Primary cutaneous lymphoma

Primary cutaneous lymphoma (PCL) is a heterogeneous group of non-Hodgkin lymphomas that originate in the skin without evidence of extracutaneous disease at the time of diagnosis. These lymphomas are broadly classified into two main categories: primary cutaneous T-cell lymphomas (CTCL), which account for approximately 75-80% of cases, and primary cutaneous B-cell lymphomas (CBCL), which make up the remaining 20-25%. The most common subtypes include mycosis fungoides, Sézary syndrome, and primary cutaneous anaplastic large cell lymphoma among T-cell types, and primary cutaneous marginal zone lymphoma, primary cutaneous follicle center lymphoma, and primary cutaneous diffuse large B-cell lymphoma, leg type, among B-cell types. The disease primarily affects the skin, presenting with patches, plaques, tumors, or erythroderma (widespread reddening of the skin) depending on the subtype and stage. Patients may experience persistent itching (pruritus), skin lesions that may ulcerate, lymphadenopathy (swollen lymph nodes), and in advanced cases, involvement of the blood, lymph nodes, and internal organs. Sézary syndrome is characterized by erythroderma, circulating malignant T-cells (Sézary cells) in the blood, and lymphadenopathy. Symptoms can significantly impact quality of life, including sleep disturbance and psychological distress. Treatment depends on the specific subtype, stage, and clinical behavior. For early-stage CTCL such as mycosis fungoides, skin-directed therapies are the mainstay, including topical corticosteroids, phototherapy (PUVA or narrowband UVB), topical nitrogen mustard, and localized radiation therapy. For more advanced or refractory disease, systemic therapies may be employed, including retinoids (bexarotene), interferon-alpha, histone deacetylase inhibitors (vorinostat, romidepsin), monoclonal antibodies (mogamulizumab, brentuximab vedotin), and in select cases, allogeneic stem cell transplantation. Indolent subtypes of CBCL may be managed with local radiation therapy or surgical excision, while aggressive subtypes typically require systemic chemotherapy with or without rituximab. Prognosis varies widely depending on the subtype, with indolent forms having an excellent long-term outlook and aggressive subtypes carrying a more guarded prognosis.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Common questions about Primary cutaneous lymphoma