The British journal of dermatology | 2021
Lymph node and visceral progression without erythroderma or blood worsening in erythrodermic cutaneous T-cell lymphoma: nine cases.
Abstract
The prognosis of erythrodermic cutaneous T-cell lymphomas (ECTL) depends upon lymph node (N), visceral (M) involvement, and blood stage (B, i.e circulating Sézary cells, identified upon peripheral blood immunophenotyping, B0: absolute count <250/mm3 , B1: 250-1000/mm3 , and B2 stage, Sézary syndrome: ≥1000/mm3 ) with a 5-year overall survival of 36% for Sézary syndrome, the most common subtype of ECTL.1 Other prognostic markers include male sex, age >60 years, an elevated rate of lactate dehydrogenase (LDH), and histologic large-cell transformation (LCT).1-3 ECTL progression is usually homogeneous and simultaneously involves all compartments of the disease. We report nine patients with B1 or B2 ECTL displaying an unusually discrepant outcome profile with the rapid onset of N or M involvement contrasting with the lack of progression of cutaneous and blood involvement.