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Sweet Syndrome Associated with Myelodysplastic Syndrome

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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. Up to 80% of SS cases are associated with hematological diseases, predominantly myelodysplastic syndrome (MDS). Myelodysplastic syndrome is a clonal disease of the bone marrow characterized by inefficient hematopoiesis, dysplasia of the bone marrow and peripheral cytopenias. After analyzing later studies and current practical aspects regarding MDS-related SS, in this report an algorithm for evaluating these patients is suggested. Read the full article here.

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