Chłoniak angioimmunoblastyczny z komórek T

Kod Orpha: 86886Kod OMIM:

Definicja

A rare T-cell non-Hodgkin lymphoma characterized by infiltration of lymph nodes by neoplastic cells of T follicular helper cell origin with a polymorphous inflammatory background including markedly increased follicular dendritic cells and EBV-positive B-cells, as well as prominent proliferation of high endothelial venules. The spleen, liver, skin, and bone marrow are also frequently involved. Patients typically present with generalized lymphadenopathy, hepatosplenomegaly, systemic symptoms, and polyclonal hypergammaglobulinemia. Pruritic skin rash, arthritis, pleural effusion, and ascites may also be observed. The condition is aggressive with generally poor prognosis.

Dane
Klasyfikacja

Choroba

Synonimy
AILT
AILT
Chłoniak z komórek T, typ AILD
Limfadenopatia limfoblastyczna
Limfogranulomatoza X
Immunoblastic lymphadenopathy
Lymphogranulomatosis X
T-cell lymphoma, AILD type
Kod ORPHA
86886
Kod OMIM
-
Kod ICD10
C86.5
Kod ICD11
2A90.9

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