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721093000: Dianzani autoimmune lymphoproliferative disease (disorder)


Status: current, Primitive. Date: 31-Jan 2017. Module: SNOMED CT core

Descriptions:

Id Description Lang Type Status Case? Module
3323468019 Dianzani autoimmune lymphoproliferative disease (disorder) en Fully specified name Active Case sensitive SNOMED CT core
3323469010 Dianzani autoimmune lymphoproliferative disease en Synonym Active Case sensitive SNOMED CT core
3323470011 DALD - Dianzani autoimmune lymphoproliferative disease en Synonym Active Case sensitive SNOMED CT core
3323471010 A very rare disorder with characteristics of autoimmunity, lymphadenopathy and/or splenomegaly. The prevalence is not known. The disorder has been reported in fewer than 30 patients to date. Age of onset is highly variable, ranging from childhood to young adulthood. A possible increased risk of cancer has been suggested in these patients. The cause is not known but it is thought to be hereditary. Biologically, DALD has characteristics of normal double-negative T-cells (DNTs) and defective in vitro FAS-mediated apoptosis. The pattern of inheritance of DALD is not known. en Definition Active Case sensitive SNOMED CT core


0 descendants.

Expanded Value Set


Outbound Relationships Type Target Active Characteristic Refinability Group Values
Dianzani autoimmune lymphoproliferative disease Associated morphology Lymphoproliferative disorder false Inferred relationship Some
Dianzani autoimmune lymphoproliferative disease Is a Hereditary disease false Inferred relationship Some
Dianzani autoimmune lymphoproliferative disease Is a Autoimmune disease true Inferred relationship Some
Dianzani autoimmune lymphoproliferative disease Is a Lymphoproliferative disorder true Inferred relationship Some
Dianzani autoimmune lymphoproliferative disease Pathological process Autoimmune process false Inferred relationship Some
Dianzani autoimmune lymphoproliferative disease Is a Hereditary neoplastic syndrome true Inferred relationship Some
Dianzani autoimmune lymphoproliferative disease Associated morphology Lymphoproliferative disorder true Inferred relationship Some 1
Dianzani autoimmune lymphoproliferative disease Pathological process Autoimmune process true Inferred relationship Some 1

Inbound Relationships Type Active Source Characteristic Refinability Group

Reference Sets

Australian emergency department reference set

Clinical finding foundation reference set

Problem/Diagnosis reference set

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