MCID: CST005
MIFTS: 54

Castleman Disease

Categories: Immune diseases, Rare diseases

Aliases & Classifications for Castleman Disease

MalaCards integrated aliases for Castleman Disease:

Name: Castleman Disease 12 53 37 55 44 15
Angiofollicular Ganglionic Hyperplasia 53 44 72
Giant Lymph Node Hyperplasia 12 53 17
Angiofollicular Lymph Hyperplasia 12 53
Castleman's Disease 75 15
Angiofollicular Lymph Node Hyperplasia 12
Angiolymphoid Hyperplasia 72
Benign Giant Lymphoma 53
Lymphoid Hamartoma 12

Classifications:



External Ids:

Disease Ontology 12 DOID:0111157
KEGG 37 H01479
NCIt 50 C3056
SNOMED-CT 68 69484003
ICD10 33 D47.Z2
UMLS 72 C0017531 C2931179

Summaries for Castleman Disease

KEGG : 37
Castleman disease is a rare lymphoproliferative disorder with two primary subtypes that vary in presentation and course. Unicentric Castleman disease is localized and carries an excellent prognosis. Multicentric Castleman disease can be associated with HIV and human herpesvirus-8 and is characterized by generalized lymphadenopathy and systemic symptoms, such as fever, fatigue, anorexia, anemia, and cachexia. Castleman disease is unique in that dysregulated secretion of interleukin-6 (IL-6) plays a central pathogenetic role, although the exact events precipitating the oversecretion of IL-6 are unknown. Recently, two antibody-based therapeutics targeting components of the IL-6/IL-6R complex have been approved for the treatment of Castleman disease. Tocilizumab is a monoclonal antibody directed against the IL-6 receptor, and siltuximab is a monoclonal antibody specific for IL-6.

MalaCards based summary : Castleman Disease, also known as angiofollicular ganglionic hyperplasia, is related to multicentric castleman disease and human herpesvirus 8. An important gene associated with Castleman Disease is IL6 (Interleukin 6), and among its related pathways/superpathways are Cytokine-cytokine receptor interaction and JAK-STAT signaling pathway. The drugs Dexamethasone acetate and Dexamethasone have been mentioned in the context of this disorder. Affiliated tissues include lymph node, b cells and bone, and related phenotypes are fatigue and anemia

Disease Ontology : 12 A lymphoproliferative syndrome characterized by one or more enlarged lymph nodes containing cells with hyaline-vascular, plasmacytic, or mixed appearance microscopically.

NIH Rare Diseases : 53 Castleman disease (CD) is a rare condition that affects the lymph nodes and related tissues. There are two main forms: unicentric CD and multicentric CD. Unicentric CD is a "localized" condition that is generally confined to a single set of lymph nodes, while multicentric CD is a "systemic" disease that affects multiple sets of lymph nodes and other tissues throughout the body. The exact underlying cause of CD is currently unknown; however, it is thought to occur sporadically in people with no family history of the condition. Treatment varies based on the form of the condition, the severity of symptoms and whether or not the affected person also has an HIV and/or human herpes virus type 8 (HHV-8) infection. For more specific information about each form of CD, please visit GARD's unicentric Castleman disease and multicentric Castleman disease pages.

Wikipedia : 75 Castleman disease is a group of uncommon lymphoproliferative disorders characterized by lymph node... more...

