Castleman disease

Castleman disease (CD) is a benign lymphoproliferative disorder that may present as a localized or multicentric form (see these terms). The clinical manifestations are heterogeneous, ranging from asymptomatic discrete lymphadenopathy to recurrent episodes of diffuse lymphadenopathy with severe systemic symptoms.

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Narrow down the case reports

Total: 220 (papers)

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Matched Phenotype Gene Mutation MeSH

Rank (Similarity)	PMID (PMCID)
21 (43.8%)	26490523	Multicentric Castleman Disease with Monoclonal Incomplete IgH Restriction: A Rare Coexistence. Goyal G, Kendric K, Silberstein PT, Caponetti GC, Vivekanandan R. J Clin Exp Hematop. 2015;55(2):103-8. [ Show abstract ] [ Hide abstract ]
		Anemia Fever Lymphadenopathy
		IGH

		Females Gene Rearrangement HIV Seropositivity Homo sapiens Immunoglobulin Heavy Chains Lymphatic Diseases Middle Aged
21 (43.8%)	20194090	Multicentric Castleman disease: report of rare disease in Kuwait. Mohammad BA, Al-Brahim N, Kunjhibava S, Ahmad M, Ahmad M. Gulf J Oncolog. 2009;(6):48-50. [ Show abstract ] [ Hide abstract ]
		Anemia Fever Follicular hyperplasia


		Homo sapiens Male Middle Aged
21 (43.8%)	1542967	[Angiofollicular lymph node hyperplasia (Castleman disease)]. Pedersen LM, Nielsen H. Ugeskr Laeger. 1992;154(10):621-4. [ Show abstract ] [ Hide abstract ]
		Anemia Fever Lymphadenopathy


		Differential Diagnosis Homo sapiens Male Middle Aged
24 (40.3%)	17616028 (2687612)	Castleman disease presenting with jaundice: a case with the multicentric hyaline vascular variant. Park JB, Hwang JH, Kim H, Choe HS, Kim YK, Kim HB, Bang SM. Korean J Intern Med. 2007;22(2):113-7. [ Show abstract ] [ Hide abstract ]
		Jaundice Lymphadenopathy Lymphoproliferative disorder


		Abdominal Pain Adrenal Cortex Hormones Biopsy Homo sapiens Icterus Male Middle Aged Positron-Emission Tomography Vascular Diseases X-Ray Computed Tomography
25 (39.6%)	31253137	Hyaline vascular variant of unicentric Castleman disease of the tonsil: a case report. Li P, Liu H, Li H, Li A, Yu G, Yin W. Diagn Pathol. 2019;14(1):70. [ Show abstract ] [ Hide abstract ]
		Plethora Follicular hyperplasia Lymphoproliferative disorder


		Adult Differential Diagnosis Homo sapiens Hyalin Substance Immunohistochemistry Lymphocyte Lymphoid Tissue Lymphoproliferative Disorders Male Palatine Tonsil
26 (38.8%)	30009125 (6038051)	Successful rituximab treatment of TAFRO syndrome with pathological findings of glomerular endothelial damage. Noda Y, Saka Y, Kato A, Mimura T, Naruse T. Clin Nephrol Case Stud. 2018;6:16-20. [ Show abstract ] [ Hide abstract ]
		Edema Ascites Thrombocytopenia



26 (38.8%)	28367597	Salvage therapy with lenalidomide containing regimen for relapsed/refractory Castleman disease: a report of three cases. Zhou X, Wei J, Lou Y, Xu G, Yang M, Liu H, Mao L, Tong H, Jin J. Front Med. 2017;11(2):287-292. [ Show abstract ] [ Hide abstract ]
		Ascites Pleural effusion Lymphoproliferative disorder


		Adult Combined Modality Therapy Homo sapiens Male Middle Aged Remission Induction Salvage Therapy Transplantation, Autologous Young Adult
28 (37.6%)	30235674 (6160051)	Castleman disease mimicking systemic lupus erythematosus: A case report. Wang L, Chen H, Shi J, Tang H, Li H, Zheng W, Zhang F. Medicine (Baltimore). 2018;97(38):e12291. [ Show abstract ] [ Hide abstract ]
		Proteinuria Glomerulonephritis Anemia
		DDIT3

		Anemia Differential Diagnosis Fever Glomerulonephritis, Membranoproliferative Homo sapiens Lupus Erythematosus, Systemic Male Middle Aged Nephrotic Syndrome Serositis Thrombocytopenia
28 (37.6%)	26387507	Anemia and autoimmunity markers in an adolescent with Castleman disease. Fretzayas A, Stasinopoulou A, Moustaki M, Stefos E, Kaniouras G, Nicolaidou P. Pediatr Int. 2015;57(6):1199-201. [ Show abstract ] [ Hide abstract ]
		Anemia Autoimmunity Low-grade fever
		IL6

		Anemia Biological Markers Differential Diagnosis Females Homo sapiens Magnetic Resonance Imaging Positron-Emission Tomography X-Ray Computed Tomography
30 (37.6%)	24367988	Iron-refractory microcytic anemia as the presenting feature of unicentric Castleman disease in children. Chandrakasan S, Bakeer N, Mo JQ, Cost C, Quinn CT. J Pediatr. 2014;164(4):928-30. [ Show abstract ] [ Hide abstract ]
		Anemia Lymphadenopathy


