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August 1996, Vol 132, No. 8 >
Article | August 1996

Successful Treatment of Sézary Syndrome With Lymphomatous Transformation to Large Cell Lymphoma With Fludarabine Phosphate

Anthony P. Nikko; Cynthia J. Rutherford, MD; Amit G. Pandya, MD
[+] Author Affiliations

Dallas

Department of Dermatology UT Southwestern Medical Center 5323 Harry Hines Blvd Dallas, TX 75235-9069


Arch Dermatol. 1996;132(8):978-979. doi:10.1001/archderm.1996.03890320128028
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Sézary syndrome (SS), a leukemic form of cutaneous T-cell lymphoma (CTCL), is highly resistant to contemporary therapeutic modalities. A more aggressive form of disease that is even less responsive to therapy and characterized by transformation to large cell lymphoma develops in a substantial percentage of patients with SS. We describe a patient with SS that underwent lymphomatous transformation to large cell lymphoma. Treatment with the nucleoside analogue fludarabine phosphate resulted in complete remission for 14 months.

Report of a Case.  A 72-year-old white woman sought treatment because of an 11-month history of a chronic, relapsing, intensely pruritic eruption that had progressed to a generalized erythroderma. She had been treated with topical and systemic corticosteroids, oral psoralen with UV-A, etretinate, and hydroxyurea without substantial improvement. A physical examination revealed diffuse erythema and multiple, widespread, thin, scaly plaques. Erythema and keratoderma of her palms and soles were present. Laboratory data revealed

REFERENCES

1 +
Bunn PA Jr, Hoffman SJ, Norris D, Golitz LE, Aeling JL.  Systemic therapy of cutaneous T-cell lymphomas (mycosis fungoides and the Sézary syndrome) . Ann Intern Med. 1994;;121:592-602.
2 +
Wieselthier JS, Koh HK.  Sézary syndrome: diagnosis, prognosis, and critical review of treatment options . J Am Acad Dermatol. 1990;;22:381-401.
3 +
Dmitrovsky E, Matthews MJ, Bunn PA, et al.  Cytologic transformation in cutaneous T-cell lymphoma: a clinicopathologic entity associated with poor prognosis . J Clin Oncol. 1987;;5:208-215.
4 +
Greer JP, Salhany KE, Cousar JB, et al.  Clinical features associated with transformation of cerebriform T-cell lymphoma to a large cell process . Hematol Oncol. 1990;;8:215-227.
5 +
Kantarjian HM, Redman JR, Keating MJ.  Fludarabine phosphate therapy in other lymphoid malignancies . Semin Oncol. 1990;;17( (suppl 8) ):66-70.
6 +
Von Hoff DD, Dahlberg S, Hartstock RJ, Eyre HJ.  Activity of fludarabine monophosphate in patients with advanced mycosis fungoides: a Southwest Oncology Group study . J Natl Cancer Inst. 1990;;82:1353-1355.
7 +
Heald P, Rook A, Perez M, et al.  Treatment of erythrodermic cutaneous T-cell lymphoma with extracorporeal photochemotherapy . J Am Acad Dermatol. 1992;;27:427-433.

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Country-Specific Mortality and Growth Failure in Infancy and Yound Children and Association With Material Stature

Use interactive graphics and maps to view and sort country-specific infant and early dhildhood mortality and growth failure data and their association with maternal

1 +
Bunn PA Jr, Hoffman SJ, Norris D, Golitz LE, Aeling JL.  Systemic therapy of cutaneous T-cell lymphomas (mycosis fungoides and the Sézary syndrome) . Ann Intern Med. 1994;;121:592-602.
2 +
Wieselthier JS, Koh HK.  Sézary syndrome: diagnosis, prognosis, and critical review of treatment options . J Am Acad Dermatol. 1990;;22:381-401.
3 +
Dmitrovsky E, Matthews MJ, Bunn PA, et al.  Cytologic transformation in cutaneous T-cell lymphoma: a clinicopathologic entity associated with poor prognosis . J Clin Oncol. 1987;;5:208-215.
4 +
Greer JP, Salhany KE, Cousar JB, et al.  Clinical features associated with transformation of cerebriform T-cell lymphoma to a large cell process . Hematol Oncol. 1990;;8:215-227.
5 +
Kantarjian HM, Redman JR, Keating MJ.  Fludarabine phosphate therapy in other lymphoid malignancies . Semin Oncol. 1990;;17( (suppl 8) ):66-70.
6 +
Von Hoff DD, Dahlberg S, Hartstock RJ, Eyre HJ.  Activity of fludarabine monophosphate in patients with advanced mycosis fungoides: a Southwest Oncology Group study . J Natl Cancer Inst. 1990;;82:1353-1355.
7 +
Heald P, Rook A, Perez M, et al.  Treatment of erythrodermic cutaneous T-cell lymphoma with extracorporeal photochemotherapy . J Am Acad Dermatol. 1992;;27:427-433.

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