Related Diseases for Castleman Disease

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

(show top 50) (show all 610)
# Related Disease Score Top Affiliating Genes
1 multicentric castleman disease 33.3 PAX5 IL6 IL10 CRP BCL6
2 human herpesvirus 8 33.1 IL6R IL6 BCL6
3 poems syndrome 32.4 VEGFA IL6
4 primary effusion lymphoma 31.0 VEGFA IL6 BCL6
5 bronchiolitis 30.6 IL6 IL10 CRP
6 pericardial effusion 30.4 VEGFA IL6 CRP
7 hodgkin's lymphoma, lymphocytic-histiocytic predominance 30.4 CR2 BCL6
8 acquired immunodeficiency syndrome 30.3 IL6 IL10 BCL6
9 neutrophilia, hereditary 30.2 IL6 CRP
10 ulcerative colitis 30.2 IL6 IL10 CRP
11 endocarditis 30.2 IL6 IL10 CRP
12 peritonitis 30.1 IL6 IL10 CRP
13 follicular dendritic cell sarcoma 30.0 PPL EVPL DSP CR2 CR1
14 pneumonia 30.0 IL6 IL10 CRP
15 bronchiolitis obliterans 29.9 PPL IL6 IL10 EVPL
16 appendicitis 29.9 IL6 IL10 CRP
17 acute pyelonephritis 29.8 IL6 IL10 CRP
18 pyelonephritis 29.8 IL6 IL10 CRP
19 lymph node disease 29.8 IL6 IL10 CRP
20 myeloma, multiple 29.8 VEGFA IL6R IL6 CRP
21 proteasome-associated autoinflammatory syndrome 1 29.7 IL6 IL10 CRP
22 acute pancreatitis 29.7 IL6 IL10 CRP
23 hemophagocytic lymphohistiocytosis 29.6 IL10 CR2 CR1
24 human immunodeficiency virus type 1 29.6 IL6 IL10 CR2 CR1
25 rheumatoid arthritis 29.1 VEGFA IL6R IL6 IL10 CRP
26 pemphigus 29.1 PPL EVPL DSP DSG3 DSG1
27 paraneoplastic pemphigus 29.1 PPL EVPL DSP DSG3 DSG1
28 systemic lupus erythematosus 28.9 IL6 IL10 CRP CR2 CR1
29 cellulitis 28.7 PPL DSP DSG3 DSG1
30 heart disease 28.6 VEGFA IL6 IL10 DSP CRP
31 skin disease 28.1 IL10 DSP DSG3 DSG1
32 bullous pemphigoid 28.0 PPL EVPL DSP DSG3 DSG1
33 bullous skin disease 27.9 PPL EVPL DSP DSG3 DSG1
34 unicentric castleman disease 12.7
35 pediatric castleman disease 12.4
36 kaposi sarcoma 12.2
37 lymphoproliferative syndrome 10.9
38 cold agglutinin disease 10.7 IL6 CRP
39 parapharyngeal meningioma 10.6 CR2 CR1
40 cryopyrin-associated periodic syndrome 10.6 IL6 CRP
41 liver inflammatory pseudotumor 10.6 CRP CR2
42 acute cholangitis 10.6 IL6 CRP
43 uremic pruritus 10.5 IL6 CRP
44 interdigitating dendritic cell sarcoma 10.5 CR2 CR1
45 langerhans cell sarcoma 10.5 CR2 CR1
46 viral pneumonia 10.5 IL6 CRP CR1
47 splenomegaly 10.5
48 sarcoma 10.5
49 spindle cell sarcoma 10.5
50 preterm premature rupture of the membranes 10.5 IL6R IL6 CRP

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



Diseases related to Castleman Disease

Symptoms & Phenotypes for Castleman Disease

Human phenotypes related to Castleman Disease:

32 (show all 26)
# Description HPO Frequency HPO Source Accession
1 fatigue 32 frequent (33%) HP:0012378
2 anemia 32 frequent (33%) HP:0001903
3 abdominal pain 32 frequent (33%) HP:0002027
4 weight loss 32 frequent (33%) HP:0001824
5 mediastinal lymphadenopathy 32 frequent (33%) HP:0100721
6 elevated c-reactive protein level 32 frequent (33%) HP:0011227
7 elevated erythrocyte sedimentation rate 32 frequent (33%) HP:0003565
8 follicular hyperplasia 32 frequent (33%) HP:0002729
9 increased serum interleukin-6 32 frequent (33%) HP:0030783
10 nausea and vomiting 32 occasional (7.5%) HP:0002017
11 cough 32 occasional (7.5%) HP:0012735
12 jaundice 32 occasional (7.5%) HP:0000952
13 abdominal distention 32 occasional (7.5%) HP:0003270
14 abdominal mass 32 occasional (7.5%) HP:0031500
15 flank pain 32 occasional (7.5%) HP:0030157
16 generalized lymphadenopathy 32 occasional (7.5%) HP:0008940
17 decreased mean corpuscular volume 32 occasional (7.5%) HP:0025066
18 renal insufficiency 32 very rare (1%) HP:0000083
19 dyspnea 32 very rare (1%) HP:0002094
20 hematuria 32 very rare (1%) HP:0000790
21 thrombocytopenia 32 very rare (1%) HP:0001873
22 intestinal obstruction 32 very rare (1%) HP:0005214
23 anasarca 32 very rare (1%) HP:0012050
24 restrictive cardiomyopathy 32 very rare (1%) HP:0001723
25 myelofibrosis 32 very rare (1%) HP:0011974
26 ureteral obstruction 32 very rare (1%) HP:0006000

