Popular science stickers: peripheral T-cell lymphoma (PTCL)

PTCL overview

Peripheral T-cell lymphoma (peripheral T cell lymphomas, PTCLs) is a group originated in the thymus after T cell or NK cell maturation heterogeneous disease

Prognostic assessment

The International Prognostic Index (IPI) is used to assess the risk of PTCLs and is related to overall survival (OS).

Ann-Arbor staging

The staging system for lymphoma uses the Ann-Arbor (Revised Cotswolds) staging system:

Stage I: Invades a lymph node area (I), or invades a single extra-lymph node organ or site (IE);

Stage Ⅱ: Invading two or more lymph node areas on one side of the diaphragm (Ⅱ) or a localized invasion of an extranodal organ or part (ⅡE);

Stage Ⅲ: The affected lymph node area is on both sides of the diaphragm (Ⅲ) or a localized extranodal organ or part (ⅢE) or spleen (ⅢS) or both (ⅢES);

Stage IV: Diffuse or disseminated invasion of one or more extranodal organs, with or without lymph node invasion

Common subtypes of PTCL

Peripheral T-cell lymphoma is not a disease, but a group of diseases.

Several common subtypes of PTCLs that are currently of clinical concern are:

1

Peripheral T Cell Lymphoma (Not Otherwise Specified, PTCL-NOS) is the most common histological subtype

Vascular immune mother cell T-cell lymphoma (T-Angioimmunoblastic the Cell Lymphoma, AITL)

Anaplastic Large-Cell Lymphoma (ALCL), ALCL can be divided into systemic anaplastic, primary skin anaplastic and breast implant-related anaplastic

Peripheral T Cell Lymphoma (Not Otherwise Specified, PTCL-NOS) is the most common histological subtype

1

1

Peripheral T Cell Lymphoma (Not Otherwise Specified, PTCL-NOS) is the most common histological subtype

Peripheral T Cell Lymphoma (Not Otherwise Specified, PTCL-NOS) is the most common histological subtype

2

Vascular immune mother cell T-cell lymphoma (T-Angioimmunoblastic the Cell Lymphoma, AITL)
.
The occurrence of AITL is related to Epstein-Barrvirus (EBV), human herpes virus (HHV), human immunodeficiency virus (HIV), bacterial and fungal infections , and these viral infections may be related to immune disorders
.

2

2

Vascular immune mother cell T-cell lymphoma (T-Angioimmunoblastic the Cell Lymphoma, AITL)
.
The occurrence of AITL is related to Epstein-Barrvirus (EBV), human herpes virus (HHV), human immunodeficiency virus (HIV), bacterial and fungal infections , and these viral infections may be related to immune disorders
.

Vascular immune mother cell T-cell lymphoma (T-Angioimmunoblastic the Cell Lymphoma, AITL)
.
The occurrence of AITL is related to Epstein-Barrvirus (EBV), human herpes virus (HHV), human immunodeficiency virus (HIV), bacterial and fungal infections , and these viral infections may be related to immune disorders
.

3

Anaplastic Large-Cell Lymphoma (ALCL), ALCL can be divided into systemic anaplastic, primary skin anaplastic and breast implant-related anaplastic
.
Systemic anaplastic enlargement can be divided into ALK-positive and ALK-negative
.
The main feature of ALCL is that tumor cells are highly heterogeneous and almost all express CD30 antigen.
The immunophenotype can be T cell expression or non-T non-B cell expression
.

3

3

Anaplastic Large-Cell Lymphoma (ALCL), ALCL can be divided into systemic anaplastic, primary skin anaplastic and breast implant-related anaplastic
.
Systemic anaplastic enlargement can be divided into ALK-positive and ALK-negative
.
The main feature of ALCL is that tumor cells are highly heterogeneous and almost all express CD30 antigen.
The immunophenotype can be T cell expression or non-T non-B cell expression
.

Anaplastic Large-Cell Lymphoma (ALCL), ALCL can be divided into systemic anaplastic, primary skin anaplastic and breast implant-related anaplastic
.
Systemic anaplastic enlargement can be divided into ALK-positive and ALK-negative
.
The main feature of ALCL is that tumor cells are highly heterogeneous and almost all express CD30 antigen.
The immunophenotype can be T cell expression or non-T non-B cell expression
.

The incidence of the main subtypes of PTCLs varies from region to region.
PTCLs originating from lymph nodes are the most common in Europe.
Among them, PTCLs-NOS accounted for 34%, AITL accounted for 28%, ALK+ALCL accounted for 6%, and ALK-ALCL accounted for 9%.
PTCLs that originate outside the lymph nodes are more common in Asia.
In Japan, adult T cell leukemia/lymphoma (Adult T cell leukemia/lymphoma) related to Human T lymphocyte virus I (HTLV-1) lymphoma, ATLL), while Asian non-Japanese countries are mainly extranodal natural killer or T cell lymphoma (Extranodel nature killer or T cell lymphoma, ENKTCL).
In Northern Europe, due to human leukocyte antigen haplotype-related abdominal diseases The rate is higher, so enteropathy-associated T cell lymphoma (EATL) occurs more frequently [5]
.

2016 WHO T cell tumor classification

(※: Difference from the 2008 WHO classification; boldface: provisional classification) [6]

references

[1]James O.
Armitage.
The aggressive peripheral T-cell lymphomas: 2017, Am J Hematol.
2017;92:706–715.

[2] Li Xiaoqiu, et al.
Distribution of lymphoma subtypes in China: analysis of 10002 cases of domestic multicenter cases.
Diagnostics Theory and Practice.
2012;11(2):111-115

[3] Standards for diagnosis and treatment of lymphoma (2020 edition)

[4] Chinese Society of Clinical Oncology (CSCO) Lymphoma Diagnosis and Treatment Guidelines 2021 Edition