Variety of large pleomorphic cells including hallmark-type cells may be present
Hallmark cells present
Both may show large pleomorphic T cells with abundant cytoplasm and be CD30+. *Cases currently classified as anaplastic large cell lymphoma which are ALK negative are clinically indistinguishable from peripheral T cell lymphoma, unspecified and will likely be merged with this category in the future.
Both may show CD30+, CD15+, EBV+, CD20+ large cells with Reed-Sternberg morphology and polymorphic background infiltrate including plasma cells, histiocytes, and eosinophils; generalized lymphadenopathy with constitutional symptoms.
No follicular dendritic cell proliferation outside germinal centers
Aggressive clinical course.
Both may show paracortical expansion with admixed histiocytes, plasma cells, and eosinophils, increased vascularity, reactive or atretic follicles.
*Except in cases with concomitant lymph node involvement by cutaneous T cell lymphoma.
Both may show diffuse effacement by atypical T cell population.
Most common T cell lymphoma category in non-EBV-endemic populations (North America/Europe)
Predominantly nodal presentation
Extranodal involvement common
skin, gastrointestinal tract, liver, bone marrow
Generally aggressive lymphomas with poor response to therapy
Category likely comprises heterogeneous clinical entities which cannot be reliably distinguished
Morphologic categories not prognostically useful
Cytotoxic marker (granzyme and/or TIA-1) positivity has been proposed as a poor prognostic factor in a Japanese study; however the poor outcome could also have been due to concomitant EBV positivity, and it is not clear whether this is true in non-EBV endemic populations
Grading / Staging / Report
Grading not applicable
Ann Arbor Staging System
I if involvement of a single lymph node region
IE if involvement of a single extralymphatic organ or site
II if two or more lymph node regions on same side of diaphragm
IIE if localized involvement of an extralymphatic organ or site and one or more lymph node regions on the same side of the diaphragm
III if Involvement of lymph node regions on both sides of the diphragm
IIIS if spleen involved
IIIE if extralymphatic site involved
Diffuse or disseminated involvement of one or more extralymphatic organs or tissues, with or without associated lymph node involvement
Systemic Symptoms in 6 months preceding admission
Fever, night sweats, 10% weight loss
A = absent
B = present
Extranodal sites are also designated
M+ = marrow
L+ = lung
H+ = liver
P+ = pleura
O+ = bone
D+ = skin and subcutaneous tissue
Although originally designed for Hodgkin lymphoma, the Ann Arbor System is also used for non-Hodgkin lymphomas.
The pathology report should contain the following information:
Diagnosis in the World Health Organization (WHO) classification
Equivalent diagnosis in other classifications used by relevant clinicians
Results of supplementary studies if performed
Relationship to other specimens from the same patient
Information relevant to staging if available
Mature T and NK cell neoplasms (WHO classification)
T cell prolymphocytic leukemia
T cell large granular lymphocytic leukemia
Aggressive NK cell leukemia
Adult T cell leukemia/lymphoma
Anaplastic large cell lymphoma, primary cutaneous
CD4+/CD56+ hematodermic neoplasm (Blastic NK cell lymphoma)
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Jaffe ES, Harris NL Stein H, Vardiman JW . Pathology and Genetics of Tumours of Haematopoietic and Lymphoid Tissues, World Health Organization Classification of Tumours 2001
Ioachim HL, Ratech H. Ioachim’s Lymph Node Pathology. Lippincott Williams and Wilkins, 3rd Edition, 2002.
Tan BT, Warnke RA, Arber DA. The frequency of B- and T-cell gene rearrangements and epstein-barr virus in T-cell lymphomas: a comparison between angioimmunoblastic T-cell lymphoma and peripheral T-cell lymphoma, unspecified with and without associated B-cell proliferations. J Mol Diagn. 2006 Sep;8(4):466-75.
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