MGI Mouse Phenotypes related to Castleman Disease:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 digestive/alimentary MP:0005381 9.63 DSG3 DSP IL10 IL6 KRT15 VEGFA
2 immune system MP:0005387 9.61 BCL6 CR2 CRP DSG3 IL10 IL6
3 integument MP:0010771 9.17 DSG1 DSG3 DSP EVPL IL10 IL6

Drugs & Therapeutics for Castleman Disease

Drugs for Castleman Disease (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 65)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 2 1177-87-3
2
Dexamethasone Approved, Investigational, Vet_approved Phase 2 50-02-2 5743
3
Bortezomib Approved, Investigational Phase 2 179324-69-7 93860 387447
4 Siltuximab Approved, Investigational Phase 1, Phase 2 541502-14-1
5
Ganciclovir Approved, Investigational Phase 2 82410-32-0 3454
6
Lenograstim Approved, Investigational Phase 2 135968-09-1
7
Zidovudine Approved Phase 2 30516-87-1 35370
8
Sirolimus Approved, Investigational Phase 2 53123-88-9 6436030 5284616 46835353
9
Everolimus Approved Phase 2 159351-69-6 6442177 70789204
10
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
11
Suramin Investigational Phase 2 145-63-1 5361
12 Anthelmintics Phase 2
13 Antiparasitic Agents Phase 2
14 Antiprotozoal Agents Phase 2
15 Analgesics Phase 2
16 Histamine H1 Antagonists Phase 2
17 Histamine Antagonists Phase 2
18 Antipyretics Phase 2
19 Pharmaceutical Solutions Phase 2
20 Antibodies Phase 1, Phase 2
21 Immunoglobulins Phase 1, Phase 2
22 Antibodies, Monoclonal Phase 1, Phase 2
23 Gastrointestinal Agents Phase 2
24 Antiemetics Phase 2
25 Peripheral Nervous System Agents Phase 2
26 Autonomic Agents Phase 2
27 BB 1101 Phase 2
28 Ganciclovir triphosphate Phase 2
29 Adjuvants, Immunologic Phase 2
30 Interferon-alpha Phase 2
31 Reverse Transcriptase Inhibitors Phase 2
32 Anti-HIV Agents Phase 2
33 Anti-Retroviral Agents Phase 2
34 interferons Phase 2
35 Nucleic Acid Synthesis Inhibitors Phase 2
36 Antimetabolites Phase 2
37 Antifungal Agents Phase 2
38
Cetuximab Approved Phase 1 205923-56-4 56842117 2333
39
Valproic acid Approved, Investigational Phase 1 99-66-1 3121
40
Bevacizumab Approved, Investigational Phase 1 216974-75-3
41
Methotrexate Approved Phase 1 1959-05-2, 59-05-2 126941
42
leucovorin Approved Phase 1 58-05-9 143 6006
43
Daunorubicin Approved Phase 1 20830-81-3 30323
44
Folic acid Approved, Nutraceutical, Vet_approved Phase 1 59-30-3 6037
45 Neurotransmitter Agents Phase 1
46 Immunoglobulin G Phase 1
47 Tranquilizing Agents Phase 1
48 Central Nervous System Depressants Phase 1
49 GABA Agents Phase 1
50 Mitogens Phase 1

Interventional clinical trials:

