A rare case of marginal zone lymphoma in a 15-year old ataxia telangiectasia patient with massive bone marrow involvement and a challenging nodal diagnosis

Abstract

This is a report of a very rare case of marginal zone lymphoma in a 15-years old male with Ataxia Telangiectasia. Besides being a rare diagnosis within the pediatric age group, this case exhibited a true challenge not only from diagnostic point of view but also regarding management plans. Marginal zone lymphoma is an indolent disease in which transformation is extremely rare particularly the pediatric subtype. However, there isn't enough data regarding marginal zone lymphoma in a sitting of immunodeficiency conditions had been published so far. The case described here showed some features pointing to more aggressive disease with massive bone marrow involvement and areas of focal transformation unlike the subtle pattern of infiltration that is usually seen in cases of marginal zone lymphoma. Another interesting finding in our case is the presence of a clone of CD4/CD8 double positive T-cells of moderate size (12%) with restricted expansion of Vb1 region. The significance of this clone is uncertain as it could be a transient reactive clone or a pre-malignant one. Keywords: Ataxia telangiectasia, Marginal zone lymphoma, Low grade lymphoma