Premium
SEZARY'S SYNDROME
Author(s) -
Scamps R. A.,
O'Neill B. J.,
Constance T. J.
Publication year - 1972
Publication title -
medical journal of australia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.904
H-Index - 131
eISSN - 1326-5377
pISSN - 0025-729X
DOI - 10.5694/j.1326-5377.1972.tb46706.x
Subject(s) - erythroderma , mycosis fungoides , medicine , pathology , lymph node biopsy , lymph node , disease , peripheral blood , biopsy , reticulum cell sarcoma , dermatology , infiltration (hvac) , lymphoma , immunology , physics , thermodynamics
Two cases are described of generalized erythroderma, malignant lymphomatous infiltration of the skin and large, bizarre, mononuclear cells circulating in the peripheral blood. These are the classical features of Sézary's syndrome, and our cases appear to be the first examples of this rare condition published in Australia. These two cases support the concept that this condition is not a distinct, slowly progressive, clinical entity, but simply a rare syndrome which may be very occasionally seen in the spectrum of the malignant lymphoreticular disorders Our first patient survived for five years. An initial lymph‐node biopsy suggested a diagnosis of Hodgkin's disease; midway through his illness the peripheral blood findings changed to those of chronic lymphocytic leukaemia, and terminally he had a typical clinical picture of mycosis fungoides. The second patient, at the time when he had all the features of Sézary's syndrome, also had evidence of reticulum‐cell sarcoma.