Angioimmunoblastic T-cell lymphoma

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Angioimmunoblastic T-cell lymphoma
Other namesimmunoblastic lymphadenopathy (Lukes-Collins Classification), AILD-type (lymphogranulomatosis X) T-cell lymphoma (Kiel Classification)[1]
SpecialtyHematology and oncology

Angioimmunoblastic T-cell lymphoma (AITL, sometimes misspelled AILT, formerly known as "angioimmunoblastic lymphadenopathy with dysproteinemia"[2]: 747 ) is a mature T-cell lymphoma of blood or lymph vessel immunoblasts characterized by a polymorphous lymph node infiltrate showing a marked increase in follicular dendritic cells (FDCs) and high endothelial venules (HEVs) and systemic involvement.[1]

Signs and symptoms

Patients with AITL usually present at an advanced stage and show

skin rash and possibly edema, ascites, pleural effusions, and arthritis.[3][4]

Sites of involvement

Due to the systemic nature of AITL, neoplastic cells can be found in

]

Causes

AITL was originally thought to be a premalignant condition, termed angioimmunoblastic lymphadenopathy, and this atypical reactive lymphadenopathy carried a risk for transformation into a lymphoma. It is postulated that the originating cell for AITL is a mature (post-thymic) CD4+

EBV+ angioimmunoblastic T cell lymphoma, the role of the virus in the development and progression of EBV+ angioimmunoblastic T cell lymphoma is unclear.[8] Immunodeficiency is also seen with AITL, but it is a sequela and not a predisposing factor.[1]

Diagnosis

Laboratory findings

The classical laboratory finding is polyclonal

cold agglutinins, circulating immune complexes, anti-smooth muscle antibodies, and positive rheumatoid factor.[1][3]

Lymph node

The normal architecture of a lymph node is partially effaced by a polymorphous infiltrate and residual follicles are commonly seen. The polymorphous infiltrate consists of lymphocytes of moderate size with pale/clear cytoplasm and smaller reactive

Reed-Sternberg-like cells can also be seen.[9][10]

Immunophenotype

AITL typically has the phenotype of a mixture of

Molecular findings

Clonal

T-cells.[7][13] Trisomy 3, trisomy 5, and +X are the most frequent chromosomal abnormalities found in AITL cases.[14][15]

Treatment

There is no proven or standard first-line chemotherapy that works for the majority of AITL patients. There are several

Stem cell transplantation is the treatment of choice, with the allogeneic one being the preference because AITL tends to recur after autologous transplants.[citation needed
]

Epidemiology

The typical patient with angioimmunoblastic T-cell lymphoma (AITL) is either middle-aged or elderly, and no gender preference for this disease has been observed.[1] AITL comprises 15–20% of peripheral T-cell lymphomas and 1–2% of all non-Hodgkin lymphomas.[16]

See also

References

External links