(show all 37)
# Name Status NCT ID Phase Drugs
1 Clinical and Virologic Response to HHV-8 Associated Multicentric Castleman's Disease to Valganciclovir Withdrawn NCT00361933 Phase 4 Valganciclovir
2 A PHASE II PILOT STUDY OF SURAMIN IN PREVIOUSLY TREATED PATIENTS WITH MULTIPLE MYELOMA AND PATIENTS WITH CASTLEMAN'S DISEASE Completed NCT00002652 Phase 2 suramin
3 A Randomized, Double Blind, Placebo Controlled Study to Assess the Efficacy and Safety of CNTO 328 (Anti IL 6 Monoclonal Antibody) Plus Best Supportive Care Compared With Best Supportive Care in Subjects With Multicentric Castleman's Disease Completed NCT01024036 Phase 2 Siltuximab;Placebo;Best Supportive Care (BSC)
4 An Open-label, Multicenter Study to Evaluate the Safety of Long-term Treatment With Siltuximab in Subjects With Multicentric Castleman's Disease Completed NCT01400503 Phase 2 Siltuximab
5 A Phase II Clinical Trial of 17-(Allylamino)-17- Demethoxygeldanamycin (17-AAG, NSC 330507 and EPL Diluent, NSC 704057) in Adults With Systemic Mastocytosis Completed NCT00132015 Phase 2 tanespimycin
6 Phase IIa of Tocilizumab In the Treatment of Polymyalgia Rheumatica Completed NCT01396317 Phase 2 Tocilizumab
7 Thalidomide, Cyclophosphamide and Prednisone in Newly Diagnosed Multicentric Castleman's Disease: a Prospective, Single-center, Single-arm, Phase-II Pilot Trial Recruiting NCT03043105 Phase 2 Thalidomide, cyclophosphamide and prednisone
8 Bortezomib, Cyclophosphamide and Dexamethasone (BCD) in Newly Diagnosed Idiopathic Multicentric Castleman's Disease (iMCD) : a Prospective, Single-center, Single-arm, Phase-II Pilot Trial Recruiting NCT03982771 Phase 2 Bortezomib;Cyclophosphamide;Dexamethason
9 Targeted Oncolytic Virotherapy and Natural History Study of KSHV-Associated Multicentric Castleman's Disease With Laboratory and Clinical 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
10 Pilot Study of Tocilizumab in Patients With Symptomatic Kaposi Sarcoma Herpesvirus (KSHV) - Associated Multicentric Castleman Disease Recruiting NCT01441063 Phase 2 Zidovudine;Tocilizumab;Valganciclovir (VGC)
11 A Randomized Controlled Trial of Adjunctive Siltuximab in Schizophrenia Recruiting NCT02796859 Phase 1, Phase 2 Siltuximab;normal saline
12 Natural History Study of the KSHV Inflammatory Cytokine Syndrome (KICS) Incorporating Pilot Evaluation of KSHV Targeted Therapies Recruiting NCT01419561 Phase 2 Zidovudine;Liposomal Doxorubicin;Valganiclovir;Rituximab
13 A Phase II, Single-arm Open-label Multi-center Study of Sirolimus in Previously Treated Idiopathic Multicentric Castleman Disease Not yet recruiting NCT03933904 Phase 2 Sirolimus
14 Multicenter, Phase II Trial Assessing the Efficacy of Rituximab in HIV Infected Patients With Multicentric Castleman Disease Dependent on Chemotherapy (ANRS 117 Study, CastlemaB) Terminated NCT00127569 Phase 2 Rituximab
15 Phase I/II Study of Pomalidomide Combined With Modified DA-EPOCH and Rituximab in KSHV-Associated Lymphomas (Primary Effusion Lymphoma and Large Cell Lymphoma Arising in KSHV-Associated Multicentric Castleman Disease) Withdrawn NCT02228512 Phase 1, Phase 2 Pomalidomide;Rituximab;Prednisone;Etoposide;Doxorubicin;Vincristine;Cyclophosphamide
16 A Phase 1, Multi-Center, Open-Label, Dose-Escalation, Safety, Pharmacokinetic, and Pharmacodynamic Study of CX-4945 Administered Orally to Patients With Advanced Solid Tumors, Castleman's Disease or Multiple Myeloma Unknown status NCT00891280 Phase 1 CX-4945 oral formulation
17 Provision of Tocilizumab for Patients With Multicentric Castleman's Disease Who Have Demonstrated Benefit From Previous Tocilizumab Treatment Completed NCT01183598 Phase 1 tocilizumab [RoActemra/Actemra]
