Search Database ICD-O-3 Code Lists

Name

Nodal T follicular helper cell lymphoma, angioimmunoblastic type

ICD-O-3 Morphology

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

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

Module 7
Most common sites of involvement: lymph nodes
Common metastatic sites: liver, bone marrow
Spleen involvement is common due to dissemination of disease

Assign C779 if specific primary site cannot be determined.

See abstractor notes.

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

Nodal T follicular helper cell lymphoma, angioimmunoblastic type is part of the Mature T-cell and NK-cell neoplasms lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B20)

Most patients with nTFHL-AI present with generalized lymphadenopathy}. The spleen, liver, skin, and bone marrow are frequently involved. Leukemic presentation is rare, but neoplastic T cells may be detected in the peripheral blood by flow cytometry.

Most patients present with advanced stage and B symptoms. Peripheral lymphadenopathy, hepatosplenomegaly, and bone marrow involvement are frequent.

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.

Module Rule

None

Alternate Names

Angioimmunoblastic lymphoma [OBS]
Angioimmunoblastic T-cell lymphoma (AITL)
Follicular helper T-cell lymphoma, angioimmunoblastic type
Peripheral T-cell lymphoma, AILD (Angioimmunoblastic Lymphadenopathy with Dysprotenemia) [OBS]

Definition

Nodal T follicular helper (TFH) cell lymphoma, angioimmunoblastic type (nTFHL-AI), is a neoplasm of mature T cells with a TFH phenotype, characterized by systemic disease and a polymorphous lymphoid infiltrate involving lymph nodes, accompanied by a prominent proliferation of high endothelial venules (HEVs) and follicular dendritic cells (FDCs). (WHO 5th edition)

Definitive Diagnostic Methods

Genetic testing
Histologic confirmation
Immunophenotyping

Genetics Data

IDH2 p.R172 mutation
p.G17V (NP_001655.1) mutated variant
Somatic genetic changes with TET2 and DNMT3A
TFH gene expression signature

Immunophenotyping

BCL6+ (expression/positive)
CD2+ (expression/positive)
CD3+ (expression/positive)
CD4+ (expression/positive)
CD5+ (expression/positive)
CD8- (no expression/negative)
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-10 Codes (Cause of Death codes only)

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 - September 30, 2024)
C86.50 Angioimmunoblastic T-cell lymphoma, not having achieved remission (effective October 01, 2024)
C86.51 Angioimmunoblastic T-cell lymphoma, in remission (effective October 01, 2024)

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
Bone marrow aspiration and biopsy
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

Incidence: 36% of non-cutaneous T-cell lymphomas
Age: middle age and elderly adults
Sex: slight male predilection

Sources

WHO Classification of Tumours Editorial Board. Haematolymphoid tumours. Lyon (France): International Agency for Research on Cancer; 2024. (WHO classification of tumours series, 5th ed.; vol. 11). https://publications.iarc.who.int/637.
Section: Mature T- and NK-cell neoplasms
Pages: Part B: 744-751

International Classification of Diseases for Oncology, 3rd edition (including revisions). Geneva: World Health Organization, 2001, 2011, 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

PDQ® Adult Treatment Editorial Board. PDQ Peripheral T-Cell Non-Hodgkin Lymphoma Treatment. Bethesda, MD: National Cancer Institute. Updated <03/13/2025>. Available at: https://www.cancer.gov/types/lymphoma/hp/peripheral-t-cell-lymphoma-pdq. Accessed <03/31/2025>. [PMID: 37437079]
Section: Peripheral T-Cell Non-Hodgkin Lymphoma Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/lymphoma/hp/peripheral-t-cell-lymphoma-pdq#_2289
Glossary