Sezary syndrome with pulmonary involvement: a case report
Keywords:Mycosis fungoides, Sezary syndrome, Lung
AbstractMycosis fungoides and sezary syndrome are the type of cutaneous T cell lymphoma characterized by localization of malignant T-lymphocytes in the skin at presentation. Mycosis fungoides (MF) is characterized by an epidermotropic skin infiltrate of atypical CD4-positive helper T-cell clones, and sezary syndrome (SS), is characterized by erythroderma and leukemia. Here we are presenting a case of 50 year old male who presented with extensive skin lesions along with breathlessness since 25 days and fever, was misdiagnosed as end stage sarcoidosis on CT chest, but later proved to be pulmonary involvement of sezary syndrome. The objective of our study is to describe our experience identifying lung involvement by CTCL on the basis of clinical and radiographic findings.
Girardi M, Heald PW, Wilson LD. The pathogenesis
of mycosis fungoides. N Engl J Med. 2004;350:1978–88.
Willemze R, Jaffe ES, Burg G. WHO-EORTC classification for cutaneous lymphomas. Blood. 2005;105: 3768–85.
Weinstock MA, Gardstein B. Twenty-year trends in the reported incidence of mycosis fungoides and associated mortality. Am J Public Health. 1999;89:1240–4.
Rubin DL, Blank N. Rapid pulmonary dissemination in mycosis fungoides simulating pneumonia. A case report and review of the literature. Cancer. 1985;56:649–51.
Scarisbrick JJ, Whittaker S, Evans AV. Prognostic significance of tumour burden in the blood of patients with erythrodermic primary cutaneous T cell lymphoma. Blood. 2001;97:624.
Zackheim HS, Amin S, Kashani-Saket M. Prognosis in cutaneous T cell lymphoma by skin stage: Long term survival in 489 patients. J Am Acad Dermatol. 1999;40:418–25.