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Chronic relapsing remitting Sweet syndrome--a harbinger of myelodysplastic syndrome.
- Source :
-
British journal of haematology [Br J Haematol] 2015 Sep; Vol. 170 (5), pp. 649-56. Date of Electronic Publication: 2015 May 12. - Publication Year :
- 2015
-
Abstract
- Sweet syndrome (SS) is an acute febrile neutrophilic dermatosis. It has been associated with malignant disease, especially acute myeloid leukaemia (AML), infections, autoimmune disorders and drugs, particularly granulocyte colony-stimulating factor (GCSF). No cause is found in the rest, which are labelled idiopathic. We describe 15 patients with SS, which we believe represent 'immune dysregulation' secondary to myelodysplastic syndrome (MDS). We initially identified 31 patients with SS in a cohort of 744 patients with MDS and 215 with AML seen over a 6-year period (2004-10). The cause in 16 patients could be attributed either to administration of GCSF or chemotherapy. The eruption was brief and resolved spontaneously or following withdrawal of GCSF. Fifteen patients however, had a chronic debilitating illness dominated by the skin eruptions. Diagnosis of chronic relapsing SS was delayed because the pathology was not always typical of classical neutrophil-rich SS and included lymphocytic and histiocytoid infiltrates and bone marrow was not always performed because the relevance of the eruption to MDS was often not immediately appreciated. All these patients had 'low risk' MDS, diagnosed at a median of 17 months (range 0-157) following the diagnosis of SS. We describe a chronic debilitating episodic clinically distinctive skin eruption with features of SS but not always definitive histopathology often associated with immunological abnormalities affecting other systems related to underlying low risk MDS.<br /> (© 2015 John Wiley & Sons Ltd.)
- Subjects :
- Adult
Aged
Aged, 80 and over
Female
Granulocyte Colony-Stimulating Factor administration & dosage
Granulocyte Colony-Stimulating Factor adverse effects
Humans
Male
Middle Aged
Myelodysplastic Syndromes complications
Myelodysplastic Syndromes drug therapy
Myelodysplastic Syndromes epidemiology
Myelodysplastic Syndromes pathology
Skin pathology
Sweet Syndrome epidemiology
Sweet Syndrome etiology
Sweet Syndrome pathology
Subjects
Details
- Language :
- English
- ISSN :
- 1365-2141
- Volume :
- 170
- Issue :
- 5
- Database :
- MEDLINE
- Journal :
- British journal of haematology
- Publication Type :
- Academic Journal
- Accession number :
- 25962438
- Full Text :
- https://doi.org/10.1111/bjh.13485