MCID: UNC014
MIFTS: 32

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 54
Localized Castleman Disease 54 60
Unicentric Angiofollicular Ganglionic Hyperplasia 54
Localized Angiofollicular Lymphoid Hyperplasia 74
Unicentric Angiofollicular Lymph Hyperplasia 54
Angiolymphoid Hyperplasia 74

Classifications:



External Ids:

ICD10 via Orphanet 35 D36.0
Orphanet 60 ORPHA93685

Summaries for Unicentric Castleman Disease

NIH Rare Diseases : 54 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 Valganciclovir and Anti-Infective Agents have been mentioned in the context of this disorder. Affiliated tissues include lymph node, b cells and endothelial.

Related Diseases for Unicentric Castleman Disease

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

(show all 23)
# Related Disease Score Top Affiliating Genes
1 castleman disease 11.8
2 multicentric castleman disease 11.4
3 microcytic anemia 10.3
4 prostate cancer 10.1
5 thymoma, familial 10.1
6 prostate cancer, hereditary, 8 10.1
7 langerhans cell histiocytosis 10.1
8 prostate cancer, hereditary, 6 10.1
9 bronchiolitis obliterans 10.1
10 hepatocellular adenoma 10.1
11 nephrotic syndrome 10.1
12 bronchiolitis 10.1
13 thymoma 10.1
14 histiocytosis 10.1
15 adenoma 10.1
16 pemphigus 10.1
17 human herpesvirus 8 9.9
18 lymphoblastic lymphoma 9.9
19 lymphoma 9.9
20 igg4-related disease 9.9
21 cryopyrin-associated periodic syndrome 9.7 CRP SAA4
22 amyloidosis aa 9.6 CRP SAA4
23 familial mediterranean fever 9.5 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 89)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Valganciclovir Approved, Investigational Phase 4,Phase 2 175865-60-8 64147
2 Anti-Infective Agents Phase 4,Phase 2,Phase 1,Early Phase 1
3 Antiviral Agents Phase 4,Phase 2,Early Phase 1
4 Siltuximab Approved, Investigational Phase 2,Phase 1,Early Phase 1 541502-14-1
5
Zidovudine Approved Phase 2 30516-87-1 35370
6
Prednisone Approved, Vet_approved Phase 2,Phase 1 53-03-2 5865
7
Cyclophosphamide Approved, Investigational Phase 2,Phase 1 50-18-0, 6055-19-2 2907
8
Lenograstim Approved, Investigational Phase 2 135968-09-1
9
Ganciclovir Approved, Investigational Phase 2 82410-32-0 3454
10
Bortezomib Approved, Investigational Phase 2 179324-69-7 387447 93860
11
Vincristine Approved, Investigational Phase 2,Phase 1 2068-78-2, 57-22-7 5978
12
Miconazole Approved, Investigational, Vet_approved Phase 2,Phase 1 22916-47-8 4189
13
Etoposide Approved Phase 2,Phase 1 33419-42-0 36462
14
Doxorubicin Approved, Investigational Phase 2,Phase 1 23214-92-8 31703
15
Sirolimus Approved, Investigational Phase 2,Phase 1 53123-88-9 46835353 5284616 6436030
16
rituximab Approved Phase 2,Phase 1 174722-31-7 10201696
17
Everolimus Approved Phase 2,Phase 1 159351-69-6 70789204 6442177
18
Pomalidomide Approved Phase 1, Phase 2 19171-19-8
19
Suramin Investigational Phase 2 145-63-1 5361
20
Doxil Approved June 1999 Phase 2,Phase 1 31703
21 Histamine Antagonists Phase 2
22 Analgesics Phase 2
23 Histamine H1 Antagonists Phase 2
24 Immunoglobulins Phase 2,Phase 1,Early Phase 1
25 Immunologic Factors Phase 2,Phase 1,Early Phase 1
26 Antipyretics Phase 2
27 Antibodies Phase 2,Phase 1,Early Phase 1
28 Antibodies, Monoclonal Phase 2,Phase 1,Early Phase 1
29 Pharmaceutical Solutions Phase 2,Phase 1
30 Antiprotozoal Agents Phase 2
31 Antiparasitic Agents Phase 2
32 Anthelmintics Phase 2
33 Antimetabolites Phase 2,Phase 1
34 Anti-Retroviral Agents Phase 2,Early Phase 1
35 Anti-HIV Agents Phase 2,Early Phase 1
36 Reverse Transcriptase Inhibitors Phase 2
37 Nucleic Acid Synthesis Inhibitors Phase 2,Phase 1
38 Hormone Antagonists Phase 2,Phase 1
39 Antineoplastic Agents, Hormonal Phase 2,Phase 1
40 Hormones Phase 2,Phase 1
41 glucocorticoids Phase 2,Phase 1
42 Angiogenesis Inhibitors Phase 2,Phase 1
43 Alkylating Agents Phase 2,Phase 1
44 Antirheumatic Agents Phase 2,Phase 1
45 Immunosuppressive Agents Phase 2,Phase 1
46 Anti-Inflammatory Agents Phase 2,Phase 1
47 Angiogenesis Modulating Agents Phase 2,Phase 1
48 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 2,Phase 1
49 Anti-Bacterial Agents Phase 2,Phase 1
50 Antineoplastic Agents, Alkylating Phase 2,Phase 1

