CD3+, CD4-, CD8-, TCR(alpha)(beta)-, TCR(gamma)(delta)+ Granular Lymphocyte Proliferative Disorder without Lymphocytosis and Clinical Symptoms

Granular lymphocyte-proliferative disorder is characterized by a proliferation of large granular lymphocytes (LGLs). It is often associated with neutropenia, rheumatoid arthritis (RA), and pure red cell aplasia (PRCA). Phenotypic analysis has demonstrated that in most cases, the LGLs show a clonal rearrangement of the TCR(alpha)(beta) rearrangement. We are reporting a patient with TCR(gamma)(delta) LGL proliferation without clinical findings and lymphocytosis. The patient showed an expansion of the CD3+, CD16+, CD56+, and CD57+ LGL populations which involved coexpression of TCR(gamma)(delta) with TCR J(gamma) and J(delta)1 gene rearrangement. Autoimmune manifestations, including RA and PRCA, have not appeared and the results of laboratory examinations have not changed for 1 year after the diagnosis.