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Phospholipase A2 levels in acute chest syndrome of sickle cell disease
LA Styles, CG Schalkwijk, AJ Aarsman, EP Vichinsky, BH Lubin and FA Kuypers
Department of Hematology/Oncology, Children's Hospital Oakland, CA 94609
USA.
Acute chest syndrome (ACS) is associated with significant morbidity and is
the leading cause of death in patients with sickle cell disease (SCD).
Recent reports suggest that bone marrow fat embolism can be detected in
many cases of severe ACS. Secretory phospholipase A2 (sPLA2) is an
important inflammatory mediator and liberates free fatty acids, which are
felt to be responsible for the acute lung injury of the fat embolism
syndrome. We measured SPLA2 levels in 35 SCD patients during 20 admissions
for ACS, 10 admissions for vaso-occlusive crisis, and during 12 clinic
visits when patients were at the steady state. Eleven non-SCD patients with
pneumonia were also evaluated. To determine if there was a relationship
between sPLA2 and the severity of ACS we correlated SPLA2 levels with the
clinical course of the patient. In comparison with normal controls (mean =
3.1 +/- 1.1 ng/mL), the non- SCD patients with pneumonia (mean = 68.6 +/-
82.9 ng/mL) and all three SCD patient groups had an elevation of SPLA2
(steady state mean = 10.0 +/- 8.4 ng/mL; vaso-occlusive crisis mean = 23.7
+/- 40.5 ng/mL; ACS mean = 336 +/- 209 ng/mL). In patients with ACS sPLA2
levels were 100- fold greater than normal control values, 35 times greater
than values in SCD patients at baseline, and five times greater than
non-SCD patients with pneumonia. The degree of SPLA2 elevation in ACS
correlated with three different measures of clinical severity and, in
patients followed sequentially, the rise in SPLA2 coincided with the onset
of ACS. The dramatic elevation of SPLA2 in patients with ACS but not in
patients with vaso-occlusive crisis or non-SCD patients with pneumonia and
the correlation between levels of SPLA2 and clinical severity suggest a
role for SPLA2 in the diagnosis and, perhaps, in the pathophysiology of
patients with ACS.
Volume 87,
Issue 6,
pp. 2573-2578,
03/15/1996
Copyright © 1996 by The American Society of Hematology
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