Heads up for the mucous membranes! –Recurrent oral aphthosis as first sign of large granular cell lymphocyte leukemia

Abstract

Clonal disorders of large granular lymphocytes (LGLs) represent a spectrum of distinct lymphoproliferative disorders originating either from mature T cells (CD3+) or natural killer (NK) cells (CD3-). LGL leukemia comprises 2 to 5% of all T-cell/NK-cell malignancies and manifests itself mostly as an indolent disease. Clinical presentation is dominated by recurrent infections associated with neutropenia, anemia, splenomegaly, and autoimmune diseases, particularly rheumatoid arthritis. Recurrent oral aphthosis has also been described in the literature as the first manifestation of this entity, although this presentation is mostly associated with chronic neutropenia presented by these patients. The purpose of this article is to report a case of LGLL in which the development of ulcers were the initial clinical presentation, associated with hypogammaglobulinemia.