Epstein–Barr virus-induced sickle hepatopathy

Alison S. Towerman; David B. Wilson; Monica L. Hulbert

doi:10.1002/pbc.29196

Epstein–Barr virus-induced sickle hepatopathy

Alison S. Towerman, David B. Wilson, Monica L. Hulbert

Research output: Contribution to journal › Article › peer-review

2 Scopus citations

Abstract

Sickle hepatopathy comprises a spectrum of disorders that vary in severity. Intravascular sickling and sinusoidal occlusion are the principal drivers of sickle hepatopathy, but infection or autoimmunity can act as triggers. We describe two cases of acute sickle hepatopathy initiated by primary Epstein–Barr virus (EBV) infection, a previously unreported association. The first case entailed a 14-year-old girl with hemoglobin SC (HbSC) disease who developed hepatic sequestration crisis that responded to a simple transfusion of erythrocytes. The second case was that of a 16-year-old boy with HbSC disease who experienced life-threatening intrahepatic cholestasis with multiorgan failure.

Original language	English
Article number	e29196
Journal	Pediatric Blood and Cancer
Volume	68
Issue number	9
DOIs	https://doi.org/10.1002/pbc.29196
State	Published - Sep 2021

Keywords

cholestasis
hemoglobin SC disease
hepatic sequestration
infectious mononucleosis
sickle cell disease

Access to Document

10.1002/pbc.29196

Cite this

@article{91421e1227f04a94a43ceae87d798215,

title = "Epstein–Barr virus-induced sickle hepatopathy",

abstract = "Sickle hepatopathy comprises a spectrum of disorders that vary in severity. Intravascular sickling and sinusoidal occlusion are the principal drivers of sickle hepatopathy, but infection or autoimmunity can act as triggers. We describe two cases of acute sickle hepatopathy initiated by primary Epstein–Barr virus (EBV) infection, a previously unreported association. The first case entailed a 14-year-old girl with hemoglobin SC (HbSC) disease who developed hepatic sequestration crisis that responded to a simple transfusion of erythrocytes. The second case was that of a 16-year-old boy with HbSC disease who experienced life-threatening intrahepatic cholestasis with multiorgan failure.",

keywords = "cholestasis, hemoglobin SC disease, hepatic sequestration, infectious mononucleosis, sickle cell disease",

author = "Towerman, {Alison S.} and Wilson, {David B.} and Hulbert, {Monica L.}",

note = "Funding Information: We thank the patients and their families. Publisher Copyright: {\textcopyright} 2021 Wiley Periodicals LLC",

year = "2021",

month = sep,

doi = "10.1002/pbc.29196",

language = "English",

volume = "68",

journal = "Pediatric Blood and Cancer",

issn = "1545-5009",

number = "9",

}

TY - JOUR

T1 - Epstein–Barr virus-induced sickle hepatopathy

AU - Towerman, Alison S.

AU - Wilson, David B.

AU - Hulbert, Monica L.

PY - 2021/9

Y1 - 2021/9

N2 - Sickle hepatopathy comprises a spectrum of disorders that vary in severity. Intravascular sickling and sinusoidal occlusion are the principal drivers of sickle hepatopathy, but infection or autoimmunity can act as triggers. We describe two cases of acute sickle hepatopathy initiated by primary Epstein–Barr virus (EBV) infection, a previously unreported association. The first case entailed a 14-year-old girl with hemoglobin SC (HbSC) disease who developed hepatic sequestration crisis that responded to a simple transfusion of erythrocytes. The second case was that of a 16-year-old boy with HbSC disease who experienced life-threatening intrahepatic cholestasis with multiorgan failure.

AB - Sickle hepatopathy comprises a spectrum of disorders that vary in severity. Intravascular sickling and sinusoidal occlusion are the principal drivers of sickle hepatopathy, but infection or autoimmunity can act as triggers. We describe two cases of acute sickle hepatopathy initiated by primary Epstein–Barr virus (EBV) infection, a previously unreported association. The first case entailed a 14-year-old girl with hemoglobin SC (HbSC) disease who developed hepatic sequestration crisis that responded to a simple transfusion of erythrocytes. The second case was that of a 16-year-old boy with HbSC disease who experienced life-threatening intrahepatic cholestasis with multiorgan failure.

KW - cholestasis

KW - hemoglobin SC disease

KW - hepatic sequestration

KW - infectious mononucleosis

KW - sickle cell disease

UR - http://www.scopus.com/inward/record.url?scp=85110862127&partnerID=8YFLogxK

U2 - 10.1002/pbc.29196

DO - 10.1002/pbc.29196

M3 - Article

C2 - 34180131

AN - SCOPUS:85110862127

SN - 1545-5009

VL - 68

JO - Pediatric Blood and Cancer

JF - Pediatric Blood and Cancer

IS - 9

M1 - e29196

ER -

Epstein–Barr virus-induced sickle hepatopathy

Abstract

Keywords

Access to Document

Other files and links

Fingerprint

Cite this