Biclonal Sézary syndrome with capillary leak syndrome.
Sézary syndrome is a rare, usually monoclonal proliferation of T lymphoid cells. We report a case of this form of cutaneous T cell lymphoma with highly probable biclonality of neoplastic proliferation. Biclonality in malignant lymphoma is a rare feature, until now generally reported in B cell lymphoma. Its identification by accurate immunological studies could be of importance since one of the two populations can exhibit a less differentiated phenotype requiring a more aggressive therapeutic regimen than usual. The occurrence of anasarca in our patient was probably due to capillary leak syndrome, already discussed in another case of peripheral T cell lymphoma.
Dereure
O
O
Department of Dermatology-Phlebology, CHRU Montpellier, France.
Portales
P
P
Clot
J
J
Guilhou
J J
JJ
D000368:Aged; D001402:B-Lymphocytes / Q000473:pathology; D002199:Capillary Permeability; D004487:Edema / Q000209:etiology; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D012751:Sezary Syndrome / Q000150:complications / Q000473:pathology*; D012871:Skin Diseases / Q000209:etiology; D012878:Skin Neoplasms / Q000150:complications / Q000473:pathology*; D013577:Syndrome; D013601:T-Lymphocytes / Q000473:pathology