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Epinephrine test and plasma elastase as diagnostic tools in a patient with CD3+ large granular lymphocyte
J Seebach1, B Schleiffenbaum, R Rüegg
1Department of Internal Medicine, University Hospital Zurich, Switzerland.
Insights
Large granular lymphocyte (LGL) proliferation can cause neutropenia. Epinephrine stimulation tests may aid diagnosis, and steroid treatment improved neutrophil counts by reducing LGL-mediated destruction.
Area of Science:
- Immunology
- Hematology
Background:
- Large granular lymphocyte (LGL) proliferation involves cytotoxic lymphocyte expansion and is linked to neutropenia.
- CD3+ LGL proliferation presents a diagnostic challenge, particularly when circulating LGL counts are normal or low.
Observation:
- An epinephrine stimulation test (EST) significantly elevated CD3+, CD8+, CD57+ LGL counts in a patient with CD3+ LGL proliferation.
- Steroid therapy led to a decrease in plasma elastase, a marker of neutrophil destruction, from 162 to 40 µg/L.
- Neutrophil counts increased from 0.14 to 1.0 x 10(9)/L following steroid treatment.
Findings:
- The EST appears to be a valuable diagnostic tool for identifying LGL proliferation, especially in cases with low circulating LGL numbers.
- Neutropenia in CD3+ LGL proliferation is likely caused by LGL-mediated neutrophil destruction.
- Steroid treatment effectively reduced markers of neutrophil destruction and restored neutrophil counts.
Implications:
- This study suggests EST as a potential diagnostic aid for LGL proliferation.
- Findings support the mechanism of LGL-mediated neutrophil destruction in neutropenia associated with LGL proliferation.
- Steroid therapy demonstrates efficacy in managing neutropenia linked to CD3+ LGL proliferation.
Abstract:
Large granular lymphocytes (LGL) proliferation is characterized by expansion of cytotoxic lymphocytes and associated with neutropenia. In a case of CD3+ LGL-proliferation the epinephrine stimulation test (EST) induced a striking elevation of CD3+, CD8+, CD57+ LGL in peripheral blood from 2.7 x 10(9)/l to 20 x 10(9)/l and might be an additional diagnostic tool in patients with normal or low absolute numbers of circulating LGL. After treatment with steroids, plasma elastase--a marker of neutrophil destruction--decreased from 162 to 40 micrograms/l (normal < 47 micrograms/l) which correlated well with a simultaneous increase in peripheral neutrophil counts from 0.14 to 1.0 x 10(9)/l. This finding supports the hypothesis that neutropenia in CD3+ LGL proliferation is due to neutrophil destruction, possibly mediated by LGL.
