Clonal rearrangement of the T-cell receptor gamma gene associated with a bizarre lymphoproliferative syndrome.
Investigations in an unusual patient with prolonged fever, fat necrosis and haemorrhagic tendency showed widespread histiocytic phagocytosis in fat and liver biopsy specimens. Lymph nodes were also infiltrated by morphologically normal T lymphocytes predominantly expressing the OKT4 antigen. Analysis of lymph node DNA using immunoglobulin and T-cell receptor (TCR) probes showed rearrangement restricted to the gamma TCR gene combined with polyclonal beta TCR gene rearrangements, thus providing evidence of a unique lymphoproliferative disorder of T cells. The patient displayed a striking response to cytotoxic chemotherapy.
Coupe
M
M
Department of Medicine, Royal Postgraduate Medical School, London, U.K.
Foroni
L
L
Stamp
G
G
Lovatt
D
D
Barnard
M
M
Krausz
T
T
Kalofonos
H
H
Epenetos
A
A
Luzzatto
L
L
Cox
T M
TM
D004247:DNA / Q000302:isolation & purification; D015334:Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor; D006801:Humans; D007143:Immunoglobulin Heavy Chains / Q000235:genetics; D008232:Lymphoproliferative Disorders / Q000235:genetics* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D013577:Syndrome; D013601:T-Lymphocytes / Q000378:metabolism / Q000473:pathology*
D007143:Immunoglobulin Heavy Chains; D004247:DNA