Childhood Blastic Plasmacytoid Dendritic Cell Neoplasm Mimicking Acute Rheumatic Fever: Report of an Unusual Clinical Presentation and Review of Literature

J Pediatr Hematol Oncol. 2018 Jul;40(5):e327-e329. doi: 10.1097/MPH.0000000000000951.

Abstract

Background: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is very rarely diagnosed in children with less than 50 cases in the literature.

Observation: We report a case of childhood BPDCN who mimicked acute rheumatic fever at presentation. Majority of the reported childhood BPDCN received acute lymphoblastic leukemia-like chemotherapy with/without stem cell therapy, whereas those who received acute myeloid leukemia-like therapy predominantly succumbed to disease or sepsis. Overall 68% of the patients were alive and achieved complete remission with an overall prognosis slightly better in children compared with adults.

Conclusions: The case is reported due to its unique unusual clinical presentation and its rarity in pediatric population.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Allografts
  • Antineoplastic Agents / administration & dosage*
  • Child
  • Dendritic Cells*
  • Diagnosis, Differential
  • Female
  • Hematologic Neoplasms / diagnosis*
  • Hematologic Neoplasms / therapy*
  • Humans
  • Rheumatic Fever / diagnosis*
  • Rheumatic Fever / therapy*
  • Stem Cell Transplantation*

Substances

  • Antineoplastic Agents