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Mondo Disease Ontology
| Preferred Name | kaposiform lymphangiomatosis | |
| Synonyms |
KLA |
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| Definitions |
A generalized lymphatic anomaly characterized by kaposiform spindled lymphatic endothelial cells. Despite the histological similarity between KHE and KLA, the clinical and imaging features are distinctive. KHE-spectrum lesions are unifocal vascular tumors, except in rare reports of multifocal disease. KHE typically presents in early infancy with a characteristic purpuric, cutaneous lesion[PMID:24252784] |
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| ID |
http://purl.obolibrary.org/obo/MONDO_0018736 |
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| comment |
Despite the histological similarity between KHE and KLA, the clinical and imaging features are distinctive. KHE-spectrum lesions are unifocal vascular tumors, except in rare reports of multifocal disease. KHE typically presents in early infancy with a characteristic purpuric, cutaneous lesion[PMID:24252784]
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| database_cross_reference |
Orphanet:464329 GARD:13451 UMLS:C5681097 MEDGEN:1807991 icd11.foundation:1139222402
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|
| definition |
A generalized lymphatic anomaly characterized by kaposiform spindled lymphatic endothelial cells.
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| has_broad_synonym |
KLA
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| id |
MONDO:0018736
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| in_subset |
http://purl.obolibrary.org/obo/mondo/mondo-simple#orphanet_rare http://purl.obolibrary.org/obo/mondo/mondo-simple#rare http://purl.obolibrary.org/obo/mondo/mondo-simple#ordo_disorder |
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| label |
kaposiform lymphangiomatosis
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| notation |
MONDO:0018736
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| prefLabel |
kaposiform lymphangiomatosis
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| skos_exactMatch |
http://purl.obolibrary.org/obo/Orphanet_464329 http://identifiers.org/medgen/1807991 http://linkedlifedata.com/resource/umls/id/C5681097 http://purl.obolibrary.org/obo/mondo/sources/icd11foundation/1139222402 |
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