Sickle cell disease

Two fatalities due to bone marrow emboli in patients with acute chest syndrome

Dean A. Hawley, Leo J. McCarthy

Research output: Contribution to journalArticle

5 Citations (Scopus)

Abstract

Acute chest syndrome (ACS) in patients with sickle cell disease (SCD) is a common complication contributing to death. ACS can present as sudden, unexpected death, and the medical history of SCD may not be immediately available for the medical examiner. Forensic implications for ACS are more likely to be encountered in patients with the HgbSC variant of SCD because the presence of a spleen may obscure recognition of SCD. Pathologists commonly observe small bone marrow emboli within the lung vasculature postmortem after trauma or vigorous cardiopulmonary resuscitation. Consequently, the finding of pulmonary marrow emboli in ACS may be dismissed as incidental and nonpathologic. Pulmonary marrow emboli cause ACS in patients with SCD, when there is parvoviral infection with marrow necrosis. Pulmonary marrow embolism in ACS has special significance in forensic pathology. Forensic pathologists investigate sudden, unexpected deaths during exertion, such as athletics, police pursuit, or military training. A causal relationship linking sickling hemoglobinopathy with physical exertion and sudden death has been reported. In the absence of a specific and timely medical history of SCD, and with a normal or enlarged spleen in patients with HgbSC, pathologists may wrongly dismiss bone marrow emboli as consequences of cardiopulmonary resuscitation, when those emboli might actually have been causative of fatal ACS.

Original languageEnglish
Pages (from-to)69-71
Number of pages3
JournalAmerican Journal of Forensic Medicine and Pathology
Volume30
Issue number1
DOIs
StatePublished - Mar 2009

Fingerprint

Acute Chest Syndrome
Sickle Cell Anemia
Embolism
Bone Marrow
Sudden Death
Cardiopulmonary Resuscitation
Lung
Forensic Pathology
Physical Exertion
Hemoglobinopathies
Coroners and Medical Examiners
Splenomegaly
Police
Pulmonary Embolism
Sports
Necrosis
Spleen
History
Wounds and Injuries

Keywords

  • Acute chest syndrome
  • Bone marrow embolism
  • Fat embolism
  • Hemoglobinopathy
  • Sickle cell disease

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Medicine(all)

Cite this

Sickle cell disease : Two fatalities due to bone marrow emboli in patients with acute chest syndrome. / Hawley, Dean A.; McCarthy, Leo J.

In: American Journal of Forensic Medicine and Pathology, Vol. 30, No. 1, 03.2009, p. 69-71.

Research output: Contribution to journalArticle

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