Search Database ICD-O-3 Code Lists

Name

Mature T-cell lymphoma, NOS (ICD-O-4: 97020/3)

ICD-O-3 Morphology

9702/3: Mature T-cell lymphoma, NOS
Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

Module 7
Common site: lymph nodes, gastrointestinal tract. Most cases present with advanced disease.
Common metastatic sites: liver, bone marrow
Spleen involvement is common due to dissemination of disease

See abstractor notes.

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

Peripheral T cell lymphoma, NOS 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)

Per the WHO Blue Book for Hematolymphoid neoplasms, PTCL-NOS is a "diagnosis of exclusion." This code should only be used when no other information is available. A more specific T-cell lymphoma should be coded.

nTFHL-F presents with disseminated nodal involvement, extranodal sites include skin, liver, spleen and bone marrow. Most patients present with advanced stage disease and generalized lymphadenopathy.

nTFHL-NOS is characterized by disseminated disease with generalized lymphadenopathy and involvement of the spleen, liver, skin, and bone marrow. Most patients present with advanced stage disease.

EBV-positive nodal T- and NK-cell lymphoma mostly involved the lymph nodes (most common cervical, inguinal, and axillary). Extranodal involvement may be found in the liver, bone marrow, skin and GI tract.

Diagnostic Confirmation

Assign code 1 for histological confirmation WITH or WITHOUT positive immunophenotyping or genetics

Module Rule

None

Alternate Names

EBV-positive nodal T- and NK- cell lymphoma (ICD-O-4: 97025/3)
Follicular (helper) T-cell lymphoma, NOS (ICD-O-4: 97023/3)
Follicular helper T-cell lymphoma, follicular type (ICD-O-4: 97023/3)
Lennert lymphoma (ICD-O-4: 97022/3)
Lymphoepithelioid lymphoma (ICD-O-4: 97022/3)
Nodal peripheral T-cell lymphoma with T follicular helper (TFH) phenotype
Nodal EBV+ cytotoxic T-cell lymphoma (ICD-O-4: 97025/3)
Nodal T follicular helper cell lymphoma, NOS (nTFHL) (ICD-O-4: 97024/3)
Nodal T follicular helper cell lymphoma, angioimmunoblastic type (nTFHL-AL)
Nodal T follicular helper cell lymphoma, follicular type (nTFHL-F) (ICD-O-4: 97023/3)
Nodal peripheral T-cell lymphoma, EBV+ positive (ICD-O-4: 97025/3)
Nodal peripheral T-cell lymphoma with T follicular helper (TFH) phenotype (ICD-O-4: 97023/3)
Peripheral T-cell lymphoma, large cell (ICD-O-4: 97020/3)
Peripheral T-cell lymphoma, NOS (ICD-O-4: 97020/3)
Peripheral T-cell lymphoma, pleomorphic medium and large cell (ICD-O-4: 97020/3)
Primary nodal EBV-positive T/NK-cell lymphoma (ICD-O-4: 97025/3)
T-cell lymphoma, NOS (ICD-O-4: 97020/3)
T-zone lymphoma (ICD-O-4: 97020/3)

Definition

PTCL-NOS patients present with advanced disease. Lymph nodes are the most common site; however, other sites such as the bone marrow, liver, spleen, skin and the GI can also be involved. Leukemic and CNS presentations are common.

Nodal T follicular helper (TFH) cell lymphoma, follicular type (nTFHL-F), is a nodal peripheral T-cell lymphoma with a TFH phenotype that displays a follicular growth pattern and lacks the prominent high endothelial venules (HEVs) and extrafollicular follicular dendritic cell (FDC) meshworks characteristic of nodal TFH cell lymphoma, angioimmunoblastic type (nTFHL-AI). (WHO 5th edition).

Nodal T follicular helper (TFH) cell lymphoma (nTFHL) NOS is a nodal peripheral T-cell neoplasm with a TFH phenotype, demonstrated by the expression of CD4 and at least two TFH markers, that does not fulfil the required histopathological criteria for nTFHL, angioimmunoblastic type (nTFHL-AI) or nTFHL, follicular type. (WHO 5th edition)

EBV-positive nodal T- and NK-cell lymphoma is an EBV-positive lymphoma of cytotoxic T- or NK-cell lineage, presenting primarily with nodal disease in adults. (WHO 5th edition)

Definitive Diagnostic Methods

Cytogenetics
Genetic testing
Histologic confirmation
Immunohistochemistry
Immunophenotyping

Genetics Data

DNMT3A, TET2, RHOA, IDH2 mutations (nTFHL)

Immunophenotyping

PD1, ICOS1, CSCL13, CD10, BCL6 (nTFHL)
CD4, CD5, CD7, CD8, TCRab, TCRy

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)

C84.4 Peripheral T-cell lymphoma
C84.2 T-zone lymphoma
C84.3 Lymphoepithelioid lymphoma

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

C84.4_ Peripheral T-cell lymphoma, not classified (effective October 01, 2015)
C84.4A Peripheral T-cell lymphoma, not classified, in remission (effective October 01, 2024)

Signs and Symptoms

Drenching night sweats
Eosinophilia
Fatigue
Fever (for unknown reasons)
Hemophagocytic syndrome (rare)
Generalized lymphadenopathy (peripheral lymphadenopathy most common)
Pain in the chest, abdomen, or bones (for no known reason)
Pruritis
Weight loss (for no known reason)

Diagnostic Exams

Blood chemistry studies
Bone marrow aspiration and biopsy
CT (CAT) scan
Flow cytometry
Immunohistochemistry
Immunophenotyping
PET (positron emission tomography) scan
Skin biopsy

Progression and Transformation

Frequent relapses of disease after treatment

Epidemiology and Mortality

Age: mostly occurs in adults (PTCL-NOS)
Sex: male predilection (PTCL-NOS)
Survival: 20-30% 5-year survival (PTCL-NOS)
Age: median age 60-65 (nTFHL-F)
Incidence: 1.8-2.6% of non-cutaneous peripheral T-cell lymphomas (nTFHL-F)
Sex: M:F ratio 1.05-1 (nTFHL-F)
Survival: 50-60% at 2 years (limited data available) (nTFHL-F)
Incidence: rare lymphoma (mostly in eastern Asia) (EBV+)
Age: median age 61-64 (EBV+)
Sex: M:F ratio is 1.5-3.8 to 1 (EBV+)

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: 752-764

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