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Extranodal natural-killer/t-cell lymphoma, nasal type.

Harinder Gill¹, Raymond H S Liang, Eric Tse

¹Division of Haematology, Medical Oncology and Bone Marrow Transplantation, Department of Medicine, University of Hong Kong, Queen Mary Hospital, Hong Kong.

Advances in Hematology

|January 15, 2011

Summary

Extranodal NK/T-cell lymphoma, nasal type, is a rare cancer. Treatment varies by stage, with chemotherapy and radiotherapy for early disease, and stem cell transplantation for advanced cases, offering potential cures.

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Area of Science:

Hematology
Oncology
Immunology

Background:

The World Health Organization (WHO) classifies natural killer (NK) cell neoplasms into extranodal NK/T-cell lymphoma, nasal type, and aggressive NK-cell leukemia.
Extranodal NK/T-cell lymphoma, nasal type, predominantly affects populations in the Far East and Latin America.

Purpose of the Study:

To review the classification, diagnostic hallmarks, and treatment strategies for extranodal NK/T-cell lymphoma, nasal type.
To highlight the prognostic implications of disease stage and the evolving role of stem cell transplantation.

Main Methods:

Review of World Health Organization (WHO) classification criteria for NK cell neoplasms.
Analysis of histopathological and immunophenotypical features, including angiocentricity, CD3ε, CD56, cytotoxic molecules, and Epstein-Barr virus (EBV) association.
Evaluation of current treatment modalities based on disease stage.

Main Results:

Key diagnostic markers include angiocentricity, angiodestruction, cytoplasmic CD3ε, CD56 expression, cytotoxic molecules, and EBV infection.
Early-stage localized disease is managed with chemotherapy and involved-field radiotherapy.
Advanced or disseminated disease is primarily treated with multiagent chemotherapy, with L-asparaginase-containing regimens showing promise.

Conclusions:

Treatment for extranodal NK/T-cell lymphoma, nasal type, is stage-dependent.
Chemotherapy and radiotherapy are standard for early disease.
Allogeneic hematopoietic stem cell transplantation presents a potentially curative option for advanced disease, leveraging the graft-versus-lymphoma effect.