Initially lymphocytic Sweet's syndrome in male patients with myelodysplasia: a distinguished clinicopathological entity? Case report and systematic review of the literature.
Τίτλος | Initially lymphocytic Sweet's syndrome in male patients with myelodysplasia: a distinguished clinicopathological entity? Case report and systematic review of the literature. |
Publication Type | Journal Article |
Year of Publication | 2014 |
Authors | Kakaletsis, N., Kaiafa G., Savopoulos C., Iliadis F., Perifanis V., Tzalokostas V., Grekou A., Giannouli A., & Hatzitolios A. I. |
Journal | Acta Haematol |
Volume | 132 |
Issue | 2 |
Pagination | 220-5 |
Date Published | 2014 |
ISSN | 1421-9662 |
Λέξεις κλειδιά | Aged, Anemia, Refractory, with Excess of Blasts, Biopsy, Chromosomes, Human, X, Chromosomes, Human, Y, Disease Progression, Humans, Lymphocytes, Male, Middle Aged, Models, Immunological, Myelodysplastic Syndromes, Neutrophil Infiltration, Receptors, Granulocyte-Macrophage Colony-Stimulating Factor, Sex Factors, Skin, Sweet Syndrome |
Abstract | BACKGROUND: Sweet's syndrome (SS) is an acute febrile neutrophilic dermatosis. It can occur as an idiopathic, drug-induced or malignancy-associated entity. SS is also seen in patients with myelodysplastic syndrome (MDS) where it may present atypically, both clinically and histologically. In a few rare cases of MDS, lymphocytic infiltrates are the presenting feature of SS.METHODS: MEDLINE and Scopus were the data sources for our review.RESULTS: A clinicopathological subsetemerged of 12 male SS patients with MDS and a mean age of 67.3 years in which the initial SS lesions were lymphocytic infiltrates. However, from 0.5 to 8 years later, sequential biopsies revealed neutrophilic dermal infiltration typical of SS.CONCLUSION: Initially lymphocytic infiltrates in this subset could be attributed either to an early timing of the biopsy concerning the age of the lesion or to the dysgranulopoiesis syndrome. A possible relationship between the dysfunction of the receptor of the granulocyte-macrophage colony stimulating factor, the gene of which is located on the pseudoautosomal X-Y region, may exist in MDS patients with initially lymphocytic SS. This could explain the male gender of this subset and might establish initially lymphocytic SS as a distinguished clinicopathological entity for predicting the occurrence and even the prognosis of MDS. |
DOI | 10.1159/000357933 |
Alternate Journal | Acta Haematol. |
PubMed ID | 24714374 |