A Case Of Angio-immunoblastic Lymphadenopathy Associated With A Long Spontaneous Remission, Retrobulbar Neuritis, A Clonal Rearrangement Of Thet-cell Receptor Gamma Chain Gene And An Unusual Marrow Infiltration
European Journal of Haematology 41(3): Sep 1988; 295-301
View Original Source →Case Details
Personal Characteristics
A man
Clinical Characteristics
Retrobulbar neuritis, marrow infiltration with a lymphoid cell of novel surface-marker phenotype, pulmonary emboli
Remission Characteristics
Spontaneous remission that lasted 18 years, another remission was induced by prednisolone
Treatment & Mechanisms
Proposed Remission Mechanisms
Development of a lymphoid stem cell clone with no tcr gene rearrangement, 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
Clinical Treatment
Prednisolone
Additional Notes
He died of pulmonary emboli 21 years after his original presentation. Proliferation in the marrow of the original clone finally supervened.