Castleman disease
General Information (adopted from Orphanet):
| Synonyms, Signs: |
Angiofollicular ganglionic hyperplasia Angiofollicular lymph hyperplasia |
| Number of Symptoms | 34 |
| OrphanetNr: | 160 |
| OMIM Id: |
|
| ICD-10: |
D36.0 |
| UMLs: |
C0017531 C2931179 |
| MeSH: |
C536362 D005871 |
| MedDRA: |
10050251 |
| Snomed: |
207036003 238809002 |
Prevalence, inheritance and age of onset:
| Prevalence: | 1,000 cases [Orphanet] |
| Inheritance: |
Not applicable [Orphanet] |
| Age of onset: |
All ages [Orphanet] |
Disease classification (adopted from Orphanet):
| Parent Diseases: |
HHV-8 related disorders
-Rare infectious disease -Rare oncologic disease |
Symptom Information:
|
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(HPO:0000083) | Renal insufficiency | Occasional [Orphanet] | 232 / 7739 | |||
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(HPO:0000869) | Secondary amenorrhea | Occasional [Orphanet] | 42 / 7739 | |||
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(HPO:0100639) | Erectile abnormalities | Occasional [Orphanet] | 15 / 7739 | |||
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(HPO:0001970) | Tubulointerstitial nephritis | Occasional [Orphanet] | 27 / 7739 | |||
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(HPO:0100820) | Glomerulopathy | Occasional [Orphanet] | 46 / 7739 | |||
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(HPO:0000093) | Proteinuria | Occasional [Orphanet] | 169 / 7739 | |||
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(HPO:0009830) | Peripheral neuropathy | Occasional [Orphanet] | 206 / 7739 | |||
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(HPO:0000771) | Gynecomastia | Occasional [Orphanet] | 53 / 7739 | |||
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(HPO:0000819) | Diabetes mellitus | Occasional [Orphanet] | 131 / 7739 | |||
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(HPO:0000821) | Hypothyroidism | Occasional [Orphanet] | 141 / 7739 | |||
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(HPO:0001182) | Tapered finger | Occasional [Orphanet] | 93 / 7739 | |||
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(HPO:0100749) | Chest pain | Frequent [Orphanet] | 92 / 7739 | |||
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(HPO:0100769) | Synovitis | Occasional [Orphanet] | 86 / 7739 | |||
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(HPO:0001744) | Splenomegaly | Very frequent [Orphanet] | 337 / 7739 | |||
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(HPO:0001541) | Ascites | Occasional [Orphanet] | 94 / 7739 | |||
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(HPO:0002240) | Hepatomegaly | Very frequent [Orphanet] | 467 / 7739 | |||
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(HPO:0002027) | Abdominal pain | Frequent [Orphanet] | 184 / 7739 | |||
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(HPO:0004325) | Decreased body weight | Occasional [Orphanet] | 492 / 7739 | |||
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(HPO:0011362) | Abnormal hair quantity | Occasional [Orphanet] | 92 / 7739 | |||
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(HPO:0000988) | Skin rash | Occasional [Orphanet] | 98 / 7739 | |||
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(HPO:0001052) | Nevus flammeus | Occasional [Orphanet] | 88 / 7739 | |||
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(HPO:0001933) | Subcutaneous hemorrhage | Occasional [Orphanet] | 50 / 7739 | |||
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(HPO:0000953) | Hyperpigmentation of the skin | Occasional [Orphanet] | 75 / 7739 | |||
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(HPO:0200037) | Skin vesicle | Occasional [Orphanet] | 102 / 7739 | |||
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(HPO:0001873) | Thrombocytopenia | Occasional [Orphanet] | 224 / 7739 | |||
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(HPO:0001903) | Anemia | Occasional [Orphanet] | 289 / 7739 | |||
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(HPO:0001872) | Abnormality of thrombocytes | Occasional [Orphanet] | 20 / 7739 | |||
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(HPO:0000969) | Edema | Occasional [Orphanet] | 117 / 7739 | |||
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(HPO:0011227) | Elevated C-reactive protein level | Frequent [Orphanet] | 55 / 7739 | |||
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(HPO:0001945) | Fever | Occasional [Orphanet] | 218 / 7739 | |||
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(HPO:0001004) | Lymphedema | Occasional [Orphanet] | 62 / 7739 | |||
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(HPO:0002103) | Abnormality of the pleura | Occasional [Orphanet] | 58 / 7739 | |||
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(HPO:0002716) | Lymphadenopathy | Occasional [Orphanet] | 129 / 7739 | |||
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(HPO:0001324) | Muscle weakness | Occasional [Orphanet] | 859 / 7739 |
Associated genes:
ClinVar (via SNiPA)
| Gene symbol | Variation | Clinical significance | Reference |
|---|