This is an aggressive disorder that responds poorly to alkylating agents or irradiation of the mediastinum or spleen. Of 55 patients with T-PLL, five patients had complete remission and 45 percent had a good response. The clinical picture is variable, but patients typically present with frequent infections or bleeding. The spleen may be palpably enlarged, and the marrow can either be normal or show focal collections of lymphocytes. The marrow replacement is never so extensive that it would account for the presenting cytopenia.
Some patients have high serum titers of rheumatoid factor.
T Cell Large Granular Lymphocyte Leukemia Patients with T-LGL leukemia may also have rheumatoid arthritis. Clonality can be established by T cell receptor gene rearrangement. Therapy for T-LGL leukemia is usually directed at the neutropenia and the infections that follow. G-CSF may be useful. There are no reports of effective therapy.