Abstract
Introduction: Primary Cutaneous CD8+ Aggressive Epidermotropic Cytotoxic T-cell lymphoma (PCAECTCL) is a rare provisional clinical entity and a subtype of cutaneous T-cell lymphoma. It is characterized by cytotoxic T-cells that primarily involve the epidermis causing rapidly progressive epidermal necrosis and extensive skin ulcers. PCAECTCL is often resistant to conventional chemotherapy and is usually associated with a poor prognosis. We conducted this pooled database analysis to delineate key disease characteristics and clinicopathologic determinants of survival in this rare provisional cutaneous T-cell lymphoma. Methods: To study the demographic characteristics, molecular and immunohistochemical signatures, therapeutic interventions, survival, and prognostic factors, we compiled a pooled database of 128 cases. Kaplan-Meier survival curves were constructed. Cox proportional hazards model and Log-rank tests were used to assessing the influence of demographic and clinicopathologic factors on overall survival (OS). Results: A total of 128 patients with confirmed PCAECTCL were identified. The median age was 62, with a peak incidence between 60 and 75 years. There was a male predominance with M:F ratio of 1.9. The median duration of symptoms prior to diagnosis was 6 months. Most of the patients presented with stage IIB (75%), followed by IB (13%), IA(7%), IVB(3%), and IIA(1%). Constitutional symptoms (CS), hemophagocytic syndrome, and ulceration/necrosis were present in 6%, 33%, 1.5%, and 45%, respectively. PCAECTCL widely metastasized in 62% of the cases, mainly to mucocutaneous tissues and visceral organs (83%), which included testicles (11%) and CNS (22%). PCAECTCL immunophenotype consisted of: CD2(37%), CD3(99%), CD5(40%), CD7(66%), CD30(16%), CD45RA(77%), CD56(6%), Granzyme B(72%), TIA-1(100%), and a median Ki-67 score of 70%. The median OS of the whole group was 20 months. OS was significantly impacted as age increased from <40 to 40-59 and > 60 (34 vs. 27 vs. 12 months, p=0.0003). Combination chemotherapy and stem cell transplant were statistically superior to no treatment, with a median OS of 17, 50, and 1 months, respectively (p=0.0002). Males tended to have shorter OS than females (13 vs. 24 months). Similarly, the presence of CS tended to have a shorter OS (6 vs. 21 months). OS was not impacted by stage or immunohistochemical features. Conclusions: This study presents updated clinicopathologic data from a pooled cohort of patients with PCAECTCL. It details the features of this rare provisional entity and identifies the clinical, immunohistochemical, and treatment modalities that are major determinants of OS.
Disclosures
No relevant conflicts of interest to declare.
Author notes
Asterisk with author names denotes non-ASH members.
