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1998 Presentations for the Poster and Vignette Sessions
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PRIMARY CUTANEOUS T CELL LYMPHOMA, PLEOTROPHIC MEDIUM AND SMALL CELL TYPE. Matthew G. Erickson, MD, PhD. University of Wisconsin Hospitals and Clinics, Madison, WI.

Primary cutaneous lymphomas vary widely in clinical presentation, histology, immuno­phenotype, and prognosis. After primary GI lymphomas, primary cutaneous lymphomas represent the second most common group of nonHodgkin's extra nodal lymphomas; annual incidence is perhaps 1:100,000.

Unlike primary nodal lymphomas of the same histologic subtype, primary

cutaneous lymphomas have highly characteristic clinical and histologic features. In addition, there are found differences in the presence of specific translocations and in the expression of oncogenes, viral sequences, and adhesion receptors.

We present the case of a seventeen year old white female who was originally diagnosed with eczema but proved to have the pleomorphic small and medium cell variant of cutaneous T cell lymphoma. Unlike patients reported in a European series (60% survival at ten years), our patient's disease has been extremely aggressive: She has progressed in spite of multiple courses of chemotherapy with ACE (Adriamycin, cisplatin, and etoposide), CHOP, and fludarabrine; remissions to date have been measured in weeks. Since the time of diagnosis, she has developed evidence of extra cutaneous involvement.

In an effort to obtain a durable remission and improve quality of life, we are now exploring relatively new treatment modalities. These include extracorporeal photopheresis therapy, interferon therapy, and fusion protein technology; each will be discussed in some detail. Unfortunately, our patient is nestled in a small subset of a small subset; there is little clinical data available to guide selection of optimal treatment.

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