MCID: UNC014
MIFTS: 30

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 53
Localized Castleman Disease 53 59
Unicentric Angiofollicular Ganglionic Hyperplasia 53
Localized Angiofollicular Lymphoid Hyperplasia 73
Unicentric Angiofollicular Lymph Hyperplasia 53
Angiolymphoid Hyperplasia 73

Classifications:



External Ids:

Orphanet 59 ORPHA93685
ICD10 via Orphanet 34 D36.0
UMLS 73 C3898582

Summaries for Unicentric Castleman Disease

NIH Rare Diseases : 53 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.

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 Folate Metabolism. 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 14)
# Related Disease Score Top Affiliating Genes
1 castleman disease 11.6
2 multicentric castleman disease 11.2
3 microcytic anemia 10.1
4 prostate cancer 9.9
5 sarcoma 9.9
6 nephrotic syndrome 9.9
7 prostatitis 9.9
8 cervicitis 9.9
9 thymoma 9.9
10 pancreatitis 9.9
11 follicular dendritic cell sarcoma 9.9
12 cryopyrin-associated periodic syndrome 9.3 CRP SAA4
13 amyloidosis aa 9.2 CRP SAA4
14 familial mediterranean fever 9.0 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 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 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
rituximab Approved Phase 2,Phase 1 174722-31-7 10201696
9
Lenograstim Approved, Investigational Phase 2 135968-09-1
10
Etoposide Approved Phase 2,Phase 1 33419-42-0 36462
11
Doxorubicin Approved, Investigational Phase 2,Phase 1 23214-92-8 31703
12
Vincristine Approved, Investigational Phase 2,Phase 1 2068-78-2, 57-22-7 5978
13
Zidovudine Approved Phase 2 30516-87-1 35370
14
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
15
Cyclophosphamide Approved, Investigational Phase 2,Phase 1 50-18-0, 6055-19-2 2907
16
Everolimus Approved Phase 2 159351-69-6 6442177
17
Bortezomib Approved, Investigational Phase 2 179324-69-7 387447 93860
18
Sirolimus Approved, Investigational Phase 2 53123-88-9 5284616 6436030 46835353
19
Prednisone Approved, Vet_approved Phase 2,Phase 1 53-03-2 5865
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 Pharmaceutical Solutions Phase 2,Phase 1
27 Antibodies Phase 2,Phase 1,Early Phase 1
28 Immunoglobulins Phase 2,Phase 1,Early Phase 1
29 Antibodies, Monoclonal Phase 2,Phase 1,Early Phase 1
30 Antiparasitic Agents Phase 2
31 Antiprotozoal Agents Phase 2
32 Antipyretics Phase 2
33 interferons Phase 2
34 Alkylating Agents Phase 2,Phase 1
35 glucocorticoids Phase 2,Phase 1
36 Adjuvants, Immunologic Phase 2
37 Reverse Transcriptase Inhibitors Phase 2
38 Topoisomerase Inhibitors Phase 2,Phase 1
39 Hormone Antagonists Phase 2,Phase 1
40 Nucleic Acid Synthesis Inhibitors Phase 2
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 Antibiotics, Antitubercular Phase 2,Phase 1
47 Etoposide phosphate Phase 2,Phase 1
48 Immunosuppressive Agents Phase 2,Phase 1
49 Interferon-alpha Phase 2
50 Antifungal Agents 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 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 Unicentric Castleman Disease

Inferred drug relations via UMLS 73 / NDF-RT 51 :


Genetic Tests for Unicentric Castleman Disease

Anatomical Context for Unicentric Castleman Disease

MalaCards organs/tissues related to Unicentric Castleman Disease:

41
Lymph Node, B Cells, Bone, Prostate

Publications for Unicentric Castleman Disease

Articles related to Unicentric Castleman Disease:

(show all 28)
# Title Authors Year
1
Next-generation sequencing of idiopathic multicentric and unicentric Castleman disease and follicular dendritic cell sarcomas. ( 29496669 )
2018
2
Unicentric Castleman Disease. ( 29157620 )
2018
3
Is tocilizumab a potential therapeutic option for refractory unicentric Castleman disease? ( 28401573 )
2017
4
Perivesical unicentric Castleman disease initially suspected to be metastatic prostate cancer. ( 27141204 )
2016
5
Unicentric Castleman Disease: An Unusual Cause of An Isolated Neck Mass. ( 27660550 )
2016
6
Complete resection of unicentric Castleman disease in the superior mediastinum: A case report. ( 27318017 )
2016
7
Case Report Unicentric Castleman disease located in the anterior mediastinum misdiagnosed as invasive thymoma: a case report. ( 26125875 )
2015
8
Exuberant cortical thymocyte proliferation mimicking T-lymphoblastic lymphoma within recurrent large inguinal lymph node masses of localized Castleman disease. ( 25953658 )
2015
9
Unicentric Castleman disease: from screening mammography to PET/CT imaging. ( 25286148 )
2014
10
Sonographic findings of localized Castleman disease of the abdomen and pelvis. ( 25346169 )
2014
11
Endosonographic features of unicentric Castleman Disease. ( 26425506 )
2014
12
Intrapulmonary unicentric Castleman disease mimicking peripheral pulmonary malignancy. ( 26767055 )
2014
13
Iron-Refractory Microcytic Anemia as the Presenting Feature of Unicentric Castleman Disease in Children. ( 24367988 )
2013
14
Parapharengeal unicentric castleman disease with nephrotic syndrome. ( 23880810 )
2013
15
Unicentric Castleman disease relapsed after rituximab-CHOP chemotherapy or radiation therapy in an adolescent. ( 22258344 )
2012
16
Unicentric Castleman disease mimicking a pancreatic neoplasm. ( 22524754 )
2012
17
Localized Castleman disease of plasma cell type in the abdomen. ( 22040445 )
2011
18
Treatment of unicentric Castleman disease with neoadjuvant rituximab. ( 21051400 )
2010
19
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
20
Coronary artery pseudotumor: IgG4-related disease versus localized Castleman disease. ( 19134500 )
2009
21
Complete anemia reversal after surgical excision of mesenteric hyaline-vascular unicentric Castleman disease. ( 19865557 )
2009
22
Cervical unicentric Castleman disease presenting as a neck mass: case report and review of the literature. ( 19444782 )
2009
23
Localized Castleman disease in retroperitoneum: newly discovered features by multi-detector helical CT. ( 17619097 )
2008
24
Isolated microcytic anemia disclosing a unicentric Castleman disease: The interleukin-6/hepcidin pathway? ( 18549942 )
2008
25
Unicentric castleman disease is not clearly distinguished from multicentric type: a case report. ( 18765316 )
2008
26
Outcome and late complications of radiotherapy in patients with unicentric Castleman disease. ( 17118850 )
2006
27
Human herpesvirus 8 and Epstein-Barr virus coinfection in localized Castleman disease during pregnancy. ( 16266926 )
2005
28
Bilateral localized Castleman disease of the retroperitoneum. ( 8491324 )
1993

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

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
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
Content
Loading form....