18 A Phase 1 Study of Multiple Intravenous Administrations of a Chimeric Antibody Against Interleukin-6 (CNTO 328) in Subjects With B-Cell Non-Hodgkin's Lymphoma, Multiple Myeloma, or Castleman's Disease Completed NCT00412321 Phase 1 CNTO 328
19 A Study of Siltuximab (Anti-IL-6 Monoclonal Antibody) Effects on the QT Interval in Subjects With Monoclonal Gammopathy of Undetermined Significance, Smoldering Multiple Myeloma, or Indolent Multiple Myeloma Completed NCT01219010 Phase 1
20 An Open-Label Trial of Tocilizumab in Schizophrenia Completed NCT01696929 Phase 1 Tocilizumab
21 A Phase 1 Trial of Pomalidomide in Combination With Liposomal Doxorubicin in Patients With Advanced or Refractory Kaposi Sarcoma Recruiting NCT02659930 Phase 1 liposomal doxorubicin;pomalidomide
22 A Phase I Trial of Bevacizumab, Temsirolimus Alone and in Combination With Valproic Acid, or Cetuximab in Patients With Advanced Malignancy and Other Indications Recruiting NCT01552434 Phase 1 Temsirolimus;Valproic Acid
23 A Randomized Controlled Trial of Adjunctive Tocilizumab in Schizophrenia Recruiting NCT02874573 Phase 1 Tocilizumab;Normal saline
24 A Phase I Study of Subcutaneous Rituximab Hyaluronidase Combined With Local Standard-of-Care Chemotherapy for the Treatment of Burkitt Lymphoma, Diffuse Large B-Cell Lymphoma or as Monotherapy for Kaposi Sarcoma Herpesvirus Associated Multicentric Castleman Disease in Pediatrics Children, Adolescents, and Adults in Uganda Not yet recruiting NCT03864419 Phase 1 Cyclophosphamide;Vincristine;Methotrexate;Doxorubicin;Doxorubicin Hydrochloride;Prednisone;Etoposide
25 Phase I and Pharmacokinetic Study of Ibrutinib in HIV-Infected Patients With Relapsed or Refractory B-Cell Non-Hodgkin Lymphoma or Multiple Myeloma Terminated NCT02109224 Phase 1 Ibrutinib
26 Epithelioid Hemangioma of Bone And Soft Tissue: a Benign Tumor With Metastatic Potential Completed NCT03169595
27 UARK 91-004 Therapeutic Research in Multiple Myeloma Completed NCT00572338
28 A Companion Protocol to Evaluate Anogenital Human Papillomavirus (HPV) Infection and Anogenital Squamous Intraepithelial Lesions (ASIL) in Subjects Participating in AMC Clinical Trials Completed NCT00695422
29 KSHV Seroprevalence in Hospital Patients From San Antonio Completed NCT00006310
30 Unlock the Cell: Intracellular Inflammatory Pathways and Flow Cytometry Study for Castleman's Disease Recruiting NCT02853968
31 ACCELERATE (Advancing Castleman Care With an Electronic Longitudinal Registry, E-Repository, And Treatment/Effectiveness Research): An International Registry for Patients With Castleman Disease Recruiting NCT02817997
32 Molecular Characterization of Viral-associated Tumors, Tumors Occurring in the Setting of HIV or Other Immune Disorders and Castleman Disease Recruiting NCT03300830
33 Registry for Adults With Plasma Cell Disorders (PCD's) Recruiting NCT03717844
34 Collection of Blood, Bone Marrow, Tumor, or Tissue Samples From Patients With HIV Infection, KSHV Infection, Viral-Related Pre-Malignant Lesions, and/or Cancer Recruiting NCT00006518
35 A Pilot Trial of Nelfinavir for the Lytic Activation and Treatment of Gammaherpesvirus-Related Tumors Terminated NCT02080416 Early Phase 1 Nelfinavir
36 The Castleman Disease Collaborative Network Biobank: A Collection of Biospecimens and Clinical Data to Facilitate Research Withdrawn NCT02871050
37 A Single-arm, Single Center, Pilot Study of Siltuximab, an Anti-IL6 Therapy, for Patients With Myelofibrosis Withdrawn NCT02805868 Early Phase 1

