Acute febrile neutrophilic dermatosis (sweet syndrome) in acute myeloid leukemia patients: A 28-year institutional experience Journal Article


Authors: Cowen, E. A.; Barrios, D. M.; Pulitzer, M. P.; Moy, A. P.; Dusza, S. W.; De Wolf, S.; Geyer, M. B.; Markova, A.
Article Title: Acute febrile neutrophilic dermatosis (sweet syndrome) in acute myeloid leukemia patients: A 28-year institutional experience
Abstract: Introduction: Sweet syndrome (SS) is well known to be associated with underlying hematologic malignancies. The incidence and qualities of SS among novel targeted therapies for acute myeloid leukemia (AML) have not yet been described. Methods: Through retrospective review of 19,432 patients diagnosed with acute/chronic leukemia or myelodysplastic syndromes/myeloproliferative neoplasms (MDS+/-MPN) over 28 years, we calculated the incidence of SS in the setting of select hematologic malignancies and described the clinicopathologic characteristics of SS in patients with onset of SS after initiation of novel AML-targeted therapies. Results: Overall incidence of SS was 0.36% (95% CI: 0.27-0.45%), which was significantly higher among patients with AML (50/ 5,248, 0.94%; 95% CI: 0.71-1.25%). Nine AML patients were on 4 classes of novel targeted treatments - IDH1/2 inhibitor alone, FLT3 inhibitor, IDH2 and DOT1L inhibitor, and anti-CD33 therapy. In therapies inducing myeloid blast differentiation, SS occurred at later onset following treatment. Conclusions: In AML patients with fever and unusual skin lesions, physicians may consider SS earlier, which may shorten time to diagnosis. Future assessments of SS among patients treated with novel therapies for AML and molecular studies of biopsies may help further explain this dermatologic adverse event with earlier diagnosis and management of neutrophilic dermatoses in these patients. © 2023 S. Karger AG, Basel.
Keywords: adolescent; adult; aged; aged, 80 and over; middle aged; leukemia; retrospective studies; unclassified drug; major clinical study; leukemia, myeloid, acute; prednisone; clinical feature; histopathology; diarrhea; drug dose reduction; monotherapy; side effect; systemic therapy; chemotherapy; follow up; edema; pain; vomiting; incidence; protein; dexamethasone; cell differentiation; pathology; chronic myeloid leukemia; retrospective study; acute lymphoblastic leukemia; abdominal pain; coughing; drug dose escalation; fever; pneumonia; purpura; cause of death; cancer regression; myelodysplastic syndrome; neutrophil; diagnosis; erythema; allogeneic hematopoietic stem cell transplantation; targeted therapy; methylprednisolone; papule; bone marrow cell; histiocyte; histiocytosis; hydrocortisone; corticosteroid; leukemia relapse; melena; chronic lymphatic leukemia; lymphocyte; topical treatment; ulcer; myeloproliferative neoplasm; cd33 antigen; leukemia remission; clobetasol; triamcinolone; blast cell; etiology; molecularly targeted therapy; pustule; isocitrate dehydrogenase 1; cd135 antigen; complication; eosinophil; vasculitis; trunk; corticosteroid therapy; acute myeloid leukemia; mortality rate; sweet syndrome; acute febrile neutrophilic dermatosis; hidradenitis; lintuzumab; neutrophilia; upper limb; isocitrate dehydrogenase 2; quizartinib; very elderly; humans; human; male; female; article; pinometostat; gilteritinib; enasidenib; lower limb; ivosidenib; neutrophilic dermatosis; dot1l protein
Journal Title: Acta Haematologica
Volume: 147
Issue: 4
ISSN: 0001-5792
Publisher: S. Karger AG  
Date Published: 2024-07-01
Start Page: 457
End Page: 464
Language: English
DOI: 10.1159/000535399
PUBMED: 37989105
PROVIDER: scopus
PMCID: PMC11740260
DOI/URL:
Notes: The MSK Cancer Center Support Grant (P30 CA008748) is acknowledged in the PDF. Corresponding MSK author is Alina Markova -- Source: Scopus
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MSK Authors
  1. Melissa P Pulitzer
    205 Pulitzer
  2. Stephen Dusza
    289 Dusza
  3. Mark Blaine Geyer
    89 Geyer
  4. Dulce M Barrios
    13 Barrios
  5. Alina Markova
    87 Markova
  6. Andrea Primiani Moy
    32 Moy
  7. Susan E Dewolf
    50 Dewolf
  8. Emily Ann Cowen
    9 Cowen