Angioimmunoblastic T-cell lymphoma

Orpha code: 86886OMIM code:

Definition

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.

Disease data
Classification

Disease

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

No additional description.

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