Stem cell transplant for myelodysplastic syndrome-associated histiocytoid Sweet's syndrome in a patient with arthritis and myalgias.

Abstract

Introduction Sweet’s syndrome is an acute febrile neutrophilic dermatosis first described by Robert Sweet in 1964 (1). It can occur as an idiopathic entity but can also be drug induced or associated with a number of malignancies (2). Eightyfive percent of associated malignancies are hematologic, most commonly acute myelogenous leukemia (AML) (3). The diagnosis is based upon the presence of the single major criterion and 2 of 4 minor criteria. The major criterion is histologic demonstration of a neutrophilic dermatosis without evidence of vasculitis (2). The minor criteria include 1) a fever 38°C, 2) association with malignancy or connective tissue disease, 3) an excellent response to corticosteroid or potassium iodide therapy, and 4) an elevated erythrocyte sedimentation rate or leukocytosis. Sweet’s syndrome is also seen in patients with myelodysplastic syndromes (MDS). MDS is a group of stem cell disorders caused by disturbances in progenitor cell growth and maturation that are characterized by progressive and refractory cytopenias (4). The disorders frequently progress to AML. The decreased survival in these patients is generally due to infections related to the severe cytopenias. When Sweet’s syndrome is present, it portends a poor prognosis in patients with MDS (4). The association of MDS and Sweet’s syndrome with cryofibrinogenemia has been reported once previously (5). We report a case of MDS-associated Sweet’s syndrome with concomitant cryofibrinogenemia in a 57-year-old man. He proved refractory to traditional therapies (including corticosteroids, cytotoxic agents, and plasmaphoresis) and was treated with stem cell transplantation.