		Child Females Homo sapiens

Phenotype(s) retrieved from Orphanet

Total: 29

HPO ID	Term	Frequency
HP:0002716	Lymphadenopathy	Very frequent (99-80%)
HP:0001824	Weight loss	Frequent (79-30%)
HP:0001903	Anemia	Frequent (79-30%)
HP:0002027	Abdominal pain	Frequent (79-30%)
HP:0002729	Follicular hyperplasia	Frequent (79-30%)
HP:0003565	Elevated erythrocyte sedimentation rate	Frequent (79-30%)
HP:0011227	Elevated C-reactive protein level	Frequent (79-30%)
HP:0012378	Fatigue	Frequent (79-30%)
HP:0025142	Constitutional symptom	Frequent (79-30%)
HP:0030783	Increased serum interleukin-6	Frequent (79-30%)
HP:0100721	Mediastinal lymphadenopathy	Frequent (79-30%)
HP:0000952	Jaundice	Occasional (29-5%)
HP:0002017	Nausea and vomiting	Occasional (29-5%)
HP:0003270	Abdominal distention	Occasional (29-5%)
HP:0008940	Generalized lymphadenopathy	Occasional (29-5%)
HP:0011024	Abnormality of the gastrointestinal tract	Occasional (29-5%)
HP:0012735	Cough	Occasional (29-5%)
HP:0025066	Decreased mean corpuscular volume	Occasional (29-5%)
HP:0030157	Flank pain	Occasional (29-5%)
HP:0031500	Abdominal mass	Occasional (29-5%)
HP:0000083	Renal insufficiency	Very rare (4-1%)
HP:0000790	Hematuria	Very rare (4-1%)
HP:0001723	Restrictive cardiomyopathy	Very rare (4-1%)
HP:0001873	Thrombocytopenia	Very rare (4-1%)
HP:0002094	Dyspnea	Very rare (4-1%)
HP:0005214	Intestinal obstruction	Very rare (4-1%)
HP:0006000	Ureteral obstruction	Very rare (4-1%)
HP:0011974	Myelofibrosis	Very rare (4-1%)
HP:0012050	Anasarca	Very rare (4-1%)

Phenotype(s) retrieved from case reports

Total: 63

HPO ID	Term	# of case reports
HP:0005523	Lymphoproliferative disorder	36
HP:0002716	Lymphadenopathy	8
HP:0001271	Polyneuropathy	7
HP:0030731	Carcinoma	7
HP:0002664	Neoplasm	5
HP:0001945	Fever	4
HP:0002721	Immunodeficiency	4
HP:0030150	Plasmacytosis	4
HP:0000969	Edema	3
HP:0001744	Splenomegaly	3
HP:0001903	Anemia	3
HP:0031501	Pelvic mass	3
HP:0100242	Sarcoma	3
HP:0001028	Hemangioma	2
HP:0001433	Hepatosplenomegaly	2
HP:0001891	Iron deficiency anemia	2
HP:0002729	Follicular hyperplasia	2
HP:0008940	Generalized lymphadenopathy	2
HP:0011974	Myelofibrosis	2
HP:0031500	Abdominal mass	2
HP:0000100	Nephrotic syndrome	1
HP:0000112	Nephropathy	1
HP:0000135	Hypogonadism	1
HP:0000572	Visual loss	1
HP:0000790	Hematuria	1
HP:0000819	Diabetes mellitus	1
HP:0000821	Hypothyroidism	1
HP:0000846	Adrenal insufficiency	1
HP:0000952	Jaundice	1
HP:0001138	Optic neuropathy	1
HP:0001370	Rheumatoid arthritis	1
HP:0001541	Ascites	1
HP:0001873	Thrombocytopenia	1
HP:0001894	Thrombocytosis	1
HP:0001909	Leukemia	1
HP:0001935	Microcytic anemia	1
HP:0001955	Unexplained fevers	1
HP:0002024	Malabsorption	1
HP:0002027	Abdominal pain	1
HP:0002094	Dyspnea	1
HP:0002617	Dilatation	1
HP:0002668	Paraganglioma	1
HP:0002835	Aspiration	1
HP:0002960	Autoimmunity	1
HP:0003072	Hypercalcemia	1
HP:0003073	Hypoalbuminemia	1
HP:0003418	Back pain	1
HP:0005200	Retroperitoneal fibrosis	1
HP:0005521	Disseminated intravascular coagulation	1
HP:0009830	Peripheral neuropathy	1
HP:0010280	Stomatitis	1
HP:0011134	Low-grade fever	1
HP:0011506	Choroidal neovascularization	1
HP:0011510	Drusen	1
HP:0011946	Bronchiolitis obliterans	1
HP:0012050	Anasarca	1
HP:0012432	Chronic fatigue	1
HP:0012593	Nephrotic range proteinuria	1
HP:0025289	Cervical lymphadenopathy	1
HP:0032046	Focal cortical dysplasia	1
HP:0100008	Schwannoma	1
HP:0100632	Pulmonary sequestration	1
HP:0100726	Kaposi's sarcoma	1

Causative gene(s) retrieved from Orphanet

Total: 0

Gene Symbol	Gene Name	Entrez Gene ID