Search NIH Clinical Center for Castleman Disease

Inferred drug relations via UMLS 72 / NDF-RT 51 :


siltuximab
Siltuximab

Cochrane evidence based reviews: castleman disease

Genetic Tests for Castleman Disease

Anatomical Context for Castleman Disease

MalaCards organs/tissues related to Castleman Disease:

41
Lymph Node, B Cells, Bone, Bone Marrow, T Cells, Endothelial, Liver

Publications for Castleman Disease

Articles related to Castleman Disease:

(show top 50) (show all 1210)
# Title Authors PMID Year
1
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. 9 38
19730402 2009
2
Mechanisms and pathologic significances in increase in serum interleukin-6 (IL-6) and soluble IL-6 receptor after administration of an anti-IL-6 receptor antibody, tocilizumab, in patients with rheumatoid arthritis and Castleman disease. 9 38
18784373 2008
3
Reversible cardiomyopathy associated with Multicentric Castleman disease: successful treatment with tocilizumab, an anti-interleukin 6 receptor antibody. 9 38
17483056 2007
4
Bone marrow findings in multicentric Castleman disease in HIV-negative patients. 9 38
17325481 2007
5
Interleukin 6: from bench to bedside. 9 38
17075601 2006
6
[A case of HIV-1 and HHV-8-associated Castleman disease with a relapsing high fever and lymphoadenopathy]. 9 38
16922487 2006
7
Autoantibody production from a thymoma and a follicular dendritic cell sarcoma associated with paraneoplastic pemphigus. 9 38
16120143 2005
8
Intraventricular chordoid meningioma presenting with Castleman disease due to overproduction of interleukin-6. Case report. 9 38
15871519 2005
9
Acute myelogenous leukemia M5b developed during clinical remission of Castleman disease. 9 38
12731671 2003
10
High incidence of Kaposi sarcoma-associated herpesvirus-related non-Hodgkin lymphoma in patients with HIV infection and multicentric Castleman disease. 9 38
11895764 2002
11
The Kaposi sarcoma-associated herpesvirus (KSHV) induces cellular interleukin 6 expression: role of the KSHV latency-associated nuclear antigen and the AP1 response element. 9 38
11781250 2002
12
High levels of human herpesvirus 8 viral load, human interleukin-6, interleukin-10, and C reactive protein correlate with exacerbation of multicentric castleman disease in HIV-infected patients. 9 38
10979949 2000
13
[Advances in interleukin-6 therapy]. 9 38
10340005 1999
14
Castleman disease in POEMS syndrome with elevated interleukin-6. 9 38
8499003 1993
15
Castleman Disease. 38
31352991 2019
16
Ultrasonographic Findings of 1385 Adrenal Masses: A Retrospective Study of 1319 Benign and 66 Malignant Masses. 38
29194699 2019
17
Identifying and targeting pathogenic PI3K/AKT/mTOR signaling in IL-6-blockade-refractory idiopathic multicentric Castleman disease. 38
31408438 2019
18
Tocilizumab Cannot Prevent the Development of Bronchiolitis Obliterans in Patients With Castleman Disease-Associated Paraneoplastic Pemphigus. 38
29389687 2019
19
Epidemiology of Castleman disease associated with AA amyloidosis: description of 2 new cases and literature review. 38
31364863 2019
20
POEMS Syndrome: 2019 Update on diagnosis, risk-stratification, and management. 38
31012139 2019
21
Quantitative analysis of a rare disease network's international contact database and E-repository provides insights into biobanking in the electronic consent era. 38
31296233 2019
22
Imaging and clinical features of Castleman Disease. 38
31345268 2019
23
Unicentric Castleman disease in the lesser omentum: A case report. 38
31350088 2019
24
The potential role of follicular helper T cells in idiopathic multicentric Castleman disease with and without TAFRO syndrome. 38
31358479 2019
25
Clinical and pathological characteristics of Castleman disease: an observational study in a Spanish tertiary hospital. 38
