Characteristics of Sweet Syndrome in patients with acute myeloid leukemia.

BACKGROUND

Sweet syndrome (SS) is associated with hematologic malignancies including acute myeloid leukemia (AML).

METHODS

Records of patients with AML treated at our institution were reviewed to identify those with SS. Patient characteristics, laboratory values, and cytogenetic and molecular abnormalities were retrospectively reviewed.

RESULTS

We identified 21 of 2178 (1%) AML patients who demonstrated clinical signs and symptoms, and histological features consistent with SS. Eleven patients (52%) were classified as AML with myelodysplasia-related features and 3 patients had therapy-related AML. Three patients had received treatment with granulocyte colony stimulation factor, 1 patient liposomal all-trans-retinoic acid, and 2 patients received hypomethylating agents before development of SS. Cytogenetic analysis revealed diploid karyotype in 7 patients (33%); -5/del(5q) in 8 patients (38%): 3 patients had -5/del(5q) as the sole abnormality and 5 patients had -5/del(5q) as part of complex cytogenetics; and complex cytogenetics in 5 patients (24%). Gene mutations in FMS-related tyrosine kinase-3 (FLT3) gene were identified in 7 of 18 evaluable patients (39%), including FLT3-internal tandem duplication in 4 patients and FLT3-D835 tyrosine kinase domain mutation in 3 patients.

CONCLUSIONS

SS occurs in 1% of AML patients; -5/del(5q) karyotype, FLT3 mutations, and AML with myelodysplasia-related features were more frequent among patients with SS.