Refining the value of secretory phospholipase A 2 as a predictor of acute chest syndrome in sickle cell disease: Results of a feasibility study (PROACTIVE)

Lori Styles*, Carrie G. Wager, Richard J. Labotka, Kim Smith-Whitley, Alexis A. Thompson, Peter A. Lane, Lillian E.C. McMahon, Robin Miller, Susan D. Roseff, Rathi V. Iyer, Lewis L. Hsu, Oswaldo L. Castro, Kenneth I. Ataga, Onyinye Onyekwere, Maureen Okam, Rita Bellevue, Scott T. Miller

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

38 Scopus citations


Acute chest syndrome (ACS) is defined as fever, respiratory symptoms and a new pulmonary infiltrate in an individual with sickle cell disease (SCD). Nearly half of ACS episodes occur in SCD patients already hospitalized, potentially permitting pre-emptive therapy in high-risk patients. Simple transfusion of red blood cells may abort ACS if given to patients hospitalized for pain who develop fever and elevated levels of secretory phospholipase A2 (sPLA2). In a feasibility study (PROACTIVE; NCT00951808), patients hospitalized for pain who developed fever and elevated sPLA2 were eligible for randomization to transfusion or observation; all others were enrolled in an observational arm. Of 237 enrolled, only 10 were randomized; one of the four to receive transfusion had delayed treatment. Of 233 subjects receiving standard care, 22 developed ACS. A threshold level of sPLA2 ≥ 48 ng/ml gave optimal sensitivity (73%), specificity (71%) and accuracy (71%), but a positive predictive value of only 24%. The predictive value of sPLA2 was improved in adults and patients with chest or back pain, lower haemoglobin concentration and higher white blood cell counts, and in those receiving less than two-thirds maintenance fluids. The hurdles identified in PROACTIVE should facilitate design of a larger, definitive, phase 3 randomized controlled trial.

Original languageEnglish (US)
Pages (from-to)627-636
Number of pages10
JournalBritish Journal of Haematology
Issue number5
StatePublished - Jun 2012


  • Acute chest syndrome
  • Pulmonary fat embolism
  • SPLA2
  • Transfusion
  • Vaso-occlusive episode

ASJC Scopus subject areas

  • Hematology

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