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Treatment of T-LGL with Cyclosporine A-Long Term Follow-Up

Treatment of T-LGL with Cyclosporine A-Long Term Follow-Up

Blood 100(11): Abstract No 2635, November 16

T-LGL, a rare lymphoproliferative disorder caused by an expansion of activated cytotoxic T lymphocytes (CTLs), presents with the morphological appearance of circulating large granular lymphocytes and is associated with life threatening cytopenias and various autoimmune phenomena. The abnormal CTLs are believed to cause the cytopenia by suppressing hematopoiesis. Unlike the aggressive NK-cell variant of LGL leukemia, T-LGL responds to a variety of immunosuppressive agents: methotrexate, purine analogs and cyclosporine. We report the results of a trial of cyclosporine A (CyA) in the treatment of T-LGL patients with cytopenias (NIH-protocol 96-H-0142). Twenty-five consenting subjects (11 men, 14 women; median age 60, range (21-79)) with T-LGL and cytopenia had their diagnosis confirmed by morphology and flow cytometry. TCR gene-rearrangements could be detected in 19/25 by PCR or Southern blot. Ten subjects had coincident myelodysplasia (RA=6, RAEB=3, RARS=1). Several patients had immune dysregulation suggested either by history (15/25), abnormal immunoglobulin levels (12/25), elevated ANA (8/22) or elevated RF (8/22). Patients were treated with CyA 10mg/kg/day for at least three months, with subsequent taper. CyA was stopped in two patients, one on day 14 for serious neurological toxicity and the other on day 161 for erythroid hypoplasia. Follow up is between 36 and 72 months. Seven patients died and 18 survive. Five deaths were in nonresponders. About half the patients responded to CyA (13/25). Of these, six had complete normalization of blood counts. Most patients with a sustained response are dependent on low doses of CyA. Interestingly, one subject achieved a PR and two converted to a CR only after adding erythropoietin. Three out of the four responses seen in LGL-MDS had the RA subtype of MDS. Several possible factors to predict response were explored. Neither LGL count before or after therapy, marrow infiltration by LGL, marrow cellularity nor the presence of MDS had significant predictive value for response. In conclusion, continuous suppression of CTLs by low dose CyA maintains prolonged responses and is a reasonable treatment strategy for T-LGL.

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Accession: 035980624

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