Sézary syndrome (SS) is one of the most common clinical variants of primary cutaneous T cell lymphomas (CTCL). Advanced SS have a poor prognosis and may be beneficially affected by a multi-disciplinary approach. We report a 70-year-old man presented with a 4-year history of SS diagnosed on the basis of clinical and pathologic features of CTCL with leukemic involvement and treated by other medical officers over the years with extracorporeal photophoresis (ECP) and alpha interferon with only limited partial clinical responses. His condition gradually deteriorated coming to our observation with a poor performance status (ECOG: 3), intractable itching erythroderma involving the whole body, palmar and plantar hyperkeratosis, supraclavicular, axillary and inguinal lymph node enlargement, confirmed by PET-CT scan. He had a white blood cell count of 9.520/mL with 87% of clonal T-cell receptor gene rearranged lymphocytes CD3+, CD4+, CD5+, CD7-, CD8-, identical with those detected in neck lymph node, in the skin and bone marrow (BM). According to the current WHO–EORTC classification, all diagnostic criteria matched with SS, 4th stage. The patient was first treated with 4 doses of pentostatin without any response and 2 months later with alemtuzumab at a dose of 30 mg two times a week. The patient concomitantly received cotrimoxazole, acyclovir and itraconazole prophylaxis. Cytomegalovirus (CMV) infection was monitored weekly using polymerase chain reaction (PCR). After the first week of alemtuzumab, itching disappeared and after 2 weeks of treatment a marked improvement of erythroderma, concomitant with complete clearing of circulating Sezary cells, was observed, associated 2 weeks later with almost complete regression of lymphadenopathies. Alemtuzumab had to be discontinued at the fourth week (total dose 270 mg) due to cytomegalovirus DNA reactivation, successfully resolved after standard dose gancyclovir treatment. Two months post alemtuzumab discontinuation, reappearance of 17,1/mL circulating Sezary cells prompted us to start again ECP (two session per month in the first year and then two session every two month in the second year) leading to a progressive tapering of circulating Sezary cells and complete resolution of disease signs and symptoms. This case report further supports the efficacy and safety of alemtuzumab plus ECP in inducing prolonged responses in patients with refractory late stage Sezary syndrome.

EFFICACY OF ALEMTUZUMAB PLUS EXTRACORPOREAL PHOTOPHERESIS IN ADVANCED STAGE SEZARY SYNDROME: A CASE REPORT

SELLERI, Carmine
2013

Abstract

Sézary syndrome (SS) is one of the most common clinical variants of primary cutaneous T cell lymphomas (CTCL). Advanced SS have a poor prognosis and may be beneficially affected by a multi-disciplinary approach. We report a 70-year-old man presented with a 4-year history of SS diagnosed on the basis of clinical and pathologic features of CTCL with leukemic involvement and treated by other medical officers over the years with extracorporeal photophoresis (ECP) and alpha interferon with only limited partial clinical responses. His condition gradually deteriorated coming to our observation with a poor performance status (ECOG: 3), intractable itching erythroderma involving the whole body, palmar and plantar hyperkeratosis, supraclavicular, axillary and inguinal lymph node enlargement, confirmed by PET-CT scan. He had a white blood cell count of 9.520/mL with 87% of clonal T-cell receptor gene rearranged lymphocytes CD3+, CD4+, CD5+, CD7-, CD8-, identical with those detected in neck lymph node, in the skin and bone marrow (BM). According to the current WHO–EORTC classification, all diagnostic criteria matched with SS, 4th stage. The patient was first treated with 4 doses of pentostatin without any response and 2 months later with alemtuzumab at a dose of 30 mg two times a week. The patient concomitantly received cotrimoxazole, acyclovir and itraconazole prophylaxis. Cytomegalovirus (CMV) infection was monitored weekly using polymerase chain reaction (PCR). After the first week of alemtuzumab, itching disappeared and after 2 weeks of treatment a marked improvement of erythroderma, concomitant with complete clearing of circulating Sezary cells, was observed, associated 2 weeks later with almost complete regression of lymphadenopathies. Alemtuzumab had to be discontinued at the fourth week (total dose 270 mg) due to cytomegalovirus DNA reactivation, successfully resolved after standard dose gancyclovir treatment. Two months post alemtuzumab discontinuation, reappearance of 17,1/mL circulating Sezary cells prompted us to start again ECP (two session per month in the first year and then two session every two month in the second year) leading to a progressive tapering of circulating Sezary cells and complete resolution of disease signs and symptoms. This case report further supports the efficacy and safety of alemtuzumab plus ECP in inducing prolonged responses in patients with refractory late stage Sezary syndrome.
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/11386/4241053
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