CD8+ aggressive T-cell lymphoma (T-NHL) involving the liver and skin: a case study

Abstract

Abstract Introduction/Objective Aggressive T-NHL involving cutaneous and visceral organs is rare. We report a case with a complex presentation that was found to have hepatic and skin lesions simultaneously. We emphasize the importance of the clinicopathological correlation and diagnostic work-up for such a rare case Methods/Case Report A 60-year-old male with alcoholic cirrhosis presented with abdominal pain, fever, and night sweats for over two weeks. Detailed physical evaluation revealed a tender, indurated, erythematous papule on the left upper back. Lab results showed leukopenia, anemia, and increased LDH. PET-CT showed multiple hypermetabolic lesions in the liver, skin, and deep soft tissue above and below the diaphragm. A liver biopsy revealed a diffuse sheath of large atypical lymphoid cells with small lymphocytes in the background. Atypical lymphoid cells stained positive for CD45, CD2, CD3, CD8, TIA-1, CD5 (partial), granzyme B (partial), βF1 (partial); and negative for ALK, BCL-2, CD4, CD7, CD10, CD30, CD56, and TCRδ. Ki-67 was 70%. The skin punch biopsy revealed epidermotropic infiltration of lymphocytes with a similar immunophenotype as the liver lesion. Bone marrow biopsy showed no involvement. The differential diagnosis includes primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-NHL with visceral dissemination, peripheral CD8+ T-NHL, lymphomatoid papulosis, CD8-positive mycosis fungoides, and hepatosplenic T-NHL. The visceral involvement excludes lymphomatoid papulosis and CD8-positive mycosis fungoides. The absence of bone marrow involvement excludes hepatosplenic T-NHL. The prominent liver involvement favors the diagnosis of peripheral CD8+ T-NHL. However CD8+ aggressive epidermotropic cytotoxic T-NHL still cannot be entirely excluded due to the epidermotropic CD8 positive T-cell infiltration of the dermis and subcutaneous adipose tissue. Further evaluation for JAK2 mutation and TBX21 amplification can help distinguish it from Peripheral CD8- positive T-NHL. Results (if a Case Study enter NA) NA Conclusion The subclassification of CD8+ T-NHL into distinct entities with overlapping clinical and histological characteristics is challenging without molecular tests. However, clinicopathological correlation with appropriate work-up can help narrow the differential diagnoses.