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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.
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.
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
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
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)
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
Genetic testing
Histologic confirmation
Immunophenotyping
Genetics Data
Gains of 7q and 7p
CDK6 (7q21.2) and CARD11 (7p22.2) expressions
t(14;19)(q11;q13) implicating NECTIN2 (PVRL2)
Deletions of CDKN2A, PTEN, and TP53
Codeletion of CDKN2Aand PTEN
t(5;9)(q33;q22)/ITK::SYK mutation
Immunophenotyping
CD4+ (expression/positive)
CD5 decreased expression
CD7 decreased expression
CD8+ (expression/positive)
CD15+ (expression/positive)
SAP+ (expression/positive)
TCRαβ expression
TFH markers (CD10, BCL6, PD1, ICOS, and CXCL13)-at least two positive
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
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
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
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
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
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
