Peripheral T-cell lymphoma, NOS

ICD-O-2 Morphology
9702/3: Mature T-cell lymphoma, NOS
Effective 1992 - 2000
ICD-O-3 Morphology
9702/3: Mature T-cell lymphoma, NOS
Effective 2001 and later
for cases diagnosed 1992 and later
Primary Site(s)
See Module 7
Most common sites of involvement: lymph nodes, skin and GI tract. Less common sites of involvement: lung, salivary gland, CNS

Not Applicable
Module Rule
Alternate Names
Anaplastic large cell lymphoma, ALK-negative
Expanded mantle-zone T-cell lymphoma
Follicular T-cell lymphoma, NOS
Intrafollicular T-cell lymphoma
Lennert lymphoma
Lymphoepithelioid lymphoma
Mature T-cell lymphoma, NOS
Paracortical nodular T-cell lymphoma
Perifollicular T-cell lymphoma, NOS
T-immunoblastic lymphoma
T-zone lymphoma
A large group of lymphomas which are collectively referred to as peripheral T-cell lymphomas with the optional addition of "unspecified" to emphasize that these cases do not belong to any better defined entities. Attempts to distinguish between them on morphological basis have met with poor reproducibility.
Abstractor Notes
Disease is often generalized with infiltrates in the bone marrow, liver, spleen, and extranodal tissue. Peripheral blood is sometimes involved, but leukemic presentation is uncommon.

The diagnosis of PTCL, NOS would be made ONLY when other specific entities have been excluded.

There are four variants of PTCL, NOS:
1. Lymphoepitheliod (Lennert lymphoma) shows diffuse or more rarely interfollicular growth. Reed-Sternberg-like cells (usually EBV+) may be scattered. Majority of cases are CD8 positive.

2. Follicular T-cell lymphoma, NOS (perifollicular T-cell lymphoma, intrafollicular T-cell lymphoma, paracortical nodular T-cell lymphoma, expanded mantle zone T-cell lymphoma) usually consists of atypical clear cells forming intrafollicular aggregates (mimicking follicular lymphoma), small nodular aggregates in a background of progressively-transformed germinal centers (mimicking nodular lymphocyte-predominant Hodgkin lymphoma), or enlarged perifollicular zones/nodular aggregates surrounding hyperplastic follicles (mimicking nodal marginal zone lymphoma). Partial lymph node involvement, lack of enlarged follicular dendritic cells meshworks and lack of prominent high endothelial venules distinguish it from typical angioimmunoblastic T-cell lymphoma.

3. T-zone T-cell lymphoma-variant characterized by perifollicular growth pattern throughout the lymph nodes. Some studies suggest this variant may have a more indolent course than other PTCL, NOS.

4. Anaplastic large cell lymphoma, ALK-negative (ALCL, ALK-) involves both lymph nodes and extranodal tissue. Extranodal sites include bone, soft tissues, and skin.
Definitive Diagnostic Methods
Genetic testing
Histologic confirmation
Genetics Data
Recurrent chromosomal genes 7q, 8q, 17q, and 22q
Recurrent losses in chromosomes 4q, 5q, 6q, 9p, 10q, 12q, and 13q
T-cell receptor (TCR) genes are clonally rearranged
CD3+ (T-zone variant)
CD4+ (NOS and T-zone variant)
CD8+ (lymphoepitheliod variant)
CD8- (NOS)
CD30+ (ALCL, ALK negative)
CD57- (NOS)
Downregulation CD5
Downregulation CD7
Scattered Reed-Sternberg-like cells usually EBV+ (Lymphoepitheliod variant)
TCR beta chain (beta F1)+ (NOS)
Stem cell transplant
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)
C84.4 Peripheral T-cell lymphoma, not classified (effective October 01, 2015)
C84.7 Anaplastic large cell lymphoma, ALK-negative (effective October 01, 2015)
Signs and Symptoms
Drenching night sweats
Fever (for unknown reasons)
Hemophagocytic syndrome (rare)
Generalized lymphadenopathy (peripheral lymphadenopathy most common)
Pain in the chest, abdomen, or bones (for no known reason)
Weight loss (for no known reason)
Diagnostic Exams
Progression and Transformation
Frequent relapses of disease after treatment
Epidemiology and Mortality
Age: mostly occurs in adults
Sex: male predominance
Survival: 20-30% 5 year survival