Therapy
Sézary syndrome and seronegative polyarthritis: Treatment with extracorporeal photochemotherapy,☆☆,,★★,

https://doi.org/10.1067/mjd.2003.11Get rights and content

Abstract

We describe a patient with therapy-resistant cutaneous T-cell lymphoma, Sézary syndrome variant, in association with concurrent polyarthritis and vitiligo, who was successfully treated with extracorporeal photochemotherapy (ECP). The combination of Sézary syndrome with seronegative rheumatoid arthritis is rare. In our patient the T-cell lymphoma was refractory to standard treatments that included psoralen—UVA, lymph node irradiation, and polychemotherapy. ECP has been shown to be effective in the treatment of selected cases of Sézary syndrome. There is a strong suggestion that ECP as a monotherapy can provide a significant benefit for other T-cell-mediated diseases including rheumatoid arthritis. In spite of a disease duration of 10 years, a very low CD8 cell count (2% of lymphocytes), a very high CD4 cell count (94%), and multiple unsuccessful chemotherapeutic trials before initiation of ECP, our patient achieved a long-lasting complete remission of both diseases with normalization of the CD4+ and CD8+ T-lymphocyte subsets. Concurrent developing vitiligo was unaffected by ECP. (J Am Acad Dermatol 2003;48:220-6.)

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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      Citation Excerpt :

      In 1988, the use of ECP was approved by the U.S. Food and Drug Administration as standard therapy for the treatment of patients with advanced refractory CTCL [2]. Later, ECP was used in the treatment of several autoimmune T-cell-mediated diseases such as pemphigus vulgaris [3,4], systemic sclerosis [5,6], rheumatoid arthritis [7,8], Crohn's disease [9,10] and multiple sclerosis [11]. Moreover, ECP was introduced for the treatment of solid organ allograft rejection [12–16], acute and chronic graft versus host disease (GvHD) post-allogenic hematopoietic stem cell transplantation (HSCT) refractory to the conventional immunosuppressive therapy [17–23].

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    Funding source: None.

    ☆☆

    Conflict of interest: None identified.

    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].

    ★★

    † Deceased.

    0190-9622/2003/$30.00 + 0

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