HaematologyTier 2Disease (DEADMAN)

Peripheral T-cell lymphoma

Description

Peripheral T-cell lymphoma (PTCL) is an umbrella term for a heterogeneous group of aggressive non-Hodgkin lymphomas arising from mature (post-thymic) T cells - collectively less common than B cell lymphomas and generally associated with a worse prognosis than equivalent-stage aggressive B cell lymphoma

Represents a minority of all non-Hodgkin lymphomas overall (with regional variation - some subtypes are more common in specific geographic/ethnic populations, e.g. adult T cell leukaemia/lymphoma associated with HTLV-1 in endemic regions)

A heterogeneous category encompassing multiple distinct entities (PTCL-not otherwise specified being the most common single subtype, alongside angioimmunoblastic T cell lymphoma, anaplastic large cell lymphoma, and others) - unlike B cell lymphomas, T cell lymphoma classification and standard treatment approaches are generally less well-refined, reflecting their rarity and biological heterogeneity

Lymph node biopsy with immunohistochemistry (T cell markers, subtype-specific markers) is required for diagnosis and subtyping; anaplastic large cell lymphoma is further subclassified by ALK status (ALK-positive disease, more common in younger patients, generally carries a better prognosis than ALK-negative disease)
Staging follows standard lymphoma principles (CT/PET, bone marrow biopsy)

Combination chemotherapy (CHOP-based regimens, historically) is standard first-line treatment, though outcomes are generally inferior to equivalent B cell lymphoma regimens, reflecting the more aggressive/treatment-resistant biology of many PTCL subtypes
Autologous stem cell transplant in first remission is considered for many subtypes given the relatively high relapse rate with chemotherapy alone
Newer targeted agents (e.g. brentuximab vedotin for CD30-positive subtypes including anaplastic large cell lymphoma) have improved outcomes in specific subtypes

HTLV-1 infection (adult T cell leukaemia/lymphoma), angioimmunoblastic T cell lymphoma (associated with autoimmune-like systemic features), anaplastic large cell lymphoma (ALK status prognostic)

The relatively poorer prognosis of PTCL compared with equivalent-stage B cell lymphoma, combined with its rarity and biological heterogeneity, means clinical trial enrolment is particularly valuable where available, given the comparatively less mature evidence base for standard treatment compared with the well-established B cell lymphoma treatment paradigms

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