MCID: UNC014
MIFTS: 26

Unicentric Castleman Disease

Categories: Blood diseases, Immune diseases, Rare 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

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 Selenium Micronutrient Network. The drugs Ganciclovir and Valganciclovir have been mentioned in the context of this disorder. Affiliated tissues include lymph node and prostate.

Related Diseases for Unicentric Castleman Disease

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

(show all 18)
# Related Disease Score Top Affiliating Genes
1 castleman disease 11.8
2 multicentric castleman disease 11.4
3 microcytic anemia 10.2
4 prostate cancer 10.1
5 langerhans cell histiocytosis 10.1
6 bronchiolitis obliterans 10.1
7 nephrotic syndrome 10.1
8 bronchiolitis 10.1
9 thymoma 10.1
10 histiocytosis 10.1
11 pemphigus 10.1
12 human herpesvirus 8 9.8
13 lymphoblastic lymphoma 9.8
14 lymphoma 9.8
15 igg4-related disease 9.8
16 cryopyrin-associated periodic syndrome 9.8 CRP SAA4
17 amyloidosis aa 9.8 CRP SAA4
18 familial mediterranean fever 9.7 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 76)
# 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 Siltuximab Approved, Investigational Phase 2,Phase 1,Early Phase 1 541502-14-1
7
Zidovudine Approved Phase 2 30516-87-1 35370
8
Cyclophosphamide Approved, Investigational Phase 2,Phase 1 6055-19-2, 50-18-0 2907
9
Prednisone Approved, Vet_approved Phase 2,Phase 1 53-03-2 5865
10
Bortezomib Approved, Investigational Phase 2 179324-69-7 387447 93860
11
Lenograstim Approved, Investigational Phase 2 135968-09-1
12
Everolimus Approved Phase 2,Phase 1 159351-69-6 6442177
13
Vincristine Approved, Investigational Phase 2,Phase 1 57-22-7, 2068-78-2 5978
14
Doxorubicin Approved, Investigational Phase 2,Phase 1 23214-92-8 31703
15
Sirolimus Approved, Investigational Phase 2,Phase 1 53123-88-9 46835353 6436030 5284616
16
rituximab Approved Phase 2,Phase 1 174722-31-7 10201696
17
Etoposide Approved Phase 2,Phase 1 33419-42-0 36462
18
Miconazole Approved, Investigational, Vet_approved Phase 2,Phase 1 22916-47-8 4189
19 Pomalidomide Approved Phase 1, Phase 2 19171-19-8
20
Suramin Investigational Phase 2 145-63-1 5361
21
Doxil Approved June 1999 Phase 2,Phase 1 31703
22 Antibodies, Monoclonal Phase 2,Phase 1,Early Phase 1
23 Antibodies Phase 2,Phase 1,Early Phase 1
24 Analgesics Phase 2
25 Immunologic Factors Phase 2,Phase 1,Early Phase 1
26 Antipyretics Phase 2
27 Histamine H1 Antagonists Phase 2
28 Immunoglobulins Phase 2,Phase 1,Early Phase 1
29 Histamine Antagonists Phase 2
30 Pharmaceutical Solutions Phase 2,Phase 1
31 Antiprotozoal Agents Phase 2
32 Antiparasitic Agents Phase 2
33 Anthelmintics Phase 2
34 Reverse Transcriptase Inhibitors Phase 2
35 Anti-HIV Agents Phase 2,Early Phase 1
36 Nucleic Acid Synthesis Inhibitors Phase 2
37 Anti-Retroviral Agents Phase 2,Early Phase 1
38 Antimetabolites Phase 2
39 Hormone Antagonists Phase 2,Phase 1
40 Antineoplastic Agents, Alkylating Phase 2,Phase 1
41 Alkylating Agents Phase 2,Phase 1
42 Antineoplastic Agents, Hormonal Phase 2,Phase 1
43 Anti-Bacterial Agents Phase 2,Phase 1
44 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 2,Phase 1
45 Anti-Inflammatory Agents Phase 2,Phase 1
46 Immunosuppressive Agents Phase 2,Phase 1
47 Antirheumatic Agents Phase 2,Phase 1
48 glucocorticoids Phase 2,Phase 1
49 Angiogenesis Inhibitors Phase 2,Phase 1
50 Angiogenesis Modulating Agents Phase 2,Phase 1

Interventional clinical trials:

