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ziekte van B-celexpansie met 'nuclear factor kappa-light-chain-enhancer of activated B-cells' en T-celanergie (aandoening)
BENTA-ziekte
ziekte van B-celexpansie met nucleaire factor-kappa-B en T-celanergie
ziekte van B-celexpansie met 'nuclear factor kappa-light-chain-enhancer of activated B-cells' en T-celanergie
ziekte van B-celexpansie met NF-kB en T-celanergie
BENTA disease
B-cell expansion with NF-kB and T-cell anergy disease
BENTA (B-cell expansion with nuclear factor kappa light chain enhancer of activated B cells and T-cell anergy) disease
B-cell expansion with nuclear factor kappa light chain enhancer of activated B cells and T-cell anergy disease
A rare primary immunodeficiency characterized by infantile onset of generalized lymphadenopathy, splenomegaly and lymphocytosis with excessive polyclonal expansion of B-cells. Patients present recurrent infections and impaired T-cell and antibody responses, while overt autoimmune manifestations are usually absent. Occurrence of B-cell malignancy later in life has been reported.
Id1179300002
StatusPrimitive
Pathological processafwijkend immuunproces
SNOMED CT to Orphanet simple map464336
SNOMED CT to ICD-10 extended map
TargetD81.8
RuleTRUE
AdviceALWAYS D81.8 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified