Mycosis fungoides with large cell transformation: clinicopathological features and prognostic factors

Abstract

Summary Large cell transformation of mycosis fungoides (MF-LCT) occurs in 20–50% of advanced MF, and is generally associated with poor prognosis, although some patients have indolent disease. We sought to identify clinicopathological prognostic factors in a large number of patients with MF-LCT. We identified patients with MF-LCT treated between 1991 and 2012 at a referral centre for cutaneous lymphoma. Clinical and pathological records, and histopathological slides were reviewed. Associations of clinicopathological variables with disease-specific survival were analysed. In 51 patients with MF-LCT, factors significantly associated with shorter survival were: age >60 years (25 versus 61 months, p = 0.01), stage III/IV (25 versus 44 months, p = 0.049), high serum lactate dehydrogenase (LDH; 24 versus 53 months, p = 0.007), absent papillary dermal involvement (8 versus 30 months, p = 0.008); follicular mucin at transformation (24 versus 42 months, p = 0.007); and the absence of fibrosis at transformation (21 versus 42 months, p = 0.03). Patients presenting with transformation at diagnosis had better survival than those who started with a small cell phenotype (p = 0.02). Age >60 years was independently associated with poorer survival (HR 5.61, 95%CI 1.17–26.8, p = 0.03), and the presence of fibrosis at transformation was independently associated with improved survival (HR 0.30, 95%CI 0.09–0.97, p = 0.045). In patients with MF-LCT, clinical features (age, stage, serum LDH) are important in assessing prognosis. Additional clinical and pathological features identified in this study may also assist in prognostic stratification. Studies of larger cohorts should be performed to validate the prognostic significance of these features.

Cutaneous T-cell Lymphoma

“…Higher proportions of dermal CD30 have been found to be associated with higher stage at diagnosis and associated with an adverse prognosis although other studies have shown the converse. [42, 43]. …”

Utility of CD30, Ki‐67, and p53 in assisting with the diagnosis of mycosis fungoides with large cell transformation

“…For example, in situations where the percentage of large cells is between 20% to 30%, histological confirmation alone may be challenging. Furthermore, immunohistochemistry for CD30, while useful as a therapeutic target, is not a reliable nor a specific marker for large cell transformation …”

Survival, disease progression and prognostic factors in elderly patients with mycosis fungoides and Sézary syndrome: a retrospective analysis of 174 patients

“…Our analysis showed that the development of LCT was strongly associated with poor survival in MF/SS in both univariate and multivariate analysis. Patients who presented with LCT at diagnosis did not experience worse outcomes, and we have previously shown that patients presenting with LCT at diagnosis have better survival than those who initially present with a small cell phenotype and later develop LCT …”