for Unicentric Castleman Disease

MalaCards integrated aliases for Unicentric Castleman Disease:

Name: Unicentric Castleman Disease 49
Localized Castleman Disease 49 55
Unicentric Angiofollicular Ganglionic Hyperplasia 49
Localized Angiofollicular Lymphoid Hyperplasia 69
Unicentric Angiofollicular Lymph Hyperplasia 49
Angiolymphoid Hyperplasia 69

Classifications:



External Ids:

Orphanet 55 ORPHA93685
ICD10 via Orphanet 33 D36.0

Summaries for Unicentric Castleman Disease

NIH Rare Diseases : 49 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), and among its related pathways/superpathways is Selenium Micronutrient Network. 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 13)
# Related Disease Score Top Affiliating Genes
1 castleman disease 11.6
2 multicentric castleman disease 11.2
3 microcytic anemia 10.1
4 thymoma, familial 9.9
5 chops syndrome 9.9
6 nephrotic syndrome 9.9
7 prostatitis 9.9
8 cervicitis 9.9
9 thymoma 9.9
10 pancreatitis 9.9
11 cryopyrin-associated periodic syndrome 9.6 CRP SAA4
12 amyloidosis aa 9.5 CRP SAA4
13 familial mediterranean fever 9.4 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)
# 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 Unicentric Castleman Disease

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


Genetic Tests for Unicentric Castleman Disease

Anatomical Context for Unicentric Castleman Disease

MalaCards organs/tissues related to Unicentric Castleman Disease:

38
Lymph Node, B Cells, Bone, Prostate

Publications for Unicentric Castleman Disease

Articles related to Unicentric Castleman Disease:

(show all 20)
# Title Authors Year
1
Unicentric Castleman Disease. ( 29157620 )
2018
2
Is tocilizumab a potential therapeutic option for refractory unicentric Castleman disease? ( 28401573 )
2017
3
Unicentric Castleman Disease: An Unusual Cause of An Isolated Neck Mass. ( 27660550 )
2016
4
Perivesical unicentric Castleman disease initially suspected to be metastatic prostate cancer. ( 27141204 )
2016
5
Complete resection of unicentric Castleman disease in the superior mediastinum: A case report. ( 27318017 )
2016
6
Case Report Unicentric Castleman disease located in the anterior mediastinum misdiagnosed as invasive thymoma: a case report. ( 26125875 )
2015
7
Endosonographic features of unicentric Castleman Disease. ( 26425506 )
2014
8
Intrapulmonary unicentric Castleman disease mimicking peripheral pulmonary malignancy. ( 26767055 )
2014
9
Unicentric Castleman disease: from screening mammography to PET/CT imaging. ( 25286148 )
2014
10
Parapharengeal unicentric castleman disease with nephrotic syndrome. ( 23880810 )
2013
11
Iron-Refractory Microcytic Anemia as the Presenting Feature of Unicentric Castleman Disease in Children. ( 24367988 )
2013
12
Unicentric Castleman disease relapsed after rituximab-CHOP chemotherapy or radiation therapy in an adolescent. ( 22258344 )
2012
13
Unicentric Castleman disease mimicking a pancreatic neoplasm. ( 22524754 )
2012
14
Treatment of unicentric Castleman disease with neoadjuvant rituximab. ( 21051400 )
2010
15
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
16
Complete anemia reversal after surgical excision of mesenteric hyaline-vascular unicentric Castleman disease. ( 19865557 )
2009
17
Cervical unicentric Castleman disease presenting as a neck mass: case report and review of the literature. ( 19444782 )
2009
18
Isolated microcytic anemia disclosing a unicentric Castleman disease: The interleukin-6/hepcidin pathway? ( 18549942 )
2008
19
Unicentric castleman disease is not clearly distinguished from multicentric type: a case report. ( 18765316 )
2008
20
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

Pathways related to Unicentric Castleman Disease according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
10.96 CRP SAA4

GO Terms for Unicentric Castleman Disease

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

# 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:

# 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
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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