A case of angioimmunoblastic lymphadenopathy associated with a long spontaneous remission, retrobulbar neuritis, a clonal rearrangement of the T-cell receptor gamma chain gene and an unusual marrow infiltration.
A man with angioimmunoblastic lymphadenopathy entered a spontaneous remission that lasted 18 yr, 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 yr 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.
Matthews
J H
JH
Department of Haematology, St. Mary's Hospital Medical School, London, U.K.
Smith
N A
NA
Foroni
L
L
D000328:Adult; D001853:Bone Marrow / Q000473:pathology*; D002999:Clone Cells / Q000378:metabolism / Q000473:pathology; D015334:Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor; D005803:Genes, Immunoglobulin; D006801:Humans; D007119:Immunoblastic Lymphadenopathy / Q000150:complications* / Q000235:genetics / Q000473:pathology; D008297:Male; D009902:Optic Neuritis / Q000150:complications* / Q000235:genetics / Q000473:pathology; D012075:Remission, Spontaneous; D013601:T-Lymphocytes / Q000378:metabolism / Q000473:pathology