Incidence and treatment of hemophagocytic lymphohistiocytosis in hospitalized children with Ehrlichia infection

Pediatr Blood Cancer. 2020 Oct;67(10):e28436. doi: 10.1002/pbc.28436. Epub 2020 Jul 24.

Abstract

We report a large cohort of pediatric patients with human monocytic ehrlichiosis (HME), enabling an estimated incidence of secondary hemophagocytic lymphohistiocytosis (HLH) in hospitalized children with HME. Among 49 children with PCR-confirmed Ehrlichia infection, 8 (16%) met current criteria for HLH. Those with HLH had more significant hematologic abnormalities and longer durations from symptom onset to admission and definitive anti-infective therapy. Among these eight, three received chemotherapy plus doxycycline, one of whom died; the other five were treated with doxycycline without chemotherapy, and all survived without HLH recurrence. Our findings demonstrate that antimicrobial therapy alone can successfully resolve Ehrlichia-associated HLH.

Keywords: Ehrlichia; doxycycline; hemophagocytic lymphohistiocytosis; hyperferritinemia.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Anti-Bacterial Agents / therapeutic use*
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use*
  • Child
  • Child, Hospitalized / statistics & numerical data*
  • Child, Preschool
  • Doxycycline / therapeutic use*
  • Ehrlichia chaffeensis / isolation & purification*
  • Ehrlichiosis / complications*
  • Ehrlichiosis / drug therapy
  • Ehrlichiosis / microbiology
  • Female
  • Follow-Up Studies
  • Humans
  • Incidence
  • Infant
  • Lymphohistiocytosis, Hemophagocytic / drug therapy*
  • Lymphohistiocytosis, Hemophagocytic / epidemiology
  • Lymphohistiocytosis, Hemophagocytic / microbiology
  • Male
  • Missouri / epidemiology
  • Prognosis
  • Retrospective Studies

Substances

  • Anti-Bacterial Agents
  • Doxycycline