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Insights Into the Characteristics of Sweet Syndrome in Patients With and Without Hematologic Malignancy.

ABSTRACT: Background: Sweet syndrome is a neutrophilic dermatosis that could be associated with malignancy, especially hematologic malignancy. Few studies have systematically elaborated on this disorder and its features related with hematologic malignancy. Objective: This study aimed to describe the clinicopathological characteristics, treatment, and outcome of Sweet syndrome and to evaluate patient characteristics associated with hematologic malignancy. Methods: We retrospectively reviewed patients with Sweet syndrome at the Department of Dermatology, the First Affiliated Hospital of Zhejiang University from October 2010 to February 2019. Results: The study included 37 patients (16 men and 21 women), with a mean age of 53 years. Ten patients (27%) were classified as having malignancy-associated Sweet syndrome: nine with a hematologic malignancy including acute myeloid leukemia (4/9, 44%), myelodysplastic syndrome (4/9, 44%), and multiple myeloma (1/9, 11%) and one with a solid tumor diagnosed with liver carcinoma. The mean hemoglobin and platelet levels (P = 0.007 and P = 0.013, respectively), were significantly lower in patients with hematologic malignancy than in those with Sweet syndrome only. No significant difference in histopathology was found between patients with and without hematologic malignancy. Systemic corticosteroids were the most frequently used treatment (24/37, 65%). Higher mortality was found in patients with hematologic malignancy. Conclusion: It is important to assess Sweet syndrome patients who have laboratory evidence of lower hemoglobin and platelet levels for a hematologic malignancy.

SUBMITTER: Zheng S

PROVIDER: S-EPMC7039821 | biostudies-literature | 2020

REPOSITORIES: biostudies-literature

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Insights Into the Characteristics of Sweet Syndrome in Patients With and Without Hematologic Malignancy.

Zheng Siting S Li Sheng S Tang Shunli S Pan Yunlei Y Ding Yuwei Y Qiao Jianjun J Fang Hong H

Frontiers in medicine 20200218

Background: Sweet syndrome is a neutrophilic dermatosis that could be associated with malignancy, especially hematologic malignancy. Few studies have systematically elaborated on this disorder and its features related with hematologic malignancy. Objective: This study aimed to describe the clinicopathological characteristics, treatment, and outcome of Sweet syndrome and to evaluate patient characteristics associated with hematologic malignancy. Methods: We retrospectively re ...[more]

PMID: 32133363

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Project description:Sweet syndrome (SS), originally described as acute febrile neutrophilic dermatosis, is a rare inflammatory skin condition, considered the prototype of neutrophilic dermatoses. It is characterised by the sudden onset of well-defined tender papules, plaques and nodules often accompanied by fever, neutrophilia and elevated markers of inflammation. Several variants have been described both clinically and histopathologically. Classifications include idiopathic, malignancy-associated, and drug-induced SS. The exact pathogenesis of SS is still unclear; however, recent findings have shed light on the role of dermal infiltrating neutrophils-in the context of innate immunity, and signalling pathways related to adaptive immunity. To critically analyse the current molecular landscape of SS and discuss the recent evidence supporting novel potential immune mediators and biological signalling pathways involved in SS pathogenesis. The methodology followed PRISMA guidelines and included two bibliographical databases, searching articles published until 17 December 2023. Titles, abstracts and full text were reviewed independently by two assessors, while other two investigators resolved any opinion differences. Of 3303 records identified through database search, 22 articles met the eligibility criteria for inclusion. We considered experimental studies that performed molecular analysis, in terms of cytokines quantification, gene expression and/or immunofluorescence/immunohistochemistry. As for the latter, only studies aimed at characterising the nature of the inflammatory infiltrate and potential mechanisms leading to distinct forms of cutaneous inflammatory cell influx were included. Overall, we described research on 202 SS patients (177 skin biopsies and 25 blood specimens) revealing the predominant role of neutrophil activation and abnormal proliferation as unifying mechanisms in different SS subtypes. Interestingly, we found that hyperactivation of the IL-1 pathway might occur only in a subset of SS patients and adaptive immunity could also play a role in the pathogenic scenario of SS, with a potential significant role of IL-17 axis. This systematic review provides a wealth of evidence on the molecular landscape of SS, although further research is needed to a deeper understanding of the patho-mechanisms of this rare disease and hopefully lead to targeted therapeutic approaches.

Project description:The aim of this systematic review is to give an update of all currently available evidence on the relevance of a geriatric assessment in the treatment of older patients with hematologic malignancies. A systematic search in MEDLINE and EMBASE was performed to find studies in which a geriatric assessment was used to detect impaired geriatric domains or to address the association between geriatric assessment and survival or clinical outcome measures. The literature search included 4,629 reports, of which 54 publications from 44 studies were included. Seventy-three percent of the studies were published in the last 5 years. The median age of the patients was 73 years (range, 58-86) and 71% had a good World Health Organization (WHO) performance status. The median prevalence of geriatric impairments varied between 17% and 68%, even in patients with a good WHO performance status. Polypharmacy, nutritional status and instrumental activities of daily living were most frequently impaired. Whereas several geriatric impairments and frailty (based on a frailty screening tool or summarized geriatric assessment score) were predictive for a shorter overall survival, WHO performance status lost its predictive value in most studies. The association between geriatric impairments and treatment-related toxicity varied, with a trend towards a higher risk of (non-)hematologic toxicity in frail patients. During the follow-up, frailty seemed to be associated with treatment non-completion, especially when patients were malnourished. Patients with a good physical capacity had a shorter stay in hospital and a lower rate of hospitalization. Geriatric assessment, even in patients with a good performance status, can detect impaired geriatric domains and these impairments may be predictive of mortality. Moreover, geriatric impairments suggest a higher risk of treatment-related toxicity, treatment non-completion and use of healthcare services. A geriatric assessment should be considered before starting treatment in older patients with hematologic malignancies.

Dataset Information

Insights Into the Characteristics of Sweet Syndrome in Patients With and Without Hematologic Malignancy.

Publications

Insights Into the Characteristics of Sweet Syndrome in Patients With and Without Hematologic Malignancy.

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