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Aggressive natural killer cell leukemia in an adult with establishment of
an NK cell line
LA Fernandez, B Pope, C Lee and E Zayed
There have been many reports of cases in which chronic increases in the
numbers of natural killer (NK) cells have been reported. Whether this is
reactive or neoplastic in nature has been debated. We report the first case
of an aggressive NK cell leukemia in an adult with establishment of an NK
cell line. A 70-year-old man had two spontaneous episodes of jejunal
perforation and one month later developed a severe febrile illness with
moderate splenomegaly. Hemoglobin was 13.1 g/L, and WBC count was 1.8 X
10(9)/L with 2% large granular lymphocytes (LGLs). Platelet count was 143 X
10(9)/L; prothrombin time (PT) and partial thromboplastin time (PTT) were
normal. Bone marrow was infiltrated with 25% to 30% LGLs; serum lysozyme
was normal. Serum LDH was initially 1,191 U/L and rose to 6,408 (normal 240
to 525 U/L). Ten days later, the WBC count increased to 99.9 X 10(9)/L with
70% LGL cells; the PT and PTT increased, and the platelet count dropped. No
bacterial or viral cause of fever was identified. The cells from peripheral
blood were LGLs that stained positively for acid phosphatase. All of the
LGLs reacted with a monoclonal antibody reactive with NK cells (LEU-11b).
Functionally, the patient's peripheral blood mononuclear cells (PBMs)
demonstrated 100 times more lytic activity against K562 tumor cell lines
than did normal PBMs. The patient's PBMs were propagated in vitro. The
cultured cells showed the morphological, cytochemical, immunological, and
functional characteristics of NK cells. In addition, partial trisomy
involving chromosome 1 q with duplication in regions of q21 through q31 was
observed in all metaphases analyzed. The extra chromosome 1q with
duplication in regions q21 through q31 was translocated to the p- terminal
of chromosome 5. One percent to 5% of normal PBMs comprise NK cells; in
most cases, leukemias arise from normal phenotypic counterparts. This case
demonstrated that aggressive NK cell leukemia may occur in adults. In
addition, the chromosomal abnormalities suggest that this is not a reactive
process but a malignancy.
Volume 67,
Issue 4,
pp. 925-930,
04/01/1986
Copyright © 1986 by The American Society of Hematology
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