What is your approach to initial management of patients with suspected or confirmed primary cutaneous CD8+ positive aggressive epidermotropic T- cell lymphoma (PCAECTCL)?

PCAECTCL is an exceptionally rare and clinically aggressive subtype of cutaneous T-cell lymphoma, characterized by a rapidly progressive course, ulcerated or necrotic skin lesions, and early dissemination to extracutaneous sites. Due to its rarity and lack of standardized guidelines, management is largely extrapolated from case series, expert consensus, and experience with other aggressive lymphomas. Because of its aggressive behavior, indolent skin-directed therapies are generally insufficient, and patients are treated like systemic peripheral T-cell lymphoma:

• BV+CHP (if CD30-positive), CHOP, or CHOEP (in fit patients ≤60 years). However, responses are often short-lived.
• Consider more intensive regimens (e.g., ICE, HyperCVAD, EPOCH) for r...