Angio-immunoblastic Lymphadenopathy With Spontaneous Remission In An Over 80-year-old Female Patient

Abstract

A man with angio-immunoblastic lymphadenopathy entered a spontaneous remission that lasted 18 years, ending in retrobulbar neuritis followed by a generalized relapse. After another remission was induced by prednisolone he developed marrow infiltration with a lymphoid cell of novel surface-marker phenotype. His lymph node cells demonstrated a clonal rearrangement of the T-cell receptor gamma chain (TcR gamma) genes whereas the TcR beta genes were heterogeneously rearranged. The marrow cells did not contain the rearranged TcR gamma gene. He died of pulmonary emboli 21 years after his original presentation. We propose a model whereby the original event was the development of a lymphoid stem cell clone with no TcR gene rearrangement. Subsequently, a sub-clone bearing the TcR gamma gene rearrangement proliferated in the lymph nodes and further heterogeneous rearrangement of the TcR beta genes occurred within this subclone. Proliferation in the marrow of the original clone finally supervened.