(Last Updated On: May 13, 2022)

Katrina M. Canlas-Estrella, MD, DPDS, Joshua A. Arcaira, MD, DPDS, Filomena Legarda-Montinola, MD, FPDS, Teresita E. Dumagay, MD, FPCP

Abstract

Introduction:Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma (PCAECTCL) is a rare subtype of cutaneous T-cell lymphoma characterized by widely distributed ulcerated lesions, epidermotropic infi ltrates of CD8+ cytotoxic T cells, aggressive course, high tendency to spread to extranodal sites, poor response to conventional therapies and unfavorable prognosis.

Case Report: We report a 76-year-old Filipino male presenting with eight-month history of erythematous scaly patches evolving into widespread ulcerated nodules, unresponsive to topical and systemic steroids. Histopathology revealed prominent epidermotropism and lichenoid infiltrate of atypical lymphocytes. Immunohistochemistry showed positivity for CD3, CD8, Ki67 (5-15%), CD7, CD2; indeterminate for TIA-1, with high background staining; and was negative for CD20, CD30, CD4, CD5, CD56, granzyme-B, TdT, Epstein-Barr encoding region in situ hybridization (EBER-ISH), consistent with PCAECTCL. No overt metastasis was detected.
The patient underwent interferon alfa 2B therapy followed by three full cycles of CHOP chemotherapy. Improvement was seen as
thinning of plaques and nodules and re-epithelialization of ulcers however, severe anemia and leukopenia ensued with therapy.
He then succumbed to septic shock secondary to pneumonia during the height of the COVID-19 pandemic.

Conclusion:This case emphasizes that despite accurate diagnosis, polychemotherapy, and favorable response to therapy, complications may still arise contributing to the poor prognosis and low five-year survival rate of this condition.

 

Keywords:T-cell lymphoma, CD8 positive, neoplasms

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