Track

Abstract

A 60-year-old male presented with severe fatigue and unintentional weight loss over three months. Laboratory studies revealed pancytopenia and imaging demonstrated cervical, retroperitoneal, and inguinal lymphadenopathy, as well as hepatosplenomegaly. Bone marrow biopsy showed a mantel cell lymphoma (MCL), blastoid/pleomorphic variant, with tumor cells expressing PAX5, cyclin D1, SOX11, nuclear p53, and a high proliferative index (ki-67: 90%). The patient was treated with BOVen regimen (Zanubrutinib, obinutuzumab, and venetoclax).

Approximately 5 months after diagnosis, the patient developed subcutaneous nodules on the right flank and left lateral shin, clinically appearing as purpuric or bruised skin. CT imaging revealed confluent retroperitoneal lymphadenopathy and subcutaneous tissue nodules, with bilateral pelviectasis and ureterectasis. A punch biopsy of the flank nodule showed a dense lymphoid infiltrate in the deep dermis, extending into adnexal structures. The neoplastic cells exhibited enlarged nuclei with open, vesicular chromatin, prominent nucleoli, and expressed CD20, CD5, cyclin D1, BCL2, SOX11, nuclear p53, and kappa light chain restrictionon with a high proliferative index. These findings confirmed cutaneous involvement by MCL, blastoid variant.

Blastoid variants of MCL are aggressive and may arise de novo or through transformation of classic MCL. Cutaneous involvement by MCL is rare, often presenting as nodules or plaques, typically on extremities, and frequently represents relapse or progression. Accurate recognition of this entity is critical given its aggressive behavior and worse prognosis compared to other MCL subtypes.