Abstract
The case is reported of a 45 year old woman with the rare leukaemia of natural killer cell large granular lymphocyte (NK/ LGL) type. Cytometric analysis of leukaemic blasts showed that they were positive for CD2, CD38, and CD56 antigens but negative for a series of antigens including CD3, CD7, CD16, and HLA-DR. Rearrangements of the beta T cell receptor, and heavy and kappa immunoglobulin genes were not detected and neither were chromosomal abnormalities. Leukaemic blasts developed NK cytotoxicity. The patient failed to respond to aggressive chemotherapy and died three months after diagnosis. The lack of expression of HLA-DR is an extraordinary characteristic of this case, as all cases of acute NK cell leukaemias described to date expressed HLA-DR. The immunophenotype observed in the NK cell leukaemic blasts may represent the counterpart of a hypothetical normal cell precursor in an early stage of ontogenic NK cell development.
Full text
PDF
Selected References
These references are in PubMed. This may not be the complete list of references from this article.
- Imamura N., Kusunoki Y., Kawa-Ha K., Yumura K., Hara J., Oda K., Abe K., Dohy H., Inada T., Kajihara H. Aggressive natural killer cell leukaemia/lymphoma: report of four cases and review of the literature. Possible existence of a new clinical entity originating from the third lineage of lymphoid cells. Br J Haematol. 1990 May;75(1):49–59. doi: 10.1111/j.1365-2141.1990.tb02615.x. [DOI] [PubMed] [Google Scholar]
- Jacobs R., Stoll M., Stratmann G., Leo R., Link H., Schmidt R. E. CD16- CD56+ natural killer cells after bone marrow transplantation. Blood. 1992 Jun 15;79(12):3239–3244. [PubMed] [Google Scholar]
- Miller J. S., Alley K. A., McGlave P. Differentiation of natural killer (NK) cells from human primitive marrow progenitors in a stroma-based long-term culture system: identification of a CD34+7+ NK progenitor. Blood. 1994 May 1;83(9):2594–2601. [PubMed] [Google Scholar]
- Parrado A., Rodriguez-Fernandez J. M., Casares S., Noguerol P., Plaza E., Parody R., Espigado I., de Blas J. M., Garcia-Solis D. Generation of LAK cells in vitro in patients with acute leukemia. Leukemia. 1993 Sep;7(9):1344–1348. [PubMed] [Google Scholar]
- Robertson M. J., Ritz J. Biology and clinical relevance of human natural killer cells. Blood. 1990 Dec 15;76(12):2421–2438. [PubMed] [Google Scholar]
- Soler J., Bordes R., Ortũno F., Montagud M., Martorell J., Pons C., Nomdedeu J., Lopez-Lopez J. J., Prat J., Rutllant M. Aggressive natural killer cell leukaemia/lymphoma in two patients with lethal midline granuloma. Br J Haematol. 1994 Mar;86(3):659–662. doi: 10.1111/j.1365-2141.1994.tb04804.x. [DOI] [PubMed] [Google Scholar]
- Sánchez M. J., Spits H., Lanier L. L., Phillips J. H. Human natural killer cell committed thymocytes and their relation to the T cell lineage. J Exp Med. 1993 Dec 1;178(6):1857–1866. doi: 10.1084/jem.178.6.1857. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Tefferi A., Li C. Y., Witzig T. E., Dhodapkar M. V., Okuno S. H., Phyliky R. L. Chronic natural killer cell lymphocytosis: a descriptive clinical study. Blood. 1994 Oct 15;84(8):2721–2725. [PubMed] [Google Scholar]
- Weil-Hillman G., Fisch P., Prieve A. F., Sosman J. A., Hank J. A., Sondel P. M. Lymphokine-activated killer activity induced by in vivo interleukin 2 therapy: predominant role for lymphocytes with increased expression of CD2 and leu19 antigens but negative expression of CD16 antigens. Cancer Res. 1989 Jul 1;49(13):3680–3688. [PubMed] [Google Scholar]