1208725005: Multifocal lymphangioendotheliomatosis, thrombocytopenia syndrome (disorder)

SNOMED CT Concept\Clinical finding (finding)\...

\Finding of blood, lymphatics and immune system\Disorder of cellular component of blood (disorder)\Platelet disorder\Thrombocytopenic disorder\Congenital thrombocytopenia (disorder)\Multifocal lymphangioendotheliomatosis, thrombocytopenia syndrome
\Finding of blood, lymphatics and immune system\Finding of lymph node and lymphatics\Multifocal lymphangioendotheliomatosis, thrombocytopenia syndrome

\General finding of soft tissue\Disorder of soft tissue\Disorder of lymphatic vessel (disorder)\Lymphatic malformation\Multifocal lymphangioendotheliomatosis, thrombocytopenia syndrome

\Procedure related finding\Evaluation finding\...

\Measurement finding\Measurement finding outside reference range\Blood cell count outside reference range\Platelet count outside reference range\Platelet count below reference range (finding)\Thrombocytopenic disorder\Congenital thrombocytopenia (disorder)\Multifocal lymphangioendotheliomatosis, thrombocytopenia syndrome
\Measurement finding\Measurement finding outside reference range\Blood cell count outside reference range\Cytopenia\Platelet count below reference range (finding)\Thrombocytopenic disorder\Congenital thrombocytopenia (disorder)\Multifocal lymphangioendotheliomatosis, thrombocytopenia syndrome
\Measurement finding\Measurement finding outside reference range\Measurement finding below reference range (finding)\Cytopenia\Platelet count below reference range (finding)\Thrombocytopenic disorder\Congenital thrombocytopenia (disorder)\Multifocal lymphangioendotheliomatosis, thrombocytopenia syndrome
\Measurement finding\Platelet count - finding\Platelet count outside reference range\Platelet count below reference range (finding)\Thrombocytopenic disorder\Congenital thrombocytopenia (disorder)\Multifocal lymphangioendotheliomatosis, thrombocytopenia syndrome

\Hematopoietic system finding\Hemostatic system finding\Platelet count - finding\Platelet count outside reference range\Platelet count below reference range (finding)\Thrombocytopenic disorder\Congenital thrombocytopenia (disorder)\Multifocal lymphangioendotheliomatosis, thrombocytopenia syndrome

\Functional finding\Disorder of hemostatic system\Platelet disorder\Thrombocytopenic disorder\Congenital thrombocytopenia (disorder)\Multifocal lymphangioendotheliomatosis, thrombocytopenia syndrome

\Disease\Disorder of cellular component of blood (disorder)\Platelet disorder\Thrombocytopenic disorder\Congenital thrombocytopenia (disorder)\Multifocal lymphangioendotheliomatosis, thrombocytopenia syndrome
\Disease\Disorder of hemostatic system\Platelet disorder\Thrombocytopenic disorder\Congenital thrombocytopenia (disorder)\Multifocal lymphangioendotheliomatosis, thrombocytopenia syndrome
\Disease\Disorder of body system\Disorder of lymphatic system\Disorder of lymphatic vessel (disorder)\Lymphatic malformation\Multifocal lymphangioendotheliomatosis, thrombocytopenia syndrome
\Disease\Congenital disease (disorder)\Congenital thrombocytopenia (disorder)\Multifocal lymphangioendotheliomatosis, thrombocytopenia syndrome
\Disease\Congenital disease (disorder)\Congenital malformation\Lymphatic malformation\Multifocal lymphangioendotheliomatosis, thrombocytopenia syndrome
\Disease\Disorder of soft tissue\Disorder of lymphatic vessel (disorder)\Lymphatic malformation\Multifocal lymphangioendotheliomatosis, thrombocytopenia syndrome
\Disease\Developmental disorder\Congenital malformation\Lymphatic malformation\Multifocal lymphangioendotheliomatosis, thrombocytopenia syndrome

Status: current, Not sufficiently defined by necessary conditions definition status (core metadata concept). Date: 31-Mar 2022. Module: SNOMED CT core

Descriptions:

Id Description Lang Type Status Case? Module

4970633011 Multifocal lymphangioendotheliomatosis, thrombocytopenia syndrome en Synonym (core metadata concept) Active Entire term case insensitive (core metadata concept) SNOMED CT core

4970634017 Cutaneovisceral angiomatosis, thrombocytopenia syndrome en Synonym (core metadata concept) Active Entire term case insensitive (core metadata concept) SNOMED CT core

4970635016 MLT - multifocal lymphangioendotheliomatosis with thrombocytopenia en Synonym (core metadata concept) Active Entire term case sensitive (core metadata concept) SNOMED CT core

4970636015 Multifocal lymphangioendotheliomatosis, thrombocytopenia syndrome (disorder) en Fully specified name Active Entire term case insensitive (core metadata concept) SNOMED CT core

4970637012 A rare lymphatic system anomaly with characteristics of multifocal congenital and progressive vascular lesions of the skin, gastrointestinal tract and occasionally other anatomic sites, causing potentially life-threatening thrombocytopenic coagulopathy. Macroscopically the lesions appear as round to oval red-brown plaques and as large as a few centimeters in diameter. Histopathologically they consist of dilated thin-walled vessels with variable endothelial hyperplasia, positive for lymphatic endothelial cell markers and resembling benign lymphangioendothelioma. en Definition Active Entire term case sensitive (core metadata concept) SNOMED CT core