31305183 2019
26
Successful treatment of a child with idiopathic multicentric Castleman disease associated with hemophagocytic lymphohistiocytosis using tocilizumab. 38
30989798 2019
27
Treatment and outcome of Unicentric Castleman Disease: a retrospective analysis of 71 cases. 38
31016730 2019
28
A Case of Castleman Disease With Recurrence in the Neck: Case Report and Literature Review. 38
30825440 2019
29
Unicentric Castleman Disease Mimicking an Autoinflammatory Disorder: A Diagnostic Challenge in a Pediatric Patient With Recurrent Fever. 38
31335823 2019
30
The Contribution of Kaposi's Sarcoma-Associated Herpesvirus to Mortality in Hospitalized Human Immunodeficiency Virus-Infected Patients Being Investigated for Tuberculosis in South Africa. 38
31004430 2019
31
Remission of an HHV8-related extracavitary primary effusion lymphoma in an HIV-positive patient during antiretroviral treatment containing dolutegravir. 38
31351487 2019
32
Calcified or ossified benign soft tissue lesions that may simulate malignancy. 38
31297550 2019
33
Cutaneous Disorders Associated with Castleman's Disease. 38
31282978 2019
34
Idiopathic Multicentric Castleman Disease with Autoimmune Hemolytic Anemia and Production of Anti-drug Antibody Against Tocilizumab: A Case Report. 38
31292389 2019
35
Risk factors for death and survival in paraneoplastic pemphigus associated with hematologic malignancies in adults. 38
30981429 2019
36
miRNAs and their roles in KSHV pathogenesis. 38
30951791 2019
37
Off-Label Use of Sirolimus and Everolimus in a Pediatric Center: A Case Series and Review of the Literature. 38
31124053 2019
38
Is TAFRO syndrome a subtype of idiopathic multicentric Castleman disease? 38
31222819 2019
39
A Case of Osteosarcoma Manifesting Systemic Inflammation and Histological Features Mimicking Plasma Cell-type Castleman Disease. 38
31178507 2019
40
Castleman Disease Presenting with Pseudotumour Cerebri and Myasthenia Gravis: A Case Report and Literature Review. 38
31312243 2019
41
Hyaline vascular variant of unicentric Castleman disease of the tonsil: a case report. 38
31253137 2019
42
A Comparison of the Clinical Characteristics of TAFRO Syndrome and Idiopathic Multicentric Castleman Disease in General Internal Medicine: A 6-Year Retrospective Study. 38
31211492 2019
43
Castleman disease in pediatrics: Insights on presentation, treatment, and outcomes from a two-site retrospective cohort study. 38
30680887 2019
44
A prospective description of HIV-associated multicentric Castleman disease in Malawi. 38
30442726 2019
45
Adenopathy and extensive skin patch overlying a plasmacytoma with unusual histologic findings in a patient with polyneuropathy, organomegaly, endocrinopathy, monoclonal protein and skin changes syndrome and Castleman disease. 38
31119772 2019
46
Rheumatoid arthritis-like active synovitis with T-cell activation in a case of idiopathic multicentric Castleman disease: A case report. 38
31045763 2019
47
Kaposi's sarcoma-associated herpesvirus vIRF2 protein utilizes an IFN-dependent pathway to regulate viral early gene expression. 38
31059555 2019
48
Viral, immunologic, and clinical features of primary effusion lymphoma. 38
30782610 2019
49
Diagnostic framing of IgM monoclonal gammopathy: Focus on Waldenström macroglobulinemia. 38
30192023 2019
50
Tufted-angioma-like lesion associated with vascular endothelial growth factor and interleukin-6 in TAFRO syndrome: Is it a common histological feature of multicentric Castleman disease/POEMS syndrome? 38
30632181 2019

Variations for Castleman Disease

Expression for Castleman Disease

Search GEO for disease gene expression data for Castleman Disease.

