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
• Hydroa vacciniforme lymphoproliferative disorder
• Enteropathy‐associated T‐cell lymphoma
• Monomorphic epitheliotropic intestinal T‐cell lymphoma
• Primary cutaneous CD4 +  small/medium T‐cell lymphoproliferative disorder

• 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

T cell prolymphocytic leukemia

T cell large granular lymphocytic leukemia

Aggressive NK cell leukemia

Systemic EBV+ T cell lymphoma of childhood*

Hydroa vacciniforme-like lymphoproliferative disorder*

Adult T cell leukemia/lymphoma

Extranodal NK/T cell lymphoma, nasal type

Enteropathy-associated T cell lymphoma

Monomorphic epitheliotropic intestinal T cell lymphoma*

Hepatosplenic T cell lymphoma

Subcutaneous panniculitis-like T cell lymphoma

Mycosis fungoides

Sézary syndrome

Primary cutaneous CD30+ T cell lymphoproliferative disorders

Lymphomatoid papulosis

Primary cutaneous anaplastic large cell lymphoma

Primary cutaneous γδ T cell lymphoma

Peripheral T cell lymphoma, NOS

Angioimmunoblastic T cell lymphoma

Anaplastic large cell lymphoma, ALK+

Anaplastic large cell lymphoma, ALK–*

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