TY - JOUR
T1 - Chronic natural killer cell lymphocytosis
T2 - A descriptive clinical study
AU - Tefferi, Ayalew
AU - Li, Chin Yang
AU - Witzig, Thomas E.
AU - Dhodapkar, Madav V.
AU - Okuno, Scott H.
AU - Phyliky, Robert L.
N1 - Copyright:
Copyright 2020 Elsevier B.V., All rights reserved.
PY - 1994/10/15
Y1 - 1994/10/15
N2 - We review the clinical manifestations and long-term outlook of patients with chronic natural killer (NK) cell lymphocytosis. After reviewing more than 1,500 peripheral blood lymphoid flow cytometry reports and molecular genetics data from patients with suspected large granular lymphocyte (LGL) proliferation, we identified 10 patients (median age at diagnosis, 60 years; range, 35 to 76 years; male:female ratio, 3:2) with persistent (greater than 6 months) increase in phenotypically determined NK cells (CD3-CD16+). Southern blot analysis performed on 9 patients showed no clonal T-cell receptor gene rearrangements. Disease duration was measured from time of initial recognition of LGL or NK cell excess (greater than 40% of the lymphocyte fraction). Clinical data from these 10 patients were compared with those from 68 patients with T-cell LGL (T-LGL) leukemia. Currently, all patients are alive (median disease duration, 5 years; range, 0.8 to 8 years). Associated disease manifestations included pure red blood cell aplasia, recurrent neutropenia, recurrent neutropenic sepsis, and vasculitic syndromes, all of which were responsive to immunosuppressive therapy. No patient had palpable lymphadenopathy or splenomegaly. Compared with the patients with T-LGL leukemia, patients with chronic NK cell leukemia had similar lymphocyte counts, associated conditions, treatment responses, and survival but had less neutropenia and anemia.
AB - We review the clinical manifestations and long-term outlook of patients with chronic natural killer (NK) cell lymphocytosis. After reviewing more than 1,500 peripheral blood lymphoid flow cytometry reports and molecular genetics data from patients with suspected large granular lymphocyte (LGL) proliferation, we identified 10 patients (median age at diagnosis, 60 years; range, 35 to 76 years; male:female ratio, 3:2) with persistent (greater than 6 months) increase in phenotypically determined NK cells (CD3-CD16+). Southern blot analysis performed on 9 patients showed no clonal T-cell receptor gene rearrangements. Disease duration was measured from time of initial recognition of LGL or NK cell excess (greater than 40% of the lymphocyte fraction). Clinical data from these 10 patients were compared with those from 68 patients with T-cell LGL (T-LGL) leukemia. Currently, all patients are alive (median disease duration, 5 years; range, 0.8 to 8 years). Associated disease manifestations included pure red blood cell aplasia, recurrent neutropenia, recurrent neutropenic sepsis, and vasculitic syndromes, all of which were responsive to immunosuppressive therapy. No patient had palpable lymphadenopathy or splenomegaly. Compared with the patients with T-LGL leukemia, patients with chronic NK cell leukemia had similar lymphocyte counts, associated conditions, treatment responses, and survival but had less neutropenia and anemia.
UR - http://www.scopus.com/inward/record.url?scp=0028116298&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0028116298&partnerID=8YFLogxK
U2 - 10.1182/blood.v84.8.2721.bloodjournal8482721
DO - 10.1182/blood.v84.8.2721.bloodjournal8482721
M3 - Article
C2 - 7919384
AN - SCOPUS:0028116298
SN - 0006-4971
VL - 84
SP - 2721
EP - 2725
JO - Blood
JF - Blood
IS - 8
ER -