Clinical features associated with transformation of cerebriform T‐cell lymphoma to a large cell process

Abstract

Some patients with cerebriform T-cell lymphoma (CTCL) undergo morphologic transformation to a large cell lymphoma. From a series of 113 patients with CTCL, 22 patients were identified with transformed CTCL. Stages of involvement at diagnosis were: I (seven), II (four), III (four), IV (seven). Nine patients had transformation at the initial diagnosis while the median time from diagnosis to transformation in the other 13 patients was 16 months (range: 3 months-6 years). Thirteen had transformation extracutaneously: lymph nodes (eight), central nervous system (two), and other extranodal sites (three). T cell markers were identified in all cases; of 15 cases with complete phenotypes, there were eight T-helper, three T-suppressor, and four aberrant T phenotypes. Serology for human T-leukemia virus-I (HTLV-I) was negative in eight patients tested. Median survival from diagnosis was 27 months compared to 53 months in 53 patients without transformation (p = 0.003). Despite combination chemotherapy in 12 patients following transformation, median survival after transformation was 12 months and only 7 months with extracutaneous disease. The likelihood of transformation could not be predicted by the initial histology, immunophenotype, or stage of disease.

Cutaneous T‐cell lymphoma: 2011 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 [189][190][191][192][193], 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 [7]. 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 [143].…”

Long-term outcomes of patients with advanced-stage cutaneous T-cell lymphoma and large cell transformation

“…CTCL to be between 11% and 23%. [5][6][7][8] The incidence of transformation in our CTCL database is just 6.1% using the same diagnostic criteria. Transformation was much more common in patients with a more advanced stage of disease.…”

“…[5][6][7][8][9][10] LCT is pathologically characterized by the morphologic change of small-to mediumsized cerebriform cells to a large cell variant, 11 accompanied by clinically aggressive disease. Large cells are lymphocytes that are at least 4 times greater in size than a small lymphocyte.…”

“…[5][6][7][8] Transformation occurs more commonly in advanced-stage disease than early-stage disease (ESD) 7 and is especially common in tumor lesions (LCT in 55% of patients with tumor-stage disease in one study). 10 The prognosis of LCT is significantly worse than in classic MF.…”

Expression of Cytotoxic Proteins by Neoplastic T Cells in Mycosis Fungoides Increases with Progression from Plaque Stage to Tumor Stage Disease

“…Because all but these two follow-up biopsies had been obtained from patients without evidence of extracutaneous disease at the time of biopsy the clinical stage was determined only by the type of skin lesions, defined as the presence of patches and plaques covering more than 10% of the skin surface (T2) or the presence of tumors (T3). Because previous studies demonstrated unequivocally that skin tumors showing blastic transformation have a significantly worse prognosis than tumors without blastic transformation 23,24 within the T3 category, distinction was made between tumors with and tumors without blastic transformation, defined by the presence of more than 50% blast cells. Therefore, we will refer primarily to the type of skin lesion (patches/plaques, tumors without, and tumors with blastic transformation).…”