4970638019 A rare lymphatic system anomaly with characteristics of multifocal congenital and progressive vascular lesions of the skin, gastrointestinal tract and occasionally other anatomic sites, causing potentially life-threatening thrombocytopenic coagulopathy. Macroscopically the lesions appear as round to oval red-brown plaques and as large as a few centimetres in diameter. Histopathologically they consist of dilated thin-walled vessels with variable endothelial hyperplasia, positive for lymphatic endothelial cell markers and resembling benign lymphangioendothelioma. en Definition Active Entire term case sensitive (core metadata concept) SNOMED CT core

5609801000052113 syndrom med multifokal lymfangioendoteliomatos och trombocytopeni sv Synonym (core metadata concept) Active Entire term case insensitive (core metadata concept) SNOMED CT Sweden NRC maintained module (core metadata concept)

Expanded Value Set

Outbound Relationships	Type	Target	Active	Characteristic	Refinability	Group	Values
Multifocal lymphangioendotheliomatosis, thrombocytopenia syndrome	Is a	Lymphatic malformation	true	Inferred relationship	Some
Multifocal lymphangioendotheliomatosis, thrombocytopenia syndrome	Is a	Finding of lymph node and lymphatics	true	Inferred relationship	Some
Multifocal lymphangioendotheliomatosis, thrombocytopenia syndrome	Is a	Congenital thrombocytopenia (disorder)	true	Inferred relationship	Some
Multifocal lymphangioendotheliomatosis, thrombocytopenia syndrome	Interprets	Hemostatic function	true	Inferred relationship	Some	2
Multifocal lymphangioendotheliomatosis, thrombocytopenia syndrome	Has interpretation	Abnormal	true	Inferred relationship	Some	2
Multifocal lymphangioendotheliomatosis, thrombocytopenia syndrome	Interprets	Platelet count	true	Inferred relationship	Some	3
Multifocal lymphangioendotheliomatosis, thrombocytopenia syndrome	Has interpretation	Below reference range	true	Inferred relationship	Some	3
Multifocal lymphangioendotheliomatosis, thrombocytopenia syndrome	Occurrence	Congenital	true	Inferred relationship	Some	1
Multifocal lymphangioendotheliomatosis, thrombocytopenia syndrome	Finding site	Structure of lymphatic vessel	true	Inferred relationship	Some	1
Multifocal lymphangioendotheliomatosis, thrombocytopenia syndrome	Associated morphology	Congenital lymphatic malformation (morphologic abnormality)	true	Inferred relationship	Some	1
Multifocal lymphangioendotheliomatosis, thrombocytopenia syndrome	Pathological process (attribute)	Pathological developmental process	true	Inferred relationship	Some	1

Inbound Relationships

Type

Active

Source

Characteristic

Refinability

Group

This concept is not in any reference sets

Back to Start

Id	Description	Lang	Type	Status	Case?	Module
4970633011	Multifocal lymphangioendotheliomatosis, thrombocytopenia syndrome	en	Synonym (core metadata concept)	Active	Entire term case insensitive (core metadata concept)	SNOMED CT core
4970634017	Cutaneovisceral angiomatosis, thrombocytopenia syndrome	en	Synonym (core metadata concept)	Active	Entire term case insensitive (core metadata concept)	SNOMED CT core
4970635016	MLT - multifocal lymphangioendotheliomatosis with thrombocytopenia	en	Synonym (core metadata concept)	Active	Entire term case sensitive (core metadata concept)	SNOMED CT core
4970636015	Multifocal lymphangioendotheliomatosis, thrombocytopenia syndrome (disorder)	en	Fully specified name	Active	Entire term case insensitive (core metadata concept)	SNOMED CT core
4970637012	A rare lymphatic system anomaly with characteristics of multifocal congenital and progressive vascular lesions of the skin, gastrointestinal tract and occasionally other anatomic sites, causing potentially life-threatening thrombocytopenic coagulopathy. Macroscopically the lesions appear as round to oval red-brown plaques and as large as a few centimeters in diameter. Histopathologically they consist of dilated thin-walled vessels with variable endothelial hyperplasia, positive for lymphatic endothelial cell markers and resembling benign lymphangioendothelioma.	en	Definition	Active	Entire term case sensitive (core metadata concept)	SNOMED CT core
4970638019	A rare lymphatic system anomaly with characteristics of multifocal congenital and progressive vascular lesions of the skin, gastrointestinal tract and occasionally other anatomic sites, causing potentially life-threatening thrombocytopenic coagulopathy. Macroscopically the lesions appear as round to oval red-brown plaques and as large as a few centimetres in diameter. Histopathologically they consist of dilated thin-walled vessels with variable endothelial hyperplasia, positive for lymphatic endothelial cell markers and resembling benign lymphangioendothelioma.	en	Definition	Active	Entire term case sensitive (core metadata concept)	SNOMED CT core
5609801000052113	syndrom med multifokal lymfangioendoteliomatos och trombocytopeni	sv	Synonym (core metadata concept)	Active	Entire term case insensitive (core metadata concept)	SNOMED CT Sweden NRC maintained module (core metadata concept)