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1.
Support Care Cancer ; 29(11): 6669-6679, 2021 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-33963457

RESUMEN

PURPOSE: Cutaneous lymphomas (CLs) are a group of rare, potentially disfiguring and disabling cancers that can have a significant impact on quality of life (QoL). While previous studies have shown that mycosis fungoides (MF) and Sézary syndrome (SS) impair QoL, the effect of other types of CL on QoL has not been evaluated. OBJECTIVE: To determine the impact of disease on QoL in all CL patients and to assess how QoL between the CL sub-types varies by demographic and clinical factors. METHODS: The Cutaneous Lymphoma Distress Questionnaire (CL-DQ) was used to assess QoL. All CL patients seen in a multidisciplinary CL clinic were screened for eligibility. Questionnaire responses were collected over a 22-month period between 2017 and 2019. A cross-sectional analysis of CL-DQ scores from an initial visit was performed to determine the effect of disease on QoL across CL sub-types and the potential impact of patient demographics, CL sub-type, and type of treatment. RESULTS: The study population consisted of 151 patients presenting with distinct types of cutaneous B- and T-cell lymphomas. Notable across the study population were the findings of frustration (44%), worry about progress/spread (43%), itching/pruritus (32%), and embarrassment/shame (28%). QoL was found to be most negatively affected in SS patients, females, younger patients, Black patients, and those with advanced stages of MF/SS. CONCLUSIONS: Impairment of QoL due to CL correlates with gender, age, race/ethnicity, and stage of MF/SS. While the negative impact on QoL is most pronounced in SS patients, other CL sub-types also affect QoL and impact psychosocial distress. Our findings highlight the need for QoL assessment in all CL patients and further examination of disparities noted across demographic groups.


Asunto(s)
Síndrome de Sézary , Neoplasias Cutáneas , Estudios Transversales , Etnicidad , Femenino , Humanos , Calidad de Vida , Síndrome de Sézary/epidemiología , Neoplasias Cutáneas/epidemiología
2.
Curr Hematol Malig Rep ; 15(4): 333-342, 2020 08.
Artículo en Inglés | MEDLINE | ID: mdl-32435988

RESUMEN

PURPOSE OF REVIEW: Primary cutaneous CD30+ T-cell lymphoproliferative disorders (CD30+ LPDs) are the second most common cutaneous lymphomas after mycosis fungoides and Sezary syndrome. They include primary cutaneous anaplastic large cell lymphoma (pcALCL), lymphomatoid papulosis (LyP), and borderline lesions. The purpose of this literature review is to consolidate the available evidence on the primary cutaneous CD30+ LPD in order to define the tools for correct diagnosis and appropriate treatment. RECENT FINDINGS: The current body of knowledge regarding the clinical features, histopathologic changes, recently described genetic alterations, and therapeutic options will be covered in this comprehensive review. Primary cutaneous CD30+ LPD represent rare cutaneous lymphomas that have significant histologic overlap within the defined group as well as with other neoplastic and reactive entities. The importance of differentiating these entities is crucial, as each one has a different clinical course and prognosis.


Asunto(s)
Antígeno Ki-1/inmunología , Linfoma Anaplásico Cutáneo Primario de Células Grandes , Papulosis Linfomatoide , Trastornos Linfoproliferativos , Neoplasias Cutáneas , Diagnóstico Diferencial , Humanos , Linfoma Anaplásico Cutáneo Primario de Células Grandes/genética , Linfoma Anaplásico Cutáneo Primario de Células Grandes/inmunología , Linfoma Anaplásico Cutáneo Primario de Células Grandes/terapia , Papulosis Linfomatoide/genética , Papulosis Linfomatoide/inmunología , Papulosis Linfomatoide/terapia , Trastornos Linfoproliferativos/genética , Trastornos Linfoproliferativos/inmunología , Trastornos Linfoproliferativos/terapia , Valor Predictivo de las Pruebas , Pronóstico , Neoplasias Cutáneas/genética , Neoplasias Cutáneas/inmunología , Neoplasias Cutáneas/terapia
3.
Best Pract Res Clin Haematol ; 32(3): 239-252, 2019 09.
Artículo en Inglés | MEDLINE | ID: mdl-31585624

RESUMEN

Mycosis fungoides (MF) and Sézary syndrome (SS) are the most common types of cutaneous lymphoma, accounting for approximately 60% of cutaneous T-cell lymphomas. Diagnosis requires correlation of clinical, histologic, and molecular features. A multitude of factors have been linked to the aetiopathogenesis, however, none have been definitively proven. Erythrodermic MF (E-MF) and SS share overlapping clinical features, such as erythroderma, but are differentiated on the degree of malignant blood involvement. While related, they are considered to be two distinct entities originating from different memory T cell subsets. Differential expression of PD-1 and KIR3DL2 may represent a tool for distinguishing MF and SS, as well as a means of monitoring treatment response. Treatment of E-MF/SS is guided by disease burden, patients' ages and comorbidities, and effect on quality of life. Current treatment options include biologic, targeted, immunologic, and investigational therapies that can provide long term response with minimal side effects. Currently, allogeneic stem cell transplantation is the only potential curative treatment.


Asunto(s)
Memoria Inmunológica , Micosis Fungoide , Síndrome de Sézary , Neoplasias Cutáneas , Linfocitos T , Humanos , Micosis Fungoide/diagnóstico , Micosis Fungoide/inmunología , Micosis Fungoide/patología , Micosis Fungoide/terapia , Proteínas de Neoplasias/inmunología , Receptor de Muerte Celular Programada 1/inmunología , Receptores KIR3DL2/inmunología , Síndrome de Sézary/diagnóstico , Síndrome de Sézary/inmunología , Síndrome de Sézary/patología , Síndrome de Sézary/terapia , Neoplasias Cutáneas/diagnóstico , Neoplasias Cutáneas/inmunología , Neoplasias Cutáneas/patología , Neoplasias Cutáneas/terapia , Linfocitos T/inmunología , Linfocitos T/patología
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