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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
Author(s) -
Matthews J. H.,
Smith N. A.,
Foroni L.
Publication year - 1988
Publication title -
european journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.904
H-Index - 84
eISSN - 1600-0609
pISSN - 0902-4441
DOI - 10.1111/j.1600-0609.1988.tb01196.x
Subject(s) - t cell receptor , gene rearrangement , clone (java method) , bone marrow , biology , lymph node , pathology , t cell , immunology , medicine , cancer research , gene , immune system , genetics
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 (TcRy) genes whereas the TcRβ genes were heterogeneously rearranged. The marrow cells did not contain the rearranged TcRγ 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 TcRγ gene rearrangement proliferated in the lymph nodes and further heterogeneous rearrangement of the TcRβ genes occurred within this subclone. Proliferation in the marrow of the original clone finally supervened.