(show all 29)
# 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 Bevacizumab and Temsirolimus Alone or in Combination With Valproic Acid or Cetuximab in Treating Patients With Advanced or Metastatic Malignancy or Other Benign Disease Recruiting NCT01552434 Phase 1 Temsirolimus;Valproic Acid
19 Ibrutinib in Treating Relapsed or Refractory B-Cell Non-Hodgkin Lymphoma in Patients With HIV Infection Terminated NCT02109224 Phase 1 Ibrutinib
20 Epithelioid Hemangioma of Bone And Soft Tissue Completed NCT03169595
21 Detecting Anal and Genital Human Papillomavirus Infection and Squamous Intraepithelial Lesions in HIV-Positive Patients Enrolled in AIDS Cancer Clinical Trials Completed NCT00695422
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 Registry for Adults With Plasma Cell Disorders (PCD's) Recruiting NCT03717844
27 Nelfinavir for the Treatment of Gammaherpesvirus-Related Tumors Terminated NCT02080416 Early Phase 1 Nelfinavir
28 Castleman Disease Collaborative Network Biobank Withdrawn NCT02871050
29 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, Prostate

Publications for Unicentric Castleman Disease

Articles related to Unicentric Castleman Disease:

(show all 33)
# 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
Unicentric castleman disease complicated by paraneoplastic bronchiolitis obliterans and pemphigus. ( 30128272 )
2018
4
Hyaline vascular-type unicentric Castleman disease presenting as a subcutaneous nodule in a child. ( 30156317 )
2018
5
Unicentric Castleman disease presenting as a retroperitoneal peripancreatic mass: A report of two cases and review of literature. ( 30228787 )
2018
6
Rare finding in peripheral nerve surgery: an unicentric Castleman disease presenting as median nerve tumour. ( 30397634 )
2018
7
A Case of Unicentric Castleman Disease and Langerhans Cell Histiocytosis: Two Entities in One Lymph Node. ( 30549992 )
2018
8
Is tocilizumab a potential therapeutic option for refractory unicentric Castleman disease? ( 28401573 )
2017
9
Perivesical unicentric Castleman disease initially suspected to be metastatic prostate cancer. ( 27141204 )
2016
10
Unicentric Castleman Disease: An Unusual Cause of An Isolated Neck Mass. ( 27660550 )
2016
11
Complete resection of unicentric Castleman disease in the superior mediastinum: A case report. ( 27318017 )
2016
12
Case Report Unicentric Castleman disease located in the anterior mediastinum misdiagnosed as invasive thymoma: a case report. ( 26125875 )
2015
13
Exuberant cortical thymocyte proliferation mimicking T-lymphoblastic lymphoma within recurrent large inguinal lymph node masses of localized Castleman disease. ( 25953658 )
2015
14
Unicentric Castleman disease: from screening mammography to PET/CT imaging. ( 25286148 )
2014
15
Sonographic findings of localized Castleman disease of the abdomen and pelvis. ( 25346169 )
2014
16
Endosonographic features of unicentric Castleman Disease. ( 26425506 )
2014
17
Intrapulmonary unicentric Castleman disease mimicking peripheral pulmonary malignancy. ( 26767055 )
2014
18
Iron-Refractory Microcytic Anemia as the Presenting Feature of Unicentric Castleman Disease in Children. ( 24367988 )
2013
19
Parapharengeal unicentric castleman disease with nephrotic syndrome. ( 23880810 )
2013
20
Unicentric Castleman disease relapsed after rituximab-CHOP chemotherapy or radiation therapy in an adolescent. ( 22258344 )
2012
21
Unicentric Castleman disease mimicking a pancreatic neoplasm. ( 22524754 )
2012
22
Localized Castleman disease of plasma cell type in the abdomen. ( 22040445 )
2011
23
Treatment of unicentric Castleman disease with neoadjuvant rituximab. ( 21051400 )
2010
24
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
25
Coronary artery pseudotumor: IgG4-related disease versus localized Castleman disease. ( 19134500 )
2009
26
Complete anemia reversal after surgical excision of mesenteric hyaline-vascular unicentric Castleman disease. ( 19865557 )
2009
27
Cervical unicentric Castleman disease presenting as a neck mass: case report and review of the literature. ( 19444782 )
2009
28
Localized Castleman disease in retroperitoneum: newly discovered features by multi-detector helical CT. ( 17619097 )
2008
29
Isolated microcytic anemia disclosing a unicentric Castleman disease: The interleukin-6/hepcidin pathway? ( 18549942 )
2008
30
Unicentric castleman disease is not clearly distinguished from multicentric type: a case report. ( 18765316 )
2008
31
Outcome and late complications of radiotherapy in patients with unicentric Castleman disease. ( 17118850 )
2006
32
Human herpesvirus 8 and Epstein-Barr virus coinfection in localized Castleman disease during pregnancy. ( 16266926 )
2005
33
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

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
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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