Name

Angioimmunoblastic T-cell lymphoma

ICD-O-2 Morphology

9705/3: Angioimmunoblastic T-cell lymphoma
Effective 1992 - 2000

ICD-O-3 Morphology

9705/3: Angioimmunoblastic T-cell lymphoma
Effective 2001 and later

Reportable

for cases diagnosed 1992 and later

Primary Site(s)

See Module 7
Most common sites of involvement: lymph nodes

Grade

Not Applicable

Module Rule

None

Alternate Names

AILD [OBS]
AITL
Angioimmunoblastic lymphadenopathy with dysproteinemia [OBS]
Angioimmunoblastic lymphoma [OBS]
Follicular T-cell lymphoma
Immunoblastic lymphadenopathy [OBS]
Lymphogranulomatosis X [OBS]
Nodal peripheral T-cell lymphoma with T follicular helper phenotype

Definition

Angioimmunoblastic T-cell lymphoma (AITL) is a neoplasm of mature T follicular helper (TFH) cells characterized by systemic disease and a polymorphous infiltrate involving lymph nodes, with a prominent proliferation of high endothelial venules (HEVs) and follicular dendritic cells (FDCs). EBV-positive B cells are nearly always present, and in some cases constitute a significant part of the cellular infiltrate.

Abstractor Notes

AITL typically presents with advanced stage involvement, with spleen, liver, skin and bone marrow frequently involved.

Patients exhibit immunodeficiency secondary to the neoplastic process, in the majority of cases the expansion of B-cells positive for EBV is seen, thought to be a consequence of underlying immune dysfunction.

Aggressive clinical course with patients often succumbing to infectious complications, which makes delivery of aggressive chemotherapy difficult.

Definitive Diagnostic Methods

Genetic testing
Histologic confirmation
Immunophenotyping

Genetics Data

Clonal IG rearrangements
DNMT3A
EBV-positive B cells
IDH2 R172
TET2

Immunophenotyping

BCL6 expression
CD2 expression
CD3 expression
CD4 positive
CD5 expression
CD8 positive T cells
CD10 expression
CDXCL13 expression
ICOS expression
PD1 (CD279) expression

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Hormone therapy

Transformations to

There are no known transformations

Transformations from

There are no known transformations

Corresponding ICD-9 Codes

202.7 Peripheral T-cell lymphoma

Corresponding ICD-10 Codes

C84.4 Peripheral T-cell lymphoma

Corresponding ICD-10-CM Codes (U.S. only)

C86.5 Angioimmunoblastic T-cell lymphoma (effective October 01, 2015)

Signs and Symptoms

Drenching night sweats
Fever (for no known reason)
Pain in the chest, abdomen, or bones (for no known reason)
Polyclonal hypergammaglobulinemia
Skin rash or itchy skin
Weight loss (for no known reason)

Diagnostic Exams

CT (CAT) scan
Complete blood count (CBC)
Laparoscopy (rarely performed)
Laparotomy (rarely performed)
MRI (magnetic resonance imaging)
PET (positron emission tomography) scan
Weight loss (for no known reason)

Progression and Transformation

None

Epidemiology and Mortality

Age: middle age and elderly adults
Sex: no male or female predominance

Sources

Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (Eds):
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Mature T- and NK-cell neoplasms
Pages: 408-410

International Classification of Diseases for Oncology, Third Edition, First Revision. Geneva: World Health Organization, 2013.
Section: ICD-O-3.1 (2011) Morphological Codes
Pages: http://codes.iarc.fr/codegroup/2

National Cancer Institute
Section: General Information About Adult Non-Hodgkin Lymphoma (NHL)
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq
Glossary