Castleman disease
Synonyms: Angiofollicular ganglionic hyperplasia · Angiofollicular lymph hyperplasia
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
A rare lymphoid hemopathy characterized by involvement of lymph nodes in any part of the body, most frequently the mediastinum, abdomen, neck, or spleen, and occurring as unicentric, idiopathic multicentric, or KSHV/HHV8-associated multicentric Castleman disease. Depending on the type, patients are most commonly asymptomatic or typically present with systemic symptoms.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Castleman disease | Angiofollicular ganglionic hyperplasia · Angiofollicular lymph hyperplasia |
| fr | Maladie de Castleman | Hyperplasie ganglionnaire angiofolliculaire · Hyperplasie lymphoïde angiofolliculaire · Pseudotumeur de Castleman |
| de | Castleman-Krankheit | Angiofollikuläre Lymphknotenhyperplasie · Castleman-Lymphom |
| es | Enfermedad de Castleman | |
| it | Malattia di Castleman | Iperplasia ganglionaria angiofollicolare · Iperplasia linfatica angiofollicolare |
| nl | Ziekte van Castleman | Angiofolliculaire ganglionhyperplasie · Angiofolliculaire lymfehyperplasie |
| pt | Doença Castleman | Hamartoma linfóide · Hiperplasia ganglionar angiofolicular · Linfoma benigno |
| pl | Choroba Castlemana | Angiofollicular ganglionic hyperplasia · Naczyniowogrudkowy przerost węzłów chłonnych |
| cs | Castlemanova choroba | Angiofolikulární ganglionická hyperplázie · Angiofolikulární hyperplázie uzlin |
| uk | Хвороба Кастлемана | Ангіофолікулярна гангліонарна гіперплазія · Ангіофолікулярна лімфатична гіперплазія |
| zh | Castleman病 | 血管滤泡性淋巴增生 |
| tr | Castleman hastalığı | Anjiyofoliküler lenf hiperplazisi · Anjiyofoliküler ganglionik hiperplazi |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| OMIM | 148000 | BTNT | validated | |
| ICD-10 | D47.7 | NTBT | index | validated |
| ICD-11 | 4B2Y | NTBT | index | validated |
| MONDO | 0015564 | E | validated | |
| MeSH | D005871 | E | validated | |
| UMLS | C0017531 | E | validated | |
| GARD | 12656 | E | validated | |
| MedDRA | 10050251 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:171898Lymphoid hemopathy
rare hematological diseases; rare neoplastic diseases; rare transplant-related disorders
Codes that Orphanet moved or referred to this one:
- ORPHA:93682OBSOLETE: Pediatric Castleman disease referred to · inactive
- ORPHA:93686OBSOLETE: Multicentric Castleman disease referred to · inactive
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Not yet validated | ORPHANET |
| Annual incidence | 1-9 / 100 000 | — | United States | Class only | Validated | ORPHANET_25120049[PMID] |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
29 annotations · validated 2019-04-30| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0002716 | Lymphadenopathy | |
| Frequent (79-30%) | ||
| HP:0002027 | Abdominal pain | |
| HP:0001903 | Anemia | |
| HP:0025142 | Constitutional symptom | |
| HP:0011227 | Elevated circulating C-reactive protein concentration | |
| HP:0003565 | Elevated erythrocyte sedimentation rate | |
| HP:0012378 | Fatigue | |
| HP:0002729 | Follicular hyperplasia | |
| HP:0030783 | Increased circulating interleukin 6 concentration | |
| HP:0100721 | Mediastinal lymphadenopathy | |
| HP:0001824 | Weight loss | |
| Occasional (29-5%) | ||
| HP:0003270 | Abdominal distention | |
| HP:0031500 | Abdominal mass | |
| HP:0011024 | Abnormality of the gastrointestinal tract | |
| HP:0012735 | Cough | |
| HP:0025066 | Decreased mean corpuscular volume | |
| HP:0030157 | Flank pain | |
| HP:0008940 | Generalized lymphadenopathy | |
| HP:0000952 | Jaundice | |
| HP:0002017 | Nausea and vomiting | |
| Very rare (<4-1%) | ||
| HP:0012050 | Anasarca | |
| HP:0002094 | Dyspnea | |
| HP:0000790 | Hematuria | |
| HP:0005214 | Intestinal obstruction | |
| HP:0011974 | Myelofibrosis | |
| HP:0000083 | Renal insufficiency | |
| HP:0001723 | Restrictive cardiomyopathy | |
| HP:0001873 | Thrombocytopenia | |
| HP:0006000 | Ureteral obstruction | |
Run the differential on the 1 obligate / very frequent terms
Annotation source: 30598916[PMID]_30746379[PMID]_30680887[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/160
- This record as JSON: /data/d/160.json