Interventional clinical trials:

(show all 31)
# 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 Sirolimus in Previously Treated Idiopathic Multicentric Castleman Disease Not yet recruiting NCT03933904 Phase 2 Sirolimus
11 Rituximab in the Treatment of HIV Associated Multicentric Castleman Disease Dependent on Chemotherapy Terminated NCT00127569 Phase 2 Rituximab
12 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
13 Dose-escalation Study of Oral CX-4945 Unknown status NCT00891280 Phase 1 CX-4945 oral formulation
14 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]
15 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
16 An Open-Label Trial of Tocilizumab in Schizophrenia Completed NCT01696929 Phase 1 Tocilizumab
17 Tocilizumab in Schizophrenia Recruiting NCT02874573 Phase 1 Tocilizumab;Normal saline
18 Pomalidomide in Combination With Liposomal Doxorubicin in People With Advanced or Refractory Kaposi Sarcoma Recruiting NCT02659930 Phase 1 liposomal doxorubicin;pomalidomide
19 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
20 Rituximab Hyaluronidase and Combination Chemotherapy in Treating Aggressive B-cell Lymphoma in Uganda Not yet recruiting NCT03864419 Phase 1 Cyclophosphamide;Vincristine;Methotrexate;Doxorubicin;Doxorubicin Hydrochloride;Prednisone;Etoposide
21 Ibrutinib in Treating Relapsed or Refractory B-Cell Non-Hodgkin Lymphoma in Patients With HIV Infection Terminated NCT02109224 Phase 1 Ibrutinib
22 Epithelioid Hemangioma of Bone And Soft Tissue Completed NCT03169595
23 Detecting Anal and Genital Human Papillomavirus Infection and Squamous Intraepithelial Lesions in HIV-Positive Patients Enrolled in AIDS Cancer Clinical Trials Completed NCT00695422
24 International Registry for Patients With Castleman Disease Recruiting NCT02817997
25 Unlock the Cell: Castleman's Disease Flow Cytometry Study Recruiting NCT02853968
26 Molecular Characterization of Viral-associated Tumors, Tumors Occurring in the Setting of HIV or Other Immune Disorders and Castleman Disease Recruiting NCT03300830
27 Specimen Collections From Patients With HIV Infection, KSHV Infection, Viral-Related Pre-malignant Lesions and Cancer Recruiting NCT00006518
28 Registry for Adults With Plasma Cell Disorders (PCD's) Recruiting NCT03717844
29 Nelfinavir for the Treatment of Gammaherpesvirus-Related Tumors Terminated NCT02080416 Early Phase 1 Nelfinavir
30 Castleman Disease Collaborative Network Biobank Withdrawn NCT02871050
31 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 74 / NDF-RT 52 :


Genetic Tests for Unicentric Castleman Disease

Anatomical Context for Unicentric Castleman Disease

MalaCards organs/tissues related to Unicentric Castleman Disease:

42
Lymph Node, B Cells, Endothelial

Publications for Unicentric Castleman Disease

Articles related to Unicentric Castleman Disease:

