Uncommon form of extranodal Hodgkin’s disease originating in the skin at the time of diagnosis and without evidence of extracutaneous involvement within 6 months of diagnosis.
Classified by primary cutaneous marginal zone B cell lymphoma, primary cutaneous follicle center lymphoma and primary cutaneous large B cell lymphoma, leg type.
Primary cutaneous large B cell lymphoma, leg type more common in women over the age of 70 years.
Primary cutaneous large B cell lymphoma, leg type has a strong bcl-2 protein expression which is associated with a poor prognosis.
Primary cutaneous large B cell lymphoma, leg type, presence of multiple lesions associated with a poor prognosis.
Primary cutaneous large B cell lymphoma, leg type has a worse prognosis than other B cell cutaneous lymphomas with a 5 year survival of 58%.
Brentuxmab indicated for the treatment of adult patients with primary cutaneous anaplastic large cell lymphoma (pcALCL) or CD30-expressing mycosis fungoides (MF) who have received prior systemic therapy.
FDA approval was based in part on the clinical trial results from ALCANZA, a global, phase 3, randomized study evaluating methotrexate or bexarotene.
The phase III ALCANZA trial found a 16.7 months medium progression free survival of 16.7 months with brentuximab vs 3.5 months with Physicians Choice of therapy.
In the above study the objective response rate was 67% with 16% complete response rate.