Aggressive natural killer cell leukaemia/lymphoma in two patients with lethal midline granuloma
- 12 March 1994
- journal article
- Published by Wiley in British Journal of Haematology
- Vol. 86 (3), 659-662
- https://doi.org/10.1111/j.1365-2141.1994.tb04804.x
Abstract
Summary. We report two patients with leukaemic proliferations of large granular lymphocytes. The immunophenotype study showed that the leukaemic cells were positive for CD2, CD38, CD56 and anti-HLA-DR monoclonal antibodies and negative for other T-cell (CD3, CD4, CD8) and B-cell markers (CD19, CD20 and surface immunoglobulins). The clinical course was acute and a diagnosis of aggressive natural killer cell leukaemia/lymphoma was made. No clonal rearrangements of either Cβ T-cell receptor or JH immunoglobulin genes were found. Functional studies done in one patient demonstrated non-restricted cytotoxic activity after activation with IL-2. Lethal midline granuloma had been previously diagnosed in both patients. A possible relationship between this entity and the natural killer cell leukaemia is discussed.Keywords
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