Primary Cutaneous Diffuse Large B-cell Lymphoma, Leg Type

Comments:
Treatment & Prognosis: Primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL, LT) is an aggressive tumor with poor prognosis. The 5-yr survival rate is 50% compared to >90% for cutaneous marginal zone lymphoma and >95% for primary cutaneous follicle center lymphoma.

The treatment is similar to that for systemic diffuse large B-cell lymphoma and consists of anti-CD20 antibody Rituximab + CHOP-like regimen. Relapses occur in 55-69% of cases. With the addition of rituximab, the 5-yr disease-specific survival rate has increased from 50% to about 70%.

Indicators of poor prognosis include: loss of CDKN2A by deletion or hypermethylation, MYD88 L265P mutation, and multifocal skin lesions.