Pathways for Castleman Disease

Pathways related to Castleman Disease according to KEGG:

37
# Name Kegg Source Accession
1 Cytokine-cytokine receptor interaction hsa04060
2 JAK-STAT signaling pathway hsa04630
3 Hematopoietic cell lineage hsa04640

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

(show all 19)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.75 VEGFA PPL IL6R IL6 IL10 DSP
2
Show member pathways
13.23 VEGFA PPL KRT15 EVPL DSP DSG3
3
Show member pathways
12.09 PPL KRT15 EVPL DSP DSG3 DSG1
4
Show member pathways
11.9 IL6 IL10 BCL6
5
Show member pathways
11.88 CRP CR2 CR1
6
Show member pathways
11.85 IL6R IL6 IL10
7 11.83 VEGFA IL6R IL6 IL10
8
Show member pathways
11.78 DSP DSG3 DSG1
9
Show member pathways
11.73 PPL EVPL DSP
10 11.72 VEGFA IL6R IL6
11 11.67 IL6R IL6 IL10
12
Show member pathways
11.61 IL6R IL6 CRP
13 11.54 IL6R IL6 CR2 CR1
14 11.41 VEGFA IL6R IL6 IL10 BCL6
15 11.32 VEGFA IL6R IL6
16 11.27 IL6 IL10 CR1
17 11.23 VEGFA IL6R IL6
18 10.85 IL6 CRP
19 10.75 PAX5 IL6R IL10 CR2 BCL6

GO Terms for Castleman Disease

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

# Name GO ID Score Top Affiliating Genes
1 cell junction GO:0030054 9.77 PPL EVPL DSP DSG3 DSG1
2 intermediate filament GO:0005882 9.56 PPL KRT15 EVPL DSP
3 ficolin-1-rich granule membrane GO:0101003 9.5 DSP DSG1 CR1
4 cornified envelope GO:0001533 9.35 PPL EVPL DSP DSG3 DSG1
5 interleukin-6 receptor complex GO:0005896 9.16 IL6R IL6
6 desmosome GO:0030057 9.02 PPL EVPL DSP DSG3 DSG1

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

(show all 19)
# Name GO ID Score Top Affiliating Genes
1 inflammatory response GO:0006954 9.88 IL6 IL10 CRP BCL6
2 epidermis development GO:0008544 9.73 KRT15 EVPL DSP
3 wound healing GO:0042060 9.71 PPL EVPL DSP
4 positive regulation of peptidyl-tyrosine phosphorylation GO:0050731 9.69 VEGFA IL6R IL6
5 cytokine-mediated signaling pathway GO:0019221 9.65 VEGFA IL6R IL6 IL10 BCL6
6 B cell differentiation GO:0030183 9.63 IL10 CR2 BCL6
7 positive regulation of tyrosine phosphorylation of STAT protein GO:0042531 9.61 VEGFA IL6R IL6
8 positive regulation of chemokine production GO:0032722 9.58 IL6R IL6
9 interleukin-6-mediated signaling pathway GO:0070102 9.57 IL6R IL6
10 complement receptor mediated signaling pathway GO:0002430 9.55 CR2 CR1
11 acute-phase response GO:0006953 9.54 IL6R IL6 CRP
12 positive regulation of leukocyte chemotaxis GO:0002690 9.52 IL6R IL6
13 positive regulation of cellular component movement GO:0051272 9.49 VEGFA BCL6
14 negative regulation of lipid storage GO:0010888 9.48 IL6 CRP
15 keratinization GO:0031424 9.43 PPL KRT15 EVPL DSP DSG3 DSG1
16 type 2 immune response GO:0042092 9.4 IL10 BCL6
17 intermediate filament cytoskeleton organization GO:0045104 9.33 PPL EVPL DSP
18 hepatic immune response GO:0002384 9.32 IL6R IL6
19 cornification GO:0070268 9.1 PPL KRT15 EVPL DSP DSG3 DSG1

Molecular functions related to Castleman Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 structural constituent of cytoskeleton GO:0005200 9.33 PPL KRT15 DSP
2 interleukin-6 receptor binding GO:0005138 8.96 IL6R IL6
3 growth factor activity GO:0008083 8.92 VEGFA IL6R IL6 IL10

Sources for Castleman Disease

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 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 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
Content
Loading form....