Immunochemotherapy for Bcl-2 and MUM-negative aggressive primary cutaneous B-cell non-Hodgkin's lymphoma

The case of a 44-year-old man with a primary cutaneous large B-cell non-Hodgkin's lymphoma of the scalp is reported. His mother died of gastric lymphoma and his sib brother is in a 20-year remission of T-cell lymphoma. The patient presented with a 16-year history of occipital and parietal alopecia and a recently worsening scalp rash. The histopathology and immunohistochemistry performed in April 2006 indicated a bcl-6+, MUM- and bcl-2-, primary cutaneous follicle center B-cell non-Hodgkin's lymphoma, with an aggressive transformation to a diffuse large B-cell lymphoma. Bone marrow biopsy and CT chest, abdomen, and pelvis were negative for systemic lymphoma. The patient had an excellent clinical and histological resolution following 8 cycles of rituximab and CHOP protocol immunochemotherapy, and remains in complete remission until now. The protracted indolent phase of the disease, the familial history of lymphoma, the histological aggressive features and the patient's excellent response to immunochemotherapy all contribute to a very unusual manifestation of this disease.