Coexistent T-cell lymphoblastic lymphoma and an atypical myeloproliferative disorder associated with t(8;13)(p21;q14)

This report describes a neoplasm exhibiting both lymphoid and myeloid differentiation associated with an acquired balanced translocation between chromosomes 8 and 13 occurring in a 10-year-old boy. Serial lymph node biopsies revealed the presence of both lymphoblastic lymphoma and an atypical myeloproliferative disorder within the same node. Immunophenotyping was consistent with the presence of an immature T-cell population within the nodal biopsy specimens. Cytogenetic analysis of the bone marrow and lymph node biopsy specimens revealed a unique translocation, t(8;13) (p21;q14). Molecular analysis revealed rearrangement of the immunoglobulin heavy chain gene and germline configuration of the T-cell receptor gene. The patient had a poor response to classical T-cell acute lymphocytic leukemia/lymphoma therapy and was changed to a myeloid leukemia protocol with good response. He underwent bone marrow transplantation but died soon after of overwhelming graft-versus-host disease. We found five similar cases in the literature, suggesting the existence of a subset of mixed lymphoid/myeloid disorders with 8p;13q translocations, in which the clinical picture is dictated by the myeloid element.