Primary cutaneous B-cell lymphomas: part II. Therapy and future directions

Area of Science:

• Dermatology
• Oncology
• Hematology

Background:

• Accurate histopathologic classification and exclusion of systemic disease are crucial for selecting primary cutaneous B-cell lymphoma (PCBCL) therapies.
• This article reviews therapeutic options for various PCBCL subtypes.

Purpose of the Study:

• To outline current and investigational treatment strategies for primary cutaneous B-cell lymphomas.
• To differentiate management approaches based on PCBCL subtype and prognosis.

Main Methods:

• Review of available therapeutic modalities for PCBCL.
• Classification of PCBCL into indolent (PCFCL, PCMZL) and aggressive (PCDLBCL) subtypes.
• Discussion of treatment based on disease extent and presentation.

Main Results:

• Primary cutaneous follicle center lymphoma (PCFCL) and primary cutaneous marginal zone lymphoma (PCMZL) are indolent, with excellent prognoses managed by local therapies or observation.
• Relapses are common in PCFCL and PCMZL, but overall survival remains excellent.
• Primary cutaneous diffuse large B-cell lymphoma (PCDLBCL) has a poorer prognosis, necessitating aggressive treatment with radiation and/or chemotherapy.

Conclusions:

• Management of PCBCL is subtype-dependent, with indolent forms favoring local therapies and aggressive forms requiring systemic treatment.
• While relapses occur, indolent PCBCLs maintain excellent survival rates.
• Aggressive PCBCL, particularly PCDLBCL, benefits from multi-modality treatment, with investigational approaches showing promise.