ABCL-190 primary cutaneous non-hodgkin lymphoma -leg type: long-term durable response with dose-adjusted EPOCH-R regimen

Abstract

Context: Primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBL-LT) constitutes 1% of all cutaneous lymphomas and it has an aggressive behaviour with poor outcome. PCDLBL-LT is predominantly seen in elderly people, has a female preponderance, and 70% of lesions are present in the lower extremities. Objective: We present a case of PCDLBL-LT presenting to our clinic with facial skin lesion and B symptoms and highlight the good response of this disease to dose-adjusted EPOCH-R regimen (etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin, and rituximab). Design, Setting and Participant This retrospective single case study was conducted at the Department of Medical Oncology, All India Institute of Medical Sciences (AIIMS), New Delhi. Result: The index case was a 50-year-old woman who presented with painless rapidly progressive annular plaque-like lesions over the forehead which she had had for 5 months, associated with fever, significant weight loss, and loss of appetite. Physical examination revealed palpable cervical and axillary lymphadenopathy with skin lesions over the left forehead. Laboratory evaluations showed anemia (Hb-9 gm/dl) and elevated LDH (840U/L- upper cut off: 240U/L). The rest of the observations were within normal limits. The punch biopsy from the skin lesion revealed diffuse infiltration by large atypical lymphoid cells with vesicular nuclei and pleomorphism in the dermis. These atypical cells are immunopositive for CD20, and MUM1 while negative for CD3, BCl6, EBV, and LMP. The ki67 proliferative index is 70%. Radiological evaluation by CECT scan revealed supra- and infra- diaphragmatic lymphadenopathy. She completed six cycles of chemotherapy with dose-adjusted EPOCH-R regimen. The maximum dose intensity of dose adjusted EPOCH-R regimen reached was level 3. Two episodes of febrile neutropenia were observed during her chemotherapy, which recovered with supportive care. After six cycles, she was in clinical and radiological remission and continues to be in remission after a follow-up of 3 years. Conclusion: Dose-adjusted EPOCH-R regimen is a well tolerated and effective regimen in PCDLBL-LT, where there is no standard of care. Early and accurate diagnosis is required of this condition so that early and appropriate treatment can be started due to the aggressive course of PCDLBL-LT.