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primair cutaan gamma/delta T-cellymfoom (aandoening)
primair cutaan gamma/delta T-cellymfoom
Agressieve vorm van huidlymfoom; de kwaadaardige cellen groeien snel en kunnen uitzaaien naar de slijmvliezen en andere delen van het lichaam.
Dit is een vorm van kanker in je huid. De kanker zit in de T-cellen die je lymfeklieren aanmaken. T-cellen helpen je lichaam om te vechten tegen infecties.
Primary cutaneous gamma-delta-positive T-cell lymphoma
Primary cutaneous gamma-delta T-cell lymphoma
Primary cutaneous gamma/delta-positive T-cell lymphoma is a rare, usually aggressive, subtype of cutaneous T-cell lymphoma characterized by infiltration of the epidermis, dermis or subcutaneous tissue by a clonal population of mature, gamma/delta positive cytotoxic T-cells. Typically it presents with ulcerating plaques, tumors, or subcutaneous nodules on the skin of the extremities, however, frequent involvement of mucosal and extranodal sites (such as the nasal cavity, gastrointestinal tract or lungs) is also observed. Cases associated with panniculitis may present with hemophagocytic syndrome (abrupt onset of fever, rash, cytopenia, hepatosplenomegaly and neurological compromise). Infiltration of lymph nodes, spleen and bone marrow is uncommon and resistance to multilineage chemotherapy is reported.
Id733627006
StatusDefined
DHD Diagnosis thesaurus reference set
SNOMED CT to Orphanet simple map178533
SNOMED CT to ICD-10 extended map
TargetC84.4
RuleTRUE
AdviceALWAYS C84.4 | POSSIBLE REQUIREMENT FOR MORPHOLOGY CODE
CorrelationSNOMED CT source code to target map code correlation not specified