Search Database ICD-O-3 Code Lists

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

Coding Manual: Hematopoietic Coding Manual (PDF)

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.

Diagnostic Confirmation

This histology can be determined by positive histology (including peripheral blood) with or without genetics and/or immunophenotyping. Review the Definitive Diagnostic Methods, Immunophenotyping and Genetics Data sections below, and the instructions in the Hematopoietic Manual for further guidance on assigning Diagnostic confirmation.

Grade

Not Applicable

Module Rule

None

Alternate Names

AILD [OBS]
AITL
Angioimmunoblastic lymphadenopathy with dysproteinemia [OBS]
Angioimmunoblastic lymphoma [OBS]
Immunoblastic lymphadenopathy [OBS]
Lymphogranulomatosis X [OBS]
Peripheral T-cell lymphoma, AILD

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.

Definitive Diagnostic Methods

Genetic testing
Histologic confirmation
Immunophenotyping

Genetics Data

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

Immunophenotyping

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

Same Primaries

Corresponding ICD-9 Codes

202.7 Peripheral T-cell lymphoma

Corresponding ICD-10 Codes

C84.5 Other and unspecified T-cell lymphomas

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

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

Signs and Symptoms

Arthritis
Ascites
Drenching night sweats
Fatigue
Fever (for no known reason)
Generalized lymphadenopathy
Hepatosplenomegaly
Pain in the chest, abdomen, or bones (for no known reason)
Pleural effusion
Polyclonal hypergammaglobulinemia
Skin rash or itchy skin
Weight loss (for no known reason)

Diagnostic Exams

Blood chemistry studies
CT (CAT) scan
Flow cytometry
Immunohistochemistry
Immunophenotyping
Laparoscopy (rarely performed)
Laparotomy (rarely performed)
Lymph node biopsy
PET (positron emission tomography) scan
Weight loss (for no known reason)

Progression and Transformation

None

Epidemiology and Mortality

Age: middle age and elderly adults
Incidence: 1-2% of all non-Hodgkin lymphomas
Sex: no male or female predominance
Survival: <3 years medial survival

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, Second Revision. Geneva: World Health Organization, 2020.
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577

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