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Published on: December 4, 2018
Chronic natural killer cell lymphocytosis
1Division of Hematology and Internal Medicine, Mayo Clinic, Rochester, Minnesota 55905, USA.
Insights
Chronic Natural Killer (NK) cell lymphocytosis (CNKL) often presents with severe cytopenias or vasculitis, responding to immunosuppression. This nonprogressive condition mirrors the indolent course of T-LGL leukemia.
Area of Science:
- Hematology
- Immunology
- Oncology
Background:
- Chronic proliferations of Natural Killer (NK) cells (CD3- CD16+) are identified via large granular lymphocyte (LGL) excess and flow cytometry.
- T-cell large granular lymphocyte (T-LGL) leukemia is a related indolent disorder associated with neutropenia and rheumatoid arthritis.
Purpose of the Study:
- To describe the clinical presentation and long-term outcomes of patients diagnosed with chronic NK cell lymphocytosis (CNKL).
Main Methods:
- Retrospective analysis of 14 patients with CNKL.
- Median follow-up of 4 years.
- Clinical data including presentation, treatment response, and disease course were reviewed.
Main Results:
- The majority of patients presented with severe cytopenias or vasculitic syndromes.
- Immunosuppressive therapy was effective in managing these manifestations.
- Other symptoms included fever and arthralgias.
- The disease course was generally nonprogressive.
Conclusions:
- Chronic NK cell lymphocytosis (CNKL) typically presents with significant cytopenias or vasculitis, often responsive to immunosuppressive treatment.
- CNKL shares a similar nonprogressive, indolent clinical course with T-LGL leukemia.
Abstract:
Chronic proliferations of natural killer (NK) cells (CD3- CD16+) are identified initially by detecting large granular lymphocyte (LGL) excess in a peripheral blood smear and subsequent lymphocyte immunophenotyping by flow cytometry. A related disease, T-LGL leukemia, has an indolent clinical course with chronic neutropenia and a close association with rheumatoid arthritis. Herein are described the clinical presentation and long-term clinical course of patients with chronic NK cell lymphocytosis (CNKL). The majority of the 14 patients followed up for a median of 4 years presented with severe cytopenias or vasculitic syndromes that were responsive to immunosuppressive therapy. Other manifestations included fever and arthralgias. In general, the disease was nonprogressive and had a course similar to that of T-LGL leukemia.
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