Characterization of T-Cell Large Granular Lymphocyte Leukemia Associated with Sjogren’s Syndrome—An Important but Underrecognized Association
Section snippets
Patients
Kaplan Medical Center is an academic hospital located in central Israel, which serves as a referral center for a population of about 250,000. Over the years 1987 to 2002, 48 cases of T-LGL leukemia had been diagnosed and treated at our center by 1 of the authors (A.B.). All patients met the accepted criteria of T-LGL leukemia (1, 2, 11). Only 1 patient was treated for LGL leukemia before being evaluated for SS. She received prednisone and then cyclosporine A, and at the time of diagnosis of SS,
Patient’s Clinical Characteristics
Twenty-two patients with T-LGL leukemia reported sicca symptoms (3 unsolicited; all others, only when questioned) and 21 were studied. One patient who had both positive sicca symptoms and tests declined to participate. All but 2 were women. In 8 patients (6 women, 2 men) SS was ruled out by the combination of normal eye and salivary flow tests and a normal labial biopsy. The clinical characteristics of patients who had SS were not significantly different from those of patients in whom SS was
Discussion
This is the first reported series that actively examines T-cell LGL leukemia patients for the presence of SS. The association was previously recognized but did not extend further than a few case reports (5, 6). Only 2 patients were reported in any detail (6) and the others were either mentioned very briefly (11) or had SS secondary to RA (5). The finding that over a quarter of 48 patients with T-LGL leukemia studied had SS (Table 1) was unexpected and striking. This remarkable prevalence is
Acknowledgments
We acknowledge with thanks the expert pathological analysis done by Drs N. Sokolovski and M. Huszar (KMC, Rehovot), as well as the help of Dr V. Barak (Hadassah University Hospital, Jerusalem), Dr I. Lichman, and L. Bassous (KMC) in the laboratory studies.
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