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Published on: October 30, 2016
Blastic NK-cell lymphomas (agranular CD4+CD56+ hematodermic neoplasms): a review
Tony Petrella1, Martine Bagot, Rein Willemze
1Centre de Pathologie, Department of Pathology, Centre Hospitalo-Universitaire, Dijon, France.
Insights
Blastic natural killer (NK) cell lymphoma, a rare hematologic neoplasm, is reviewed. Evidence suggests these tumors may originate from plasmacytoid dendritic cells, not NK cells.
Area of Science:
- Hematology
- Oncology
- Dermatology
Background:
- Blastic natural killer (NK) cell lymphoma, also known as CD4+CD56+ hematodermic neoplasm, is a rare and aggressive hematologic malignancy.
- First described in 1994, its cellular origin has been debated, with initial hypotheses suggesting a natural killer cell lineage.
Purpose of the Study:
- To review the clinical, histopathologic, and phenotypic characteristics of blastic natural killer (NK) cell lymphoma.
- To discuss diagnostic criteria for this entity.
- To present evidence supporting a plasmacytoid dendritic cell origin.
Main Methods:
- Literature review of published cases.
- Analysis of a series of 30 cases from French and Dutch cutaneous lymphoma study groups.
Main Results:
- The review consolidates major clinical, histopathologic, and phenotypic features of the disease.
- Diagnostic criteria are provided.
- Data supporting a plasmacytoid dendritic cell origin for the tumor cells are presented.
Conclusions:
- Blastic natural killer (NK) cell lymphoma is a distinct entity with specific diagnostic criteria.
- Current evidence suggests a lineage of plasmacytoid dendritic cells rather than natural killer cells for this neoplasm.
Abstract:
Blastic natural killer (NK) cell lymphoma (also termed CD4+CD56+ hematodermic neoplasm) is a recently described entity, with the first case reported in 1994. It was suggested initially that the disease originates from NK cells. Since 1994, single cases and a few small series have been published. In this review, data from the literature and a series of 30 cases from the French and Dutch study groups on cutaneous lymphomas are discussed. The major clinical, histopathologic, and phenotypic aspects of the disease and diagnostic criteria and data suggesting a plasmacytoid dendritic cell origin for the tumor cells are provided.
