Transformation of Mycosis Fungoides/Sezary Syndrome: Clinical Characteristics and Prognosis

Abstract

The occurrence of large cell transformation has been well documented in a subgroup of patients with mycosis fungoides/Sezary syndrome (MF/SS). However, because of the rarity of MF/SS, little is known about the influence of clinicopathologic features in predicting large cell transformation and about outcome in the transformed cases. We evaluated all patients with MF/SS who were registered in our clinic during the study period and for whom pathologic slides for review were available or could be obtained. Disease was classified as transformed if biopsy showed large cells (≥4 times the size of a small lymphocyte) in more than 25% of the infiltrate or if they formed microscopic nodules. Twenty-six patients with transformation were identified from a total of 115 evaluable cases with a diagnosis of MF/SS. The actuarial cumulative probability of transformation reached 39% in 12 years. The median time from diagnosis of MF/SS to transformation was 12 months (range, 0 to 128 months). Thirty-one percent of all patients with stage IIB-IV disease at presentation eventually transformed versus 14% of those with stage I-IIA (P= .03), with transformation being especially common in patients with tumors (T3), 46% of whom transformed. Combining elevated β2 microglobulin and lactic dehydrogenase (neither elevated v one or both elevated) was also predictive for transformation (P = .009). The median survival from initial diagnosis of MF/SS for the transformed patients was 37 months versus 163 months for the untransformed group (P = .0029). The median survival from transformation was 19.4 months (range, 2+ to 138 months). The following characteristics were associated with an inferior survival in transformed patients: (1) early transformation (<2 years from the diagnosis v ≥2 years; P = .011) and (2) advanced stage (IIB-IV v I-IIA; 2-year survival, 23% v 86%;P = .0035). We conclude that MF/SS patients with stages IIB-IV disease and, in particular, those with tumors have a high incidence of large-cell transformation. Patients with transformation have a relatively poor survival, especially if transformation occurs early (within 2 years) in the course of disease or if they are staged as IIB or higher. © 1998 by The American Society of Hematology.

Cutaneous T‐cell lymphoma: 2017 update on diagnosis, risk‐stratification, and management

“…In addition to staging, male gender, increasing age, an elevated LDH and the folliculotropic variant of MF were also independently associated with poorer overall and disease-specific survival. In contrast to previous reports highlighting the aggressive clinical course associated with large cell transformation, [141][142][143][144][145] defined as the presence of large, atypical lymphocytes comprising at least 25% of the total lymphoid infiltrate, large cell transformation was not an independent predictor of overall or disease-specific survival, but was associated with a higher risk (hazard ratio 3.32) of disease progression. 6 Given the importance of the TNMB classification in risk stratification and defining disease burden, the ISCL/EORTC recommends its use in defining the initial, maximum and current burden of disease, which will ultimately play an important role in the selection of either skin-directed or systemic therapies.…”

Transformed mycosis fungoides: clinicopathological features and outcome

“…In our series median delay from the initial diagnosis of MF to documentation of the onset of large-cell transformation was 3AE6 years (range 1-115 months) (12 months in Diamandidou et al 13 ) (Table 1). We also observed that fatal cases had a relatively shorter delay from initial diagnosis of MF to transformation.…”

“…Chromosomal rearrangement, 10 expression of CD25 antigen (interleukin 2 receptor), 11 Recent studies in a group of 26 13 cases and in a series of 45 14 patients with T-MF showed conflicting data about clinical features associated with a poor outcome. In the first study, 13 poor survival is associated with early transformation (< 2 years after initial diagnosis of MF) and advanced clinical stage at the time of T-MF (IIB-IV vs. I-IIA). In the second study, 14 older age ( ‡ 60 years) and extracutaneous invasion (stage IV) were found to be correlated with a poor prognosis.…”

A case of Sezary's syndrome associated with granulomatous lesions, myelodysplastic syndrome and transformation into CD30-positive large-cell pleomorphic lymphoma

“…8 T-cell receptor (TCR) genes are clonally rearranged in the peripheral blood, skin and lymph nodes. 9,10 Unusual and rare features of SS include its association with monoclonal gammopathies or myeloma [11][12][13][14] or other B-cell proliferations, 15 development of granulomatous lesions, [16][17][18] association with myelodysplastic syndrome (MDS), [19][20][21] transformation into a high-grade malignant lymphoma, [22][23][24][25] and development of various secondary neoplasms. 26 We report a patient with SS, who developed several unusual features during the course of the disease, namely granulomatous lesions harbouring dominant T-cell clone, transformation into CD30-positive largecell pleomorphic T-cell lymphoma, and MDS.…”