Sézary syndrome and seronegative polyarthritis: Treatment with extracorporeal photochemotherapy

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 arthri...

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Veröffentlicht in:Journal of the American Academy of Dermatology 2003-02, Vol.48 (2), p.220-226
Hauptverfasser: Macheiner, Walter, Jantschitsch, Christian, Graninger, Winfried, Pálóczy, Katalin, Bálint, Géza, Marschalkó, Márta, Kainberger, Franz, Breier, Friedrich, Knobler, Robert M.
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container_issue 2
container_start_page 220
container_title Journal of the American Academy of Dermatology
container_volume 48
creator Macheiner, Walter
Jantschitsch, Christian
Graninger, Winfried
Pálóczy, Katalin
Bálint, Géza
Marschalkó, Márta
Kainberger, Franz
Breier, Friedrich
Knobler, Robert M.
description 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.)
doi_str_mv 10.1067/mjd.2003.11
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Myelofibrosis ; Lymphocyte Count ; Male ; Medical sciences ; Middle Aged ; Photopheresis ; Radiography ; Sezary Syndrome - complications ; Sezary Syndrome - drug therapy ; Sezary Syndrome - immunology ; Skin Neoplasms - complications ; Skin Neoplasms - drug therapy ; Skin Neoplasms - immunology ; Tumors of the skin and soft tissue. 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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. 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Myelofibrosis</subject><subject>Lymphocyte Count</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Middle Aged</subject><subject>Photopheresis</subject><subject>Radiography</subject><subject>Sezary Syndrome - complications</subject><subject>Sezary Syndrome - drug therapy</subject><subject>Sezary Syndrome - immunology</subject><subject>Skin Neoplasms - complications</subject><subject>Skin Neoplasms - drug therapy</subject><subject>Skin Neoplasms - immunology</subject><subject>Tumors of the skin and soft tissue. 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subjects Arthritis - complications
Arthritis - diagnostic imaging
Arthritis - drug therapy
Arthritis - immunology
Biological and medical sciences
CD4 Lymphocyte Count
CD4-Positive T-Lymphocytes
CD8-Positive T-Lymphocytes
Dermatology
Hematologic and hematopoietic diseases
Humans
Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis
Lymphocyte Count
Male
Medical sciences
Middle Aged
Photopheresis
Radiography
Sezary Syndrome - complications
Sezary Syndrome - drug therapy
Sezary Syndrome - immunology
Skin Neoplasms - complications
Skin Neoplasms - drug therapy
Skin Neoplasms - immunology
Tumors of the skin and soft tissue. Premalignant lesions
title Sézary syndrome and seronegative polyarthritis: Treatment with extracorporeal photochemotherapy
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