Characterizing Outcomes in Visceral Cutaneous T-Cell Lymphoma: A Single Center Retrospective Study

CLINICAL LYMPHOMA MYELOMA & LEUKEMIA(2023)

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摘要
Cutaneous T-Cell Lymphoma is a rare and heterogenous non-Hodgkin lymphoma. Visceral involvement is a rare but fatal complication. We report a case retrospective analysis, describe clinical characteristics, and confirm poor survival outcomes. No treatments were associated with improved outcomes, but long-term survivors were treated with brentuximab vedotin and allogeneic transplantation. Additional studies are needed to guide treatment selection.Introduction: Visceral involvement of cutaneous T-cell lymphoma (vCTCL) is a rare but poorly studied complication of CTCL. We aimed to assess clinical characteristics, treatment, and outcomes, associated with vCTCL at our institution.Methods: We conducted a retrospective review of patients with vCTCL among patients with a confirmed histopathologic diagnosis of CTCL seen at the Winship Cancer Institute in Emory University. vCTCL was defined as a highest TNMB stage of 4B with extracutaneous metastatic disease (M1) pathologically confirmed or strongly clinically suspected based on imaging, symptoms, and the clinical judgment of the treating physician. Patients were selected from our CTCL database containing 656 patients from 1990 to 2022. Clinical characteristics were characterized. Clinical outcomes were measured as overall survival (OS) and progression-free survival (PFS) using Kaplan-Meier curve and univariable Cox regression analysis. Results: Twenty-six of 656 patients with vCTCL were identified. 42.3% of patients were black. Twenty-two patients were diagnosed with MF/SS and 4 had other CTCL subtypes including pcALCL, Gamma-Delta, and Cytotoxic T-Cell Lymphoma. The median PFS and OS were 7.3 months (3.8, 11) and 12.1 months (9.9, 18.2), respectively. Median time to metastasis from initial diagnosis was 12.1 months. The most common M1 sites were liver (19.2%) and lung (42.3%). M1 sites outside of liver or lung were associated with inferior OS (HR 8.9, 95%CI: 2.7-29.5, P -value < .001) and PFS (HR 4.3, 95%CI: 1.44-12.7, P -value = .009). No treatments or baseline factors were associated with improved survival. Conclusion: Our retrospective study confirms therapy resistance and dismal outcomes among patients with vCTCL.Clinical Lymphoma, Myeloma and Leukemia, Vol. 23, No. 9, 667-673 (c) 2023 Elsevier Inc. All rights reserved.
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关键词
Cutaneous T Cell lymphoma,Non-Hodgkin lymphoma,Mycosis fungoides,Sezary syndrome,Brentuximab
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