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Leukemia of non-T lineage natural killer cells
W Sheridan, EF Winton, WC Chan, DS Gordon, WR Vogler, C Phillips, KF Bongiovanni and TA Waldmann
Department of Medicine, Emory University School of Medicine, Atlanta, GA
30322.
An unusual case of an aggressive leukemia of natural killer (NK) cells
occurred in a 65-year-old male. Clinical characteristics of this case
included hepatosplenomegaly, ascites, marrow infiltrate with leukemic
cells, and a WBC up to 82.8 X 10(9) before therapy. One year before his
presentation he had been noted to have a WBC of 12.1 X 10(9) with 78%
lymphocytes, and 6 months before had noted intermittent fever and weight
loss. He and his brother had well documented hereditary cold urticaria. The
patient was treated with a modification of ProMACE CYTABOM regimen and had
prompt regression of the leukemia with associated acute tumor lysis. Renal,
hepatic, and marrow failure predominated during a terminal course that
ended 22 days after therapy was commenced, and at autopsy there was no
evidence for leukemic cell infiltrate in the liver, spleen or marrow. The
leukemic cells were large granular lymphocytes by light and electron
microscopic criteria, and had the following immunophenotype: CD2+, DR+,
Leu7+, NKH1+, CD11+, CD3-, CD5-, CD4-, CD8-, CD16-. The cells displayed
high antibody- dependent cell-mediated cytotoxicity (ADCC) and NK activity,
and had a high rate of spontaneous proliferation in vitro that was not
augmented by phytohemagglutinin (PHA), concanavalin A (Con A), or pokeweed
mitogen (PWM). Southern analysis of DNA from leukemic cells revealed normal
germline arrangements for the beta and gamma chains of the T cell antigen
receptor and immunoglobulin heavy chain genes. The majority of metaphases
were clonally abnormal revealing consistent rearrangements involving extra
material attached to the long arms of chromosomes 5 and 11.
Volume 72,
Issue 5,
pp. 1701-1707,
11/01/1988
Copyright © 1988 by The American Society of Hematology
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