Non lymphoblastic T cell lymphomas with prevalent skin involvement different from mycosis fungoides or Sézary's syndrome. A retrospective study of 6 cases in Europe.
A retrospective study of 23 cases of nonlymphoblastic T cell lymphomas with no features of either mycosis fungoides (MF) or Sézary syndrome (SS) was carried out. 6 patients exhibited cutaneous (or mucous) lesions and are presented herein. There was no detectable visceral or node involvement except in 1 patient. 3 cases were immunologically proven to be of peripheral T cell origin. Except T cell lymphomas in Japan and in the Caribbean area, the frequency of prevalent skin involvement is reportedly very low in non-MF/SS non lymphoblastic T cell lymphomas. However, it is likely that this frequency has been underestimated in Western countries. The histologic features of our cases run the heterogeneous spectrum of recently described node-based forms of peripheral T cell lymphomas: 'angioimmunoblastic lymphadenopathy with dysproteinemia'-like lymphoma, large T cell lymphoma, multilobulated cell lymphoma, T zone lymphoma. The histologic pattern of skin infiltration was different from that of MF/SS. One case was initially suggestive of a pseudolymphoma and had a prolonged evolution but the other patients suffered from rapid extracutaneous involvement and fatal evolution with resistance to treatment. Cutaneous plasmacytomas were seen in 1 case. Hence, certain cutaneous T cell lymphomas exhibit histologic, immunological and developmental features similar to those of node-based peripheral T cell lymphomas but different from those of MF/SS.
Grob
J J
JJ
Service de Dermatologie, Hôpital Ste-Marguerite, Marseille, France.
Horchowski
N
N
Tubiana
N
N
Gabriel
B
B
Gastaut
J A
JA
Hassoun
J
J
Jouve
I
I
Carcassone
Y
Y
Bonerandi
J J
JJ
D000368:Aged; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008198:Lymph Nodes / Q000473:pathology; D008223:Lymphoma / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009182:Mycosis Fungoides / Q000473:pathology*; D012751:Sezary Syndrome / Q000473:pathology*; D012878:Skin Neoplasms / Q000473:pathology*; D013601:T-Lymphocytes / Q000473:pathology