Turkish journal of haematology : official journal of Turkish Society of Haematology · 2025
Clinical and Prognostic Features of Erythrodermic Cutaneous T-Cell Lymphoma: A Retrospective Study of 35 Patients.
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Şanlı Hatice, Erol Mart Handan Merve, Çağlar Derya, Bayındır Aydemir Hikmet B., Öktem Ayşe, Akay Bengü Nisa
Аннотация
OBJECTIVE: Erythrodermic cutaneous T-cell lymphomas (E-CTCLs), including erythrodermic mycosis fungoides (E-MF) and Sezary syndrome (SS), are aggressive and rare variants of CTCL with overlapping clinical and pathological features. Differentiating between E-MF and SS is often challenging due to non-specific symptoms and shared diagnostic criteria. This study aimed to evaluate clinical and laboratory features, progression patterns, prognostic indicators, and survival outcomes in E-CTCL patients, comparing subgroups of E-MF, de novo SS, and secondary SS.
MATERIALS AND METHODS: A total of 35 patients with E-CTCL were analyzed (6 with E-MF, 15 with de novo SS, 14 with secondary SS). Comprehensive evaluations encompassed clinical and histopathological data, tumor-lymph node-metastasis-blood staging, flow cytometry findings, laboratory parameters, and survival outcomes. Statistical analyses included Kaplan-Meier survival estimates and Cox regression models.
RESULTS: Most patients were male (74.3%) and presented with advanced-stage disease (60%). Elevated serum lactate dehydrogenase and beta-2 microglobulin levels were common, particularly in cases of B2 blood involvement. Female sex and eosinophilia were independent predictors of mortality. Lymph node involvement was associated with rapid progression to erythroderma. No significant survival differences were observed among the E-MF, de novo SS, and secondary SS subgroups.
CONCLUSION: E-CTCL remains a diagnostic and therapeutic challenge. Female sex and eosinophilia emerged as key independent prognostic indicators in our study. While survival rates did not significantly differ between the E-MF and SS subgroups, the overall prognosis was poor. Larger prospective studies are needed to refine prognostic models and treatment strategies.
MATERIALS AND METHODS: A total of 35 patients with E-CTCL were analyzed (6 with E-MF, 15 with de novo SS, 14 with secondary SS). Comprehensive evaluations encompassed clinical and histopathological data, tumor-lymph node-metastasis-blood staging, flow cytometry findings, laboratory parameters, and survival outcomes. Statistical analyses included Kaplan-Meier survival estimates and Cox regression models.
RESULTS: Most patients were male (74.3%) and presented with advanced-stage disease (60%). Elevated serum lactate dehydrogenase and beta-2 microglobulin levels were common, particularly in cases of B2 blood involvement. Female sex and eosinophilia were independent predictors of mortality. Lymph node involvement was associated with rapid progression to erythroderma. No significant survival differences were observed among the E-MF, de novo SS, and secondary SS subgroups.
CONCLUSION: E-CTCL remains a diagnostic and therapeutic challenge. Female sex and eosinophilia emerged as key independent prognostic indicators in our study. While survival rates did not significantly differ between the E-MF and SS subgroups, the overall prognosis was poor. Larger prospective studies are needed to refine prognostic models and treatment strategies.