Sezary syndrome

Extranodal NK/T-cell lymphoma, nasal type Enteropathy-type T-cell lymphoma Hepatosplenic gamma-delta T-cell lymphoma Subcutaneous panniculitis-like T-cell lymphoma Mycosis fungoides/sezary syndrome... 

Mycosis fungoides (MF). This mature T cell lymphoma presents in the skin and is composed of small and medium-sized lymphocytes with cere-briform nuclei, which infiltrate the epidermis and dermis. Sezary syndrome is an aggressive variant of MF characterized cliniopathologically by erythroderma, lymphadenopathy, and circulating malignant cells (W9). 

Cutaneous T-Cell Lymphoma Mycosis Fungoides and Sezary Syndrome... 

Of 22 patients, median age 61 years, who had received a median of three previous types of therapy for mycosis fungoides or Sezary syndrome and were given alemtuzumab in increasing doses (from 3 to 30 mg three times a week for 12 weeks), 11 had no infectious complications, one had fatal pulmonary aspergillosis 2.5 months after the end of treatment, and another contracted fatal Mycobacterium pneumonia 10 months after the end of treatment (7). 

Lundin J, Hagberg H, Repp R, Cavallin-Stahl E, Freden S, Juliusson G, Rosenblad E, Tjonnfjord G, Wiklund T, Osterborg A. Phase 2 study of alemtuzumab (anti-CD52 monoclonal antibody) in patients with advanced mycosis fungoides/Sezary syndrome. Blood 2003 101(ll) 4267-72. 

DTncan M, Souteyrand P, Bignon YJ, Fonck Y, Roger H. Hydantoin-induced cutaneous pseudolymphoma with clinical, pathologic, and immunologic aspects of Sezary syndrome. Arch Dermatol 1992 128(10) 1371. ... 

Leach JW, Pham T, Diamandidis D, George JN. Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) following treatment with deoxycoformycin in a patient with cutaneous T cell lymphoma (Sezary syndrome) A case report. Am J Hematol 1999 61(4) 268-70. 

An illness resembling Sezary syndrome was seen after combined treatment with chloroquine 150 mg every third day (total seven tablets) plus Fansidar one tablet weekly (total six tablets) the symptoms comprised fever, diarrhea, erythroderma, jaundice, lymphadenopathy, and hepatosplenomegaly (SEDA-12, 696). 

Sezary syndrome (1981) Ferme F +, Leuk Res 5, 169 Side effects... 

Follicular lymphoma Mycosis fungoides/Sezary syndrome ... 

Cladribine, a purine nucleoside analog, with antineoplas-tic properties (0.09 mg/kg daily by continuous IV infusion for 7 days), is indicated in the treatment of active hairy cell leukemia. In addition, it has been used in advanced cutaneous T-cell lymphomas, chronic lymphocytic leukemia, non-Hodgkin s lymphomas, acute myeloid leukemias, autoimmune hemolytic anemia, mycosis fungoides, or Sezary syndrome (see also Figure 15). 

Sezary syndrome Peripheral blood RIO Mature T-cell line with inducer/helper phenotype yields and responds to interleukin-2... 

Successful monotherapy of cutaneous T cell lymphoma has been reported with etretinate [56] as well as with isotretinoin [24, 41, 57, 79] and with arotinoid Ro 13-6298 [78]. However, a combination of etretinate/PUVA therapy (RePUVA) appeared to be more effective [59]. Jones and coworker [39] reported on the successful treatment of mycosis fungoides and Sezary syndrome with a combination of etretinate and electron beam therapy. 

Hoting E, Paul E, Plewig G (1986) Treatment of rosacea with isotretinoin. Int J Dermatol 25 660-663 Imcke E, Ruszczak Zb, Mayer-da-Silva A, Detmar M, Orfanos CE (1991) Cultivation of human dermal microvascular endothelial cells in vitro Inununocytochemical and ultrastructural characterization and effect of treatment with three synthetic retinoids. Arch Dermatol Res 283 149-157 Jansen T, Plewig G (1997) Advances and perspectives in acne therapy. EurJ Med Res 28 321-334 Jones G, McLean J, Rosenthal D, Roberts J, Sander DN (1992) Combined treatment with oral etretinate and electron beam therapy in patients with cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome). J Am Acad Dermatol 26 960-967... 

F. Braut-Boucher, J. Font, J. Pichon, Y. Paulin, M. Boukhelifa, M. Aubery and C. Derappe, T lymphocytes from sezary syndrome patients express betal integrins whose beta(l-6)-branched N-linked oligosaccharides reflect their adhesive capacity. Leuk. Res., 22 (1998) 947-952. 

A meta-analysis of 19 studies in patients at all stages of cutaneous T-cell lymphoma showed an overall response rate higher than 55% using ECP. ECP was also effective in the treatment of Sezary syndrome, showing an overall response rate of 43% [216]. 

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