Distinctive Acellular Lipid Emboli in Hemoglobin SC Disease following Bone Marrow Infarction with Parvovirus Infection

An adolescent with mild hemoglobin SC disease presented with pelvic pain with subsequent respiratory and neurologic deterioration, which led to ultimately death. The autopsy demonstrated acellular fat emboli particularly in the lung and brain. There was marrow necrosis in the lumbar spine with aggre...

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Main Authors: Danielle M. Graff, Erin Owen, Robert Bendon, Salvatore Bertolone, Ashok Raj
Format: Article
Language:English
Published: Wiley 2015-01-01
Series:Case Reports in Hematology
Online Access:http://dx.doi.org/10.1155/2015/328065
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author Danielle M. Graff
Erin Owen
Robert Bendon
Salvatore Bertolone
Ashok Raj
author_facet Danielle M. Graff
Erin Owen
Robert Bendon
Salvatore Bertolone
Ashok Raj
author_sort Danielle M. Graff
collection DOAJ
description An adolescent with mild hemoglobin SC disease presented with pelvic pain with subsequent respiratory and neurologic deterioration, which led to ultimately death. The autopsy demonstrated acellular fat emboli particularly in the lung and brain. There was marrow necrosis in the lumbar spine with aggregated sickle cells and positive parvovirus immunostaining. The brain lesion both grossly and microscopically presented a distinct pathology of acellular fat emboli that led to the correct diagnosis of this increasingly recognized association of sickle hemoglobinopathies with fat embolism syndrome (FES). A clinical diagnosis of FES is difficult to confirm in many patients with sickle hemoglobinopathy presenting with pain crisis because of concurrent illness. However, this case report highlights the need for a thorough knowledge of the signs and symptoms of the syndrome and a high index of suspicion for the diagnosis to be made premortem.
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spelling doaj-art-f2fcdcaca5ab4206af5264f0007c30f62025-02-03T01:11:21ZengWileyCase Reports in Hematology2090-65602090-65792015-01-01201510.1155/2015/328065328065Distinctive Acellular Lipid Emboli in Hemoglobin SC Disease following Bone Marrow Infarction with Parvovirus InfectionDanielle M. Graff0Erin Owen1Robert Bendon2Salvatore Bertolone3Ashok Raj4University of Louisville, 571 S. Floyd Street, Suite 300, Louisville, KY 40202, USAUniversity of Louisville, 571 S. Floyd Street, Suite 300, Louisville, KY 40202, USAPathology, Norton Healthcare, Louisville, KY, USAUniversity of Louisville, 571 S. Floyd Street, Suite 300, Louisville, KY 40202, USAUniversity of Louisville, 571 S. Floyd Street, Suite 300, Louisville, KY 40202, USAAn adolescent with mild hemoglobin SC disease presented with pelvic pain with subsequent respiratory and neurologic deterioration, which led to ultimately death. The autopsy demonstrated acellular fat emboli particularly in the lung and brain. There was marrow necrosis in the lumbar spine with aggregated sickle cells and positive parvovirus immunostaining. The brain lesion both grossly and microscopically presented a distinct pathology of acellular fat emboli that led to the correct diagnosis of this increasingly recognized association of sickle hemoglobinopathies with fat embolism syndrome (FES). A clinical diagnosis of FES is difficult to confirm in many patients with sickle hemoglobinopathy presenting with pain crisis because of concurrent illness. However, this case report highlights the need for a thorough knowledge of the signs and symptoms of the syndrome and a high index of suspicion for the diagnosis to be made premortem.http://dx.doi.org/10.1155/2015/328065
spellingShingle Danielle M. Graff
Erin Owen
Robert Bendon
Salvatore Bertolone
Ashok Raj
Distinctive Acellular Lipid Emboli in Hemoglobin SC Disease following Bone Marrow Infarction with Parvovirus Infection
Case Reports in Hematology
title Distinctive Acellular Lipid Emboli in Hemoglobin SC Disease following Bone Marrow Infarction with Parvovirus Infection
title_full Distinctive Acellular Lipid Emboli in Hemoglobin SC Disease following Bone Marrow Infarction with Parvovirus Infection
title_fullStr Distinctive Acellular Lipid Emboli in Hemoglobin SC Disease following Bone Marrow Infarction with Parvovirus Infection
title_full_unstemmed Distinctive Acellular Lipid Emboli in Hemoglobin SC Disease following Bone Marrow Infarction with Parvovirus Infection
title_short Distinctive Acellular Lipid Emboli in Hemoglobin SC Disease following Bone Marrow Infarction with Parvovirus Infection
title_sort distinctive acellular lipid emboli in hemoglobin sc disease following bone marrow infarction with parvovirus infection
url http://dx.doi.org/10.1155/2015/328065
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