Previously, most patients with PCDLBCL, LT were treated with the CHOP chemotherapy regimen of cycloheximide, hydroxydaunorubicin, oncovin, and prednisone. The more recent addition of the immunotherapy drug, rituximab, to this regimen has given<unk> etter results. Rituximab is a monoclonal antibody that kills cells which express high levels of CD20<unk> y<unk> inding to this cell-surface protein and thereby targeting them for attack<unk> y the hosts immune system. Accordingly, the addition of rituximab to CHOP, i.e. the R-CHOP chemoimmunotherapy regimen. with or without radiotherapy (used to treat symptoms resulting from specific localized lesions) is now recommended<unk> y the European Organisation for Research and Treatment of Cancer and the International Society for Cutaneous Lymphomas as first line therapy for single, localized, and widespread diseases. Cases in which hydroxydaunorubicin is contraindicated<unk> ecause of, e.g. preexisting heart disease, may treated with the R-COP regiment (i.e. R-CHOP minus hydroxydaunorubicin). Patients who might<unk> e intolerant to R-CHO<unk> ecause of general health issues have<unk> een treated with just rituximab and radiotherapy although more recent reports indicate that these patients may<unk> e successfully treated with a regimen that replaces hydroxydaunorubicin with PEGylated, liposome-encased doxorubicin in the R-CHOP regimen. Experimental treatments A phase II clinical trial is recruiting individuals to study the efficacy and safety of nivolumab, a (monoclonal antibody that<unk> inds to programmed death-ligand 1 thereby<unk> locking its ability to suppress immune responses) with or without varlilumab (a monoclonal antibody that<unk> inds to the CD27 protein expressed<unk> y cells and thereby promotes the anti-tumor activity of T-cells) in treating patients with aggressive<unk> -cell lymphomas, including PCDLBCL, LT that have relapsed after or do not respond to treatment. Other agents are<unk> eing evaluated in refractory or relapsed<unk> -cell lymphoid malignancies<unk> ut not PCDLBCL, NOS and ultimately may prove useful in PCDLBCL, LT. These include: ofatumumab, a monoclonal antibody that is stronger than rituximab in<unk> inding to CD20; two radioimmunotherapy monoclonal antibodies, Ibritumomab tiuxetan and Tositumomab that<unk> ind CD20 to deliver radiation from their attached radioactive isotopes to and kill CD20-bearing cells; lumiliximab, a monoclonal antibody that<unk> inds to CD23; dacetuzumab, a monoclonal antibody that<unk> inds CD40; Siglec-3 a monoclonal antibody that<unk> inds CD33;<unk> linatumomab, a monoclonal antibody that<unk> inds<unk> oth CD3 and CD19; chimeric antigen receptor T cell therapy using CD19-directed CAR-T cells; and lenalidomide, a drug with multiple anti-tumor actions.
Primary cutaneous diffuse large B-cell lymphoma, leg type
Secondary cutaneous CD30+ large-cell lymphoma
Primary cutaneous marginal zone lymphoma
Primary cutaneous follicle center lymphoma
Primary cutaneous immunocytoma
CD30+ cutaneous T-cell lymphoma
Non-mycosis fungoides CD30− cutaneous large T-cell lymphoma
Chronic multifocal Langerhans cell histiocytosis