TherapySézary syndrome and seronegative polyarthritis: Treatment with extracorporeal photochemotherapy☆,☆☆,★,★★,♢
Section snippets
Case report
A 50-year-old white man presented with erythroderma and pruritus beginning in 1981 that had been treated with local and systemic steroids. In 1982 he had hepatomegaly develop and, in 1984, generalized lymphadenopathy was diagnosed. The diagnosis of Sézary syndrome was made on the basis of these clinical findings, including lymphocytosis with typical Sézary cells, and was supported by biopsy specimens of the skin and a lymph node showing histologic and immunohistochemical features compatible
Therapy and results
ECP was performed every 4 weeks on 2 consecutive days following standard protocols as previously described.16 High-dose prednisone (50 mg/d at the beginning) and chlorambucil (4 mg/day) therapy that had not led to any improvement was tapered for 4 months and stopped. Under ECP the patient showed a clinical improvement (Fig 1, B) within 6 months of therapy. After 1 year he went into complete remission of both diseases (Fig 2, B). This clinical response was confirmed by normalization of the
Discussion
We describe a patient with therapy-resistant Sézary syndrome and concurrent seronegative polyarthritis who was successfully treated with ECP. For selected cases of Sézary syndrome8, 17 ECP is recommended as a treatment of choice without the risk of associated severe side effects. For other forms of cutaneous T-cell lymphoma, ECP is an effective therapeutic alternative often in combination with other immunomodulating therapy such as interferons.7, 9, 10, 11 In addition, clinical studies have
Acknowledgements
We acknowledge the outstanding clinical and scientific contribution of Dr Walter Macheiner who died in an accident during a hiking tour in the Austrian Alps in the summer of 2000.
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Cited by (0)
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Funding source: None.
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Conflict of interest: None identified.
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Reprint requests: Robert M. Knobler, MD, Division of Special and Environmental Dermatology, Department of Dermatology, University of Vienna Medical School, Währinger Gürtel 18-20, A-1090 Vienna, Austria. E-Mail: [email protected].
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† Deceased.
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