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This study evaluated the demographic data, clinical characteristics, treatment approaches, and treatment responses of 43 patients with primary cutaneous CD30 lymphoproliferative disorders. Lymphomatoid papulosis (LyP) was characterized by predominantly papular (94.1%) and generalized (70.6%) lesions, while primary cutaneous anaplastic large-cell lymphoma (pcALCL) presented with tumoral (77.8%) and solitary (77.8%) lesions (p<0.001). Common treatments for LyP included methotrexate (response rate: 78.5%), topical corticosteroids (response rate: 83.3%), and phototherapy (response rate: 85.8%), but relapse rates were high. In pcALCL, complete remission was achieved with all treatments, with no relapses after brentuximab vedotin (BV). Secondary malignancies were noted in 20.6% of LyP cases. Both LyP and pcALCL had a 100% 5-year disease-specific survival rate, although two LyP patients (5.9%) died of secondary malignancies. In conclusion, LyP and pcALCL are both indolent lymphomas, with LyP being more prone to relapse. BV is effective for resistant pcALCL. LyP patients need long-term monitoring due to the risk of secondary malignancies.

Introduction Little is known about long‐term outcomes and causes of death for individuals with cutaneous T‐cell lymphoma. Methods We used SEER‐18 registry data to examine outcomes among 9886 adults with mycosis fungoides (MF), Sézary syndrome (SS), primary cutaneous anaplastic large cell lymphoma (pcALCL), and subcutaneous panniculitis‐like T‐cell lymphoma (SPTCL) diagnosed from 2000 to 2018. We calculated overall survival (Kaplan–Meier method), standardized mortality ratios (SMRs), absolute excess risk (AER) of death, and cumulative incidence of cause‐specific mortality. Results Individuals with CTCL were at increased risk of all‐cause mortality relative to age‐matched controls, with SMR ranging from 1.57 (95% CI: 1.49–1.65) in MF to 5.61 (95% CI: 4.65–6.7) in SS. This was true even for those who initially presented with early‐stage disease. After a median follow‐up of 64 months, the cumulative incidence of lymphoma‐related death was 16.5%, compared to 10.5% other causes, 9.6% cardiovascular, 9.1% second primary malignancy, 1.8% infection, and 1.1% unknown cause. People diagnosed with CTCL were at higher risk of mortality due to leukemia and infectious causes than control populations, but secondary causes made overall minor contributions to total mortality. Conclusion This population‐level analysis revealed that individuals with CTCL were at increased risk of all‐cause mortality relative to age‐matched controls and that lymphoma remained a significant cause of death even in those presenting with early‐stage disease. Trial Registration The authors have confirmed clinical trial registration is not needed for this submission.

To evaluate the frequency of the different types of cutaneous lymphoma (CL) in 1 university hospital in Brazil and compare this frequency with those observed in other countries. After review, 72 (84.7%) cases of primary cutaneous T-cell lymphoma (CTCL) and 13 (15.3%) cases of primary cutaneous B-cell lymphoma (CBCL) were included. Of the CTCLs, 40.3% were mycosis fungoides (MF); 26.4% were adult T-cell leukemias/lymphomas (ATLs); 23.6% were peripheral T-cell lymphomas, unspecified; and 8.3% were anaplastic large cell lymphomas. Of the MF cases, 17.2% progressed to transformed MF. Five-year survival for primary human T-cell lymphotropic virus type 1-negative CTCL, ATL, and CBCL was 64.0%, 42.1%, and 62.5%, respectively. MF and ATL were the most frequent primary CTCLs. The frequencies observed here are close to those observed in Peru but different from those of European countries. Unfortunately, the World Health Organization/European Organization of Research and Treatment of Cancer classification does not include primary cutaneous ATL.