Search

The prognosis of patients with mycosis fungoides (MF) and Sezary Syndrome (SS) varies greatly, from near normal life expectancy in patients with early stage, to a median survival of less than 2 years for those diagnosed with advanced stage disease. Initial response to treatment is almost always followed by relapse and, finally, most of patients enter a phase of advanced multi-drug resistant disease with a short life expectancy after multiple lines of treatment. Allogeneic stem cell transplantation (allo-SCT) is usually limited to patients with advanced disease resistant to multiple treatments. Retrospective registry-based studies have shown increased Non-relapse Mortality (NRM) rates in patients with poor performance status, as well as in patients treated with myeloablative conditioning regimens. Another major limitation of allo-SCT is the increased relapse rate which occurs in nearly 50% of the cases, and is probably due to the fact that only heavily pretreated patients with advanced disease are referred for allo-SCT. Due to the paucity of data, the ideal conditioning regimen which will provide the maximum therapeutic benefit without the cost of increased NRM is not currently known. In this article we present our experience with a novel regimen in the treatment of patients with advanced MF/SS.

Lymphomatoid papulosis (LyP) refers to an indolent cutaneous lymphoma. The association of prognostic clinicopathological risk factors with a second hematologic malignancy has not yet been determined. We investigated the prognostic effect of clinicopathological characteristics on the occurrence of a second lymphoma, as well as the first-line treatment, in 24 patients diagnosed with LyP using logistic regression models. We showed that lymphoma occurrence was associated with a lower mean age at onset of LyP symptoms, histological types B and C, head-located LyP lesions and a higher frequency of LyP recurrences. In multivariate analyses, histologic type A was associated with a lower risk of second lymphoma (odds ratio [OR] = 0.12, 95% confidence interval [CI] 0.014-0.98; p = 0.045) adjusting for age of LyP first symptomatology, and an important increased lymphoma-free survival rate (long-rank test; p = 0.06). Clinicopathological characteristics are important in defining the clearance or persistence of LyP lesions and may predict the occurrence of a second lymphoma.

Background: The new rexinoid bexarotene is a retinoid X receptor antagonist and immune response modifier. Although combinations of oral bexarotene and psoralen plus UVA (PUVA) have been tried in patients with all stages of mycosis fungoides (MF), the dosage of bexarotene used in these combination regimens has been variable. Objective: To assess the efficacy and safety of low-dose oral bexarotene and PUVA in patients with relapsed or treatment-refractory MF following monotherapy with multiple agents including PUVA, narrow-band UVB, interferon-a, oral bexarotene, and topical corticosteroids. Method: Combination therapy with PUVA three times weekly and low-dose oral bexarotene (150 or 300 mg/day, depending on physicians’ preference) was administered to 14 patients, seven men and seven women (median age 49.5 years, range 30-75 years), with relapsed or refractory MF stages I-III. All responders received maintenance treatment at the same bexarotene dose that induced remission until progression or unacceptable toxicity. Results: Low-dose oral bexarotene combined with PUVA was associated with an overall response rate (complete response or partial response) in 67% of the nine patients with refractory MF who completed the treatment course. Of these nine patients, four had a complete response, two had a partial response, one had stable disease, and two had progressive disease. Five patients withdrew because of hyperlipidemia. Oral bexarotene was continued as maintenance therapy in three of the four complete responders (one refused); two of these patients relapsed 2-10 months after PUVA discontinuation. Patients with partial response or stable disease received the combination for 3-5 months and were switched to another treatment regimen because of lack of further response. Therapy was fairly well tolerated. Conclusion: In a select population of patients who had not responded to at least one monotherapy for early-stage MF, a combination of low-dose oral bexarotene and PUVA was successful in achieving a satisfactory overall response rate in 67% of patients who completed the treatment course and was fairly well tolerated. Limitations of the study include the small number of patients evaluated, its retrospective nature, and the fact that patients were commenced on different bexarotene starting doses (150 or 300 mg/day), depending on physicians’ preference.