Sézary syndrome
General Information (adopted from Orphanet):
| Synonyms, Signs: |
Sézary lymphoma |
| Number of Symptoms | 26 |
| OrphanetNr: | 3162 |
| OMIM Id: |
|
| ICD-10: |
C84.1 |
| UMLs: |
C0036920 |
| MeSH: |
D012751 |
| MedDRA: |
10040493 |
| Snomed: |
118611004 4950009 |
Prevalence, inheritance and age of onset:
| Prevalence: | 0.18 of 100 000 [Orphanet] |
| Inheritance: |
|
| Age of onset: |
Adult [Orphanet] |
Disease classification (adopted from Orphanet):
| Parent Diseases: |
Aggressive primary cutaneous T-cell lymphoma
-Rare hematologic disease -Rare oncologic disease -Rare skin disease |
Symptom Information:
|
|
(HPO:0001999) | Abnormal facial shape | Occasional [Orphanet] | 169 / 7739 | |||
|
|
(HPO:0000492) | Abnormality of the eyelid | Occasional [Orphanet] | 41 / 7739 | |||
|
|
(HPO:0009830) | Peripheral neuropathy | Occasional [Orphanet] | 206 / 7739 | |||
|
|
(HPO:0001337) | Tremor | Occasional [Orphanet] | 200 / 7739 | |||
|
|
(HPO:0001744) | Splenomegaly | Frequent [Orphanet] | 337 / 7739 | |||
|
|
(HPO:0002240) | Hepatomegaly | Frequent [Orphanet] | 467 / 7739 | |||
|
|
(HPO:0001597) | Abnormality of the nail | Frequent [Orphanet] | 115 / 7739 | |||
|
|
(HPO:0000958) | Dry skin | Very frequent [Orphanet] | 152 / 7739 | |||
|
|
(HPO:0008069) | Neoplasm of the skin | Very frequent [Orphanet] | 84 / 7739 | |||
|
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(HPO:0010783) | Erythema | Very frequent [Orphanet] | 138 / 7739 | |||
|
|
(HPO:0000989) | Pruritus | Very frequent [Orphanet] | 111 / 7739 | |||
|
|
(HPO:0000962) | Hyperkeratosis | Frequent [Orphanet] | 216 / 7739 | |||
|
|
(HPO:0001596) | Alopecia | Frequent [Orphanet] | 162 / 7739 | |||
|
|
(HPO:0100725) | Lichenification | Very frequent [Orphanet] | 14 / 7739 | |||
|
|
(HPO:0007400) | Irregular hyperpigmentation | Occasional [Orphanet] | 72 / 7739 | |||
|
|
(HPO:0002665) | Lymphoma | Very frequent [Orphanet] | 60 / 7739 | |||
|
|
(HPO:0004332) | Abnormality of lymphocytes | Very frequent [Orphanet] | 12 / 7739 | |||
|
|
(HPO:0000969) | Edema | Occasional [Orphanet] | 117 / 7739 | |||
|
|
(HPO:0100758) | Gangrene | Occasional [Orphanet] | 25 / 7739 | |||
|
|
(HPO:0001945) | Fever | Frequent [Orphanet] | 218 / 7739 | |||
|
|
(HPO:0002103) | Abnormality of the pleura | Occasional [Orphanet] | 58 / 7739 | |||
|
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(HPO:0010978) | Abnormality of immune system physiology | Frequent [Orphanet] | 148 / 7739 | |||
|
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(HPO:0002716) | Lymphadenopathy | Very frequent [Orphanet] | 129 / 7739 | |||
|
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(HPO:0003202) | Skeletal muscle atrophy | Occasional [Orphanet] | 281 / 7739 | |||
|
|
(OMIM) | Stiff skin | Very frequent [Orphanet] | 31 / 7739 | |||
|
|
(HPO:0001522) | Death in infancy | Occasional [Orphanet] | 275 / 7739 |
Associated genes:
ClinVar (via SNiPA)
| Gene symbol | Variation | Clinical significance | Reference |
|---|