Blastic Natural Killer Cell Lymphoma/Leukemia

Abstract

Only a few blastic natural killer (NK) cell leukemias and lymphomas have been reported. As such, the clinicopathologic spectrum of this disease is incompletely understood. We report 7 cases of blastic NK cell lymphoma/leukemia. All patients were men, 5 white and 2 Arab American. All cases exhibited blastic morphologic features and were CD3- and CD56+ with germline T-cell receptor genes. Five cases were CD4+ and involved the skin. Both CD4- cases never involved the skin. Other markers of mature NK cells such as CD16, CD57, and TIA-1 were expressed infrequently. Three cases were CD33+. One CD33+ case had a clonal rearrangement of the immunoglobulin heavy chain gene. Skin and lymph nodes were involved most often, with frequent evolution to a leukemic phase. Initial responses to therapy were achieved in most patients, but the tumors invariably recurred.

Complete remission in advanced blastic NK‐cell lymphoma/leukemia in elderly patients using the hyper‐CVAD regimen

“…Blastic NK-cell lymphoma/leukemia displays striking extramedullary spread to the skin and subcutaneous tissues [2,16]. Cutaneous lesions may have a purple bruise-like or petechial appearance [9,17], as demonstrated in the present patients (Fig. 1).…”

“…Foremost is the fact that plasmacytoid dendritic cells do not express CD56. CD56 is a salient feature of blastic NKcell lymphomas and is expressed very early on in committed NK precursor cells [17,24]. Furthermore, the failure of neoplastic cells to express perforin and granzyme B in Chaperot et al's study is likely a reflection of the immature nature of blastic NK-cells, rather than an indication of their non-NK-cell derivation.…”

“…Finally, none of the features described by the authors are specific for the dendritic lineage. Therefore, origination from an early committed NK precursor that has yet to develop a mature cytotoxic phenotype currently appears to be most plausible [17]. The expression of T/NK-cell associated cytoplasmic CD3 and other similar markers, as well as the dim, blast-like expression of CD45 (Fig.…”

“…Twenty-five patients were alive at reporting. Follow-up data was available for 23 of these, with a survival range of 5-98 months, a mean of 23.8 months, and a median of 17 months [5,8,10,13,15,17,21,25,[31][32][33]. The longest survivors (76 and 98 months) were two patients in Feuillard et al's [15] series who received allogeneic bone marrow transplants.…”

“…The longest survivors (76 and 98 months) were two patients in Feuillard et al's [15] series who received allogeneic bone marrow transplants. Only seven of the 23 previously reported patients who were alive at the time of reporting (and for whom follow-up data was available) were free of disease [10,15,17,19]. Furthermore, one of these seven was suffering from pulmonary aspergillosis [17], while another patient's follow-up was limited to 5 months [15].…”