T-cell lymphoma classification

T-cell lymphoma Microchapters
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Overview
Classification Lymphoblastic lymphoma T-cell prolymphocytic leukemia T-cell granular lymphocytic leukemia Chronic lymphoproliferative disorder of NK cells Aggressive NK-cell leukemia Adult T-cell leukemia/lymphoma Enteropathy-type intestinal T-cell lymphoma Hepatosplenic T-cell lymphoma Subcutaneous panniculitis-like T-cell lymphoma Mycosis fungoides Sézary syndrome Angioimmunoblastic T-cell lymphoma Anaplastic large cell lymphoma

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sogand Goudarzi, MD [2]

Overview

There is no established system for the classification of [disease name].

OR

[Disease name] may be classified according to [classification method] into [number] subtypes/groups: [group1], [group2], [group3], and [group4].

OR

[Disease name] may be classified into [large number > 6] subtypes based on [classification method 1], [classification method 2], and [classification method 3]. [Disease name] may be classified into several subtypes based on [classification method 1], [classification method 2], and [classification method 3].

OR

Based on the duration of symptoms, [disease name] may be classified as either acute or chronic.

OR

If the staging system involves specific and characteristic findings and features: According to the [staging system + reference], there are [number] stages of [malignancy name] based on the [finding1], [finding2], and [finding3]. Each stage is assigned a [letter/number1] and a [letter/number2] that designate the [feature1] and [feature2].

OR

The staging of [malignancy name] is based on the [staging system].

OR

There is no established system for the staging of [malignancy name].

Classification

Based on the 2017 revision of the World Health Organization classification of lymphoid neoplasms classified there are four clinical variants of adult T cell leukemia-lymphoma (ATL).

• Indolent T‐cell lymphoproliferative disorder of the gastrointestinal tract
• Primary cutaneous acral CD8 +  T‐cell lymphoma
• Breast implant‐associated anaplastic large cell lymphoma
• Nomenclature changes
• Systemic EBV+ T‐cell lymphoma in childhood (systemic EBV+ T‐cell lymphoproliferative disorder
• Hydroa vacciniforme lymphoproliferative disorder (Hydroa vacciniforme‐like lymphoma)
• Enteropathy‐associated T‐cell lymphoma (Enteropathy‐associated T‐cell lymphoma type 1)
• Monomorphic epitheliotropic intestinal T‐cell lymphoma (Enteropathy‐associated T‐cell lymphoma type 2)
• Primary cutaneous CD4 +  small/medium T‐cell lymphoproliferative disorder (Primary cutaneous CD4 +  small/medium T‐cell lymphoma)

Indolent T‐cell lymphoproliferative disorder of the gastrointestinal tract

Primary cutaneous acral CD8 +  T‐cell lymphoma

Breast implant‐associated anaplastic large cell lymphoma

Nomenclature changesa

Systemic EBV+ T‐cell lymphoma in childhood (systemic EBV+ T‐cell lymphoproliferative disorder)

Hydroa vacciniforme lymphoproliferative disorder (Hydroa vacciniforme‐like lymphoma)

Enteropathy‐associated T‐cell lymphoma (Enteropathy‐associated T‐cell lymphoma type 1)

Monomorphic epitheliotropic intestinal T‐cell lymphoma (Enteropathy‐associated T‐cell lymphoma type 2)

Primary cutaneous CD4 +  small/medium T‐cell lymphoproliferative disorder (Primary cutaneous CD4 +  small/medium T‐cell lymphoma)

• Acute
• Lymphoma-type
• Chronic
• Smoldering
• 1—dysregulation of the JAK/STAT pathway due to gene mutations which are common to various aggressive and indolent neoplasmss

If the staging system involves specific and characteristic findings and features:

According to the [staging system + reference], there are [number] stages of [malignancy name] based on the [finding1], [finding2], and [finding3]. Each stage is assigned a [letter/number1] and a [letter/number2] that designate the [feature1] and [feature2].

OR

The staging of [malignancy name] is based on the [staging system].

OR

There is no established system for the staging of [malignancy name].

Updated World Health Organization Classification

• T-cell and putative NK-cell neoplasms
  • Precursor T-cell neoplasm: precursor T-acute lymphoblastic leukemia/LBL
  • Peripheral T-cell lymphomas and NK-cell lymphomas

Peripheral T Cell Lymphoma

The WHO classification of T-cells NHL categorize them into Precursor T-cell neoplasm and Peripheral T-cell lymphomas/NK-cell neoplasms. The last one can be further subdivided into:

• T-cell prolymphocytic leukemia
• T-cell large granular lymphocytic leukemia
• Chronic lymphoproliferative disorder of NK cells
• Aggressive NK-cell leukemia
• EBV positive T-cell lymphoproliferative disorders of childhood
  • Systemic EBV positive T-cell lymphoproliferative disease of childhood
  • Hydroa vacciniforme-like lymphoma
• Adult T-cell lymphoma/leukemia
• Extranodal T-/NK-cell lymphoma, nasal type
• Enteropathy-associated T-cell lymphoma
• Hepatosplenic T-cell lymphoma
• Subcutaneous panniculitis-like T-cell lymphoma
• Mycosis fungoides
• Sézary syndrome
• Primary cutaneous CD30 positive T-cell lymphoproliferative disorders
  • Primary cutaneous anaplastic large cell lymphoma
  • Lymphomatoid papulosis
• Primary cutaneous peripheral T-cell lymphomas, rare subtypes
  • Primary cutaneous gamma-delta T-cell lymphoma
  • Primary cutaneous CD8 positive aggressive epidermotropic cytotoxic T-cell lymphoma
  • Primary cutaneous CD4 positive small/medium T-cell lymphoma
• Peripheral T-cell lymphoma, not otherwise characterized
• Angioimmunoblastic T-cell lymphoma
• Anaplastic large cell lymphoma, ALK positive
• Anaplastic large cell lymphoma, ALK negative