[Dermatomyositis associated with hepatosplenic gamma delta T-cell lymphoma].
BACKGROUND
Peripheral T cell lymphomas are a heterogeneous group of post-thymic, mature lymphoid malignancies, accounting for approximately 10-15% of all non-Hodgkin's lymphomas. A rare entity within this group is represented by hepatosplenic T cell lymphoma, characterized by primary extranodal disease with infiltration of the liver and the spleen and by expression of the T cell receptor gamma delta chain.


METHODS
A 64-year old man with dermatomyositis developed rapid-onset paraparesia and deafness. Cerebrospinal fluid analysis revealed large granular lymphomatous cells with CD3+ CD4- CD8- CD7+ CD16- CD56- surface antigens, expressing the gamma delta T-cell receptor. There was no evidence of skin or bone marrow infiltration by lymphoma or any other involvement. This is the first report of dermatomyositis associated with a gamma delta T-cell lymphoma (GDTL). Moreover, primitive and isolated meningeal involvement of such lymphomas has never been described before.


CONCLUSIONS
GDTL should be added to the differential list of neoplasia associated with dermatomyositis. Physiopathological mechanisms implicated in the neurological involvement of such lymphomas need to be elucidated.
Chandesris
M-O
MO
Service de médecine interne, CHU de La Conception, 147, boulevard Baille, 13385 Marseille cedex 05, France.
Crétel-Durand
E
E
Jean
R
R
Rey
J
J
Figarella-Branger
D
D
Sainty
D
D
Durand
J-M
JM
D003882:Dermatomyositis / Q000209:etiology*; D006801:Humans; D008113:Liver Neoplasms / Q000175:diagnosis* / Q000378:metabolism; D016399:Lymphoma, T-Cell / Q000175:diagnosis* / Q000378:metabolism; D008297:Male; D008875:Middle Aged; D010257:Paraneoplastic Syndromes / Q000175:diagnosis*; D016692:Receptors, Antigen, T-Cell, gamma-delta / Q000378:metabolism; D013160:Splenic Neoplasms / Q000175:diagnosis* / Q000378:metabolism
D016692:Receptors, Antigen, T-Cell, gamma-delta