Sweet Syndrome Associated with Myelodysplastic Syndrome—A Review of a Multidisciplinary Approach
Sweet syndrome (SS) is a rare disease described as a febrile neutrophilic dermatosis with acute onset, the pathogenesis of which has not yet been elucidated. The syndrome is characterized by the sudden onset of erythematous infiltrated papules or plaques located on the upper body and is associated w...
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| Format: | Article |
| Language: | English |
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MDPI AG
2023-03-01
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| Series: | Life |
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| Online Access: | https://www.mdpi.com/2075-1729/13/3/809 |
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| author | Cătălina Roxana Ferea Stejara Nicoleta Mihai Gabriela Balan Minerva Codruta Badescu Dana Tutunaru Alin Laurențiu Tatu |
| author_facet | Cătălina Roxana Ferea Stejara Nicoleta Mihai Gabriela Balan Minerva Codruta Badescu Dana Tutunaru Alin Laurențiu Tatu |
| author_sort | Cătălina Roxana Ferea |
| collection | DOAJ |
| description | Sweet syndrome (SS) is a rare disease described as a febrile neutrophilic dermatosis with acute onset, the pathogenesis of which has not yet been elucidated. The syndrome is characterized by the sudden onset of erythematous infiltrated papules or plaques located on the upper body and is associated with fever, leukocytosis and neutrophilia. The lesions show a dense dermal infiltration with mature neutrophils. The condition is responsive to systemic steroids. The central nervous system, bones, muscles, eyes, ears, mouth, heart, lung, liver, kidneys, intestines, and spleen may be affected by SS as extracutaneous manifestations. More and more cases have been found to be associated with malignancies, particularly myelodysplastic syndrome, and, less frequently, other hematologic malignancies or solid tumors. Approximately 21% of patients with SS have an associated malignancy and up to 80% of MASS cases are associated with hematological diseases, predominantly myelodysplastic syndrome (MDS) or acute myeloid leukemia (AML). Myelodysplastic syndrome is a clonal disease of the bone marrow characterized by inefficient hematopoiesis, dysplasia of the bone marrow and peripheral cytopenias. Affected patients have a high risk of leukemic transformation. After analyzing later studies and current practical aspects regarding MDS-related SS, we suggest an algorithm for evaluating these patients. |
| first_indexed | 2024-03-11T06:17:47Z |
| format | Article |
| id | doaj.art-f251d6591fe2442185db196e5ad31bcb |
| institution | Directory Open Access Journal |
| issn | 2075-1729 |
| language | English |
| last_indexed | 2024-03-11T06:17:47Z |
| publishDate | 2023-03-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Life |
| spelling | doaj.art-f251d6591fe2442185db196e5ad31bcb2023-11-17T12:12:40ZengMDPI AGLife2075-17292023-03-0113380910.3390/life13030809Sweet Syndrome Associated with Myelodysplastic Syndrome—A Review of a Multidisciplinary ApproachCătălina Roxana Ferea0Stejara Nicoleta Mihai1Gabriela Balan2Minerva Codruta Badescu3Dana Tutunaru4Alin Laurențiu Tatu5Faculty of Medicine, “Carol Davila” University of Medicine and Farmacy, 020021 Bucharest, RomaniaFaculty of Medicine, “Carol Davila” University of Medicine and Farmacy, 020021 Bucharest, RomaniaFaculty of Medicine and Pharmacy, ”Dunărea de Jos” University of Galați, 800008 Galați, RomaniaDepartment of Internal Medicine, “Grigore T. Popa” University of Medicine and Farmacy, 700115 Iași, RomaniaFaculty of Medicine and Pharmacy, ”Dunărea de Jos” University of Galați, 800008 Galați, RomaniaFaculty of Medicine and Pharmacy, ”Dunărea de Jos” University of Galați, 800008 Galați, RomaniaSweet syndrome (SS) is a rare disease described as a febrile neutrophilic dermatosis with acute onset, the pathogenesis of which has not yet been elucidated. The syndrome is characterized by the sudden onset of erythematous infiltrated papules or plaques located on the upper body and is associated with fever, leukocytosis and neutrophilia. The lesions show a dense dermal infiltration with mature neutrophils. The condition is responsive to systemic steroids. The central nervous system, bones, muscles, eyes, ears, mouth, heart, lung, liver, kidneys, intestines, and spleen may be affected by SS as extracutaneous manifestations. More and more cases have been found to be associated with malignancies, particularly myelodysplastic syndrome, and, less frequently, other hematologic malignancies or solid tumors. Approximately 21% of patients with SS have an associated malignancy and up to 80% of MASS cases are associated with hematological diseases, predominantly myelodysplastic syndrome (MDS) or acute myeloid leukemia (AML). Myelodysplastic syndrome is a clonal disease of the bone marrow characterized by inefficient hematopoiesis, dysplasia of the bone marrow and peripheral cytopenias. Affected patients have a high risk of leukemic transformation. After analyzing later studies and current practical aspects regarding MDS-related SS, we suggest an algorithm for evaluating these patients.https://www.mdpi.com/2075-1729/13/3/809sweet syndromemyelodysplastic syndromemalignancy-associated SSmanagementneutrophilic dermatosis |
| spellingShingle | Cătălina Roxana Ferea Stejara Nicoleta Mihai Gabriela Balan Minerva Codruta Badescu Dana Tutunaru Alin Laurențiu Tatu Sweet Syndrome Associated with Myelodysplastic Syndrome—A Review of a Multidisciplinary Approach Life sweet syndrome myelodysplastic syndrome malignancy-associated SS management neutrophilic dermatosis |
| title | Sweet Syndrome Associated with Myelodysplastic Syndrome—A Review of a Multidisciplinary Approach |
| title_full | Sweet Syndrome Associated with Myelodysplastic Syndrome—A Review of a Multidisciplinary Approach |
| title_fullStr | Sweet Syndrome Associated with Myelodysplastic Syndrome—A Review of a Multidisciplinary Approach |
| title_full_unstemmed | Sweet Syndrome Associated with Myelodysplastic Syndrome—A Review of a Multidisciplinary Approach |
| title_short | Sweet Syndrome Associated with Myelodysplastic Syndrome—A Review of a Multidisciplinary Approach |
| title_sort | sweet syndrome associated with myelodysplastic syndrome a review of a multidisciplinary approach |
| topic | sweet syndrome myelodysplastic syndrome malignancy-associated SS management neutrophilic dermatosis |
| url | https://www.mdpi.com/2075-1729/13/3/809 |
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