(show all 37)
# Title Authors Year
1
Hyaline vascular-type unicentric Castleman disease presenting as a subcutaneous nodule in a child. ( 30156317 )
2019
2
Radiologic and Histopathologic Features in an Intracranial Localized Castleman Disease: A Case Report and Review of Literature. ( 30586033 )
2019
3
Recurrent PDGFRB mutations in unicentric Castleman disease. ( 30607019 )
2019
4
Treatment and outcome of Unicentric Castleman Disease: a retrospective analysis of 71 cases. ( 31016730 )
2019
5
Synchronous Unicentric Castleman Disease and Inflammatory Hepatocellular Adenoma: a Case Report. ( 30596615 )
2018
6
Unicentric castleman disease complicated by paraneoplastic bronchiolitis obliterans and pemphigus. ( 30128272 )
2018
7
Is tocilizumab a potential therapeutic option for refractory unicentric Castleman disease? ( 28401573 )
2018
8
Unicentric Castleman Disease. ( 29157620 )
2018
9
Next-generation sequencing of idiopathic multicentric and unicentric Castleman disease and follicular dendritic cell sarcomas. ( 29496669 )
2018
10
Unicentric Castleman disease presenting as a retroperitoneal peripancreatic mass: A report of two cases and review of literature. ( 30228787 )
2018
11
Rare finding in peripheral nerve surgery: an unicentric Castleman disease presenting as median nerve tumour. ( 30397634 )
2018
12
A Case of Unicentric Castleman Disease and Langerhans Cell Histiocytosis: Two Entities in One Lymph Node. ( 30549992 )
2018
13
Perivesical unicentric Castleman disease initially suspected to be metastatic prostate cancer. ( 27141204 )
2016
14
Complete resection of unicentric Castleman disease in the superior mediastinum: A case report. ( 27318017 )
2016
15
Unicentric Castleman Disease: An Unusual Cause of An Isolated Neck Mass. ( 27660550 )
2016
16
Sonographic findings of localized Castleman disease of the abdomen and pelvis. ( 25346169 )
2015
17
Exuberant cortical thymocyte proliferation mimicking T-lymphoblastic lymphoma within recurrent large inguinal lymph node masses of localized Castleman disease. ( 25953658 )
2015
18
Case Report Unicentric Castleman disease located in the anterior mediastinum misdiagnosed as invasive thymoma: a case report. ( 26125875 )
2015
19
Unicentric Castleman disease: from screening mammography to PET/CT imaging. ( 25286148 )
2014
20
Intrapulmonary unicentric Castleman disease mimicking peripheral pulmonary malignancy. ( 26767055 )
2014
21
Iron-refractory microcytic anemia as the presenting feature of unicentric Castleman disease in children. ( 24367988 )
2014
22
Endosonographic features of unicentric Castleman Disease. ( 26425506 )
2014
23
Parapharengeal unicentric Castleman disease with nephrotic syndrome. ( 23880810 )
2013
24
Unicentric Castleman disease relapsed after rituximab-CHOP chemotherapy or radiation therapy in an adolescent. ( 22258344 )
2012
25
Unicentric Castleman disease mimicking a pancreatic neoplasm. ( 22524754 )
2012
26
Localized Castleman disease of plasma cell type in the abdomen. ( 22040445 )
2011
27
Treatment of unicentric Castleman disease with neoadjuvant rituximab. ( 21051400 )
2010
28
Coronary artery pseudotumor: IgG4-related disease versus localized Castleman disease. ( 19134500 )
2009
29
Cervical unicentric Castleman disease presenting as a neck mass: case report and review of the literature. ( 19444782 )
2009
30
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
31
Complete anemia reversal after surgical excision of mesenteric hyaline-vascular unicentric Castleman disease. ( 19865557 )
2009
32
Isolated microcytic anemia disclosing a unicentric Castleman disease: The interleukin-6/hepcidin pathway? ( 18549942 )
2008
33
Unicentric castleman disease is not clearly distinguished from multicentric type: a case report. ( 18765316 )
2008
34
Localized Castleman disease in retroperitoneum: newly discovered features by multi-detector helical CT. ( 17619097 )
2008
35
Outcome and late complications of radiotherapy in patients with unicentric Castleman disease. ( 17118850 )
2006
36
Human herpesvirus 8 and Epstein-Barr virus coinfection in localized Castleman disease during pregnancy. ( 16266926 )
2005
37
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 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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