Abstract
Mycosis fungoides (MF) and Sezary Syndrome (SS) represent the most common subtypes of primary Cutaneous T-cell lymphoma (CTCL). Patients with advanced MF and SS have a poor prognosis leading to an interest in the development of new therapies with targeted mechanisms of action and acceptable safety profiles. In this review we focus on such novel strategies that have changed the treatment paradigm of this rare malignancy.
Highlights
Cutaneous T-cell lymphomas (CTCL) are a rare heterogeneous group of non-Hodgkin lymphomas derived from skin-homing mature T-cells
In 2007, a revised staging system of Mycosis fungoides (MF) and Sezary Syndrome (SS) by the International Society for Cutaneous Lymphomas (ISCL)/European Organization for Research and Treatment of Cancer (EORTC) was proposed, incorporating stratification of early stage (TI/T2) into patch alone and both patch and plaque disease, as well as detailed histologic and molecular classification of lymph node and peripheral blood involvement leading to a uniform and standardized staging and classification system [2]
In another attempt to reduce the incidence of treatment related opportunistic infections, a phase II study by Zinzani et al assessed the impact of a reduced dose (10 mg three times per week for four weeks) schedule in 10 patients with pretreated cutaneous/peripheral T-cell lymphoma [90]
Summary
Cutaneous T-cell lymphomas (CTCL) are a rare heterogeneous group of non-Hodgkin lymphomas derived from skin-homing mature T-cells. While the number of patients in this report is small, it demonstrates that SQ administration of alemtuzumab is well tolerated despite advanced age and poor performance status In another attempt to reduce the incidence of treatment related opportunistic infections, a phase II study by Zinzani et al assessed the impact of a reduced dose (10 mg three times per week for four weeks) schedule in 10 patients with pretreated cutaneous/peripheral T-cell lymphoma [90]. Phase I/II studies in indolent B-cell lymphoma demonstrated that when local radiation to expose tumor antigens was combined with intratumoral injection of TLR-9 activating CpG-oligodeoxynucleotide (to serve as an in situ vaccination maneuver), meaningful clinical responses were observed in 4 of 15 patients (27%) with one attaining CR and safe tolerability [108]. Given the chronicity of CTCL treatment and its established potential in multiple myeloma, its role as a maintenance regimen following systemic chemotherapy (gemcitabine or doxil) in advanced stage CTCL patients is being explored in a pivotal EORTC initiated phase III trial
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