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CARDIOVASCULAR JOURNAL OF AFRICA • Volume 31, No 5, September/October 2020
274
AFRICA
Case Reports
Idiopathic hypereosinophilic syndrome associated
with rapid progression of cardiac, pulmonary and skin
infiltration
Yu-Quan He, Jin-Ming Zhu, Ya-Liang Tong, Hong Zeng, Ping Yang
Abstract
Idiopathic hypereosinophilic syndrome (IHES) is a rare
myeloproliferative disease characterised by multisystem
dysfunction and persistent, extreme eosinophilia of unknown
cause. Here we present a 42-year-old patient complaining
of moderate to severe chest pain and shortness of breath,
and typical ischaemic electrocardiography changes were
recorded. He was initially suspected of having acute coronary
syndrome, however the coronary angiogram excluded coro-
nary abnormalities. Bone marrow biopsy, left ventriculogra-
phy, transthoracic echocardiography and cardiac magnetic
resonance examinations confirmed the diagnosis of IHES
and IHES-mediated cardiac involvement. The patient’s illness
was alleviated during the first hospitalisation, whereas it
had rapidly worsened one month after discharge. In addi-
tion, simultaneous pulmonary and skin-infiltrating lesions
occurred during the second hospitalisation. The patient’s
condition improved markedly with combined glucocorticoid,
hydroxyurea and warfarin therapy, as well as treatment for
heart failure. In this report the diagnostic modalities and
treatment strategies for IHES are discussed and reviewed.
Keywords:
idiopathic hypereosinophilic syndrome, cardiac
involvement, pulmonary, skin, case report
Submitted 28/10/19, accepted 13/4/20
Published online 12/6/20
Cardiovasc J Afr
2020;
31
: 274–280
www.cvja.co.zaDOI: 10.5830/CVJA-2020-009
Idiopathic hypereosinophilic syndrome (IHES) is a rare
myeloproliferative disorder with unknown aetiology,
characterised by persistent hypereosinophilia accompanied by
multiple eosinophil-mediated organ injuries.
1-3
It was initially
reported by Anderson and Hardy
4
in 1968, and Clusid
et al
.
1
in 1975 defined the following clinical diagnostic criteria: (1)
absolute peripheral eosinophil count > 1.5 × 10
9
cells/l for at least
six months; (2) evidence of one or multiple eosinophil-mediated
organ damage; and (3) exclusion of allergic, parasitic, drug and
connective tissue disorders, rheumatism, malignancies or any
other cause for secondary hypereosinophilia.
It has been reported that eosinophil-mediated cardiac
involvement occurs in more than 50% of IHES patients and is
a major cause of death.
5-7
In addition, eosinophil-mediated lung
and skin damage is occasionally reported in the literature.
8,9
We present a rare case of IHES that was accompanied by
a simultaneous occurrence of left ventricular (LV) myocardial
necrosis, endomyocardial fibrosis, intra-cardiac thrombi, as
well as pulmonary and skin-infiltrating lesions. The patient
was initially suspected of having acute coronary syndrome
due to the complaints of chest pain, ischaemic changes on
electrocardiogram (ECG), and abnormal biomarkers for blunt
cardiac injury. However, a negative coronary angiogram excluded
coronary artery anomalies. The patient’s condition deteriorated
within one month of discharge, whereas it improved markedly
with combined therapy of glucocorticoids, hydroxyurea and
warfarin, as well as treatment for heart failure. In this report
diagnostic modalities and treatment strategies for IHES are
discussed and reviewed.
Case report
A 42-year-old male Chinese farmer was admitted to the
emergency department of the China–Japan Union Hospital
of Jilin University, complaining of intermittent moderate to
severe chest pain and shortness of breath of one year duration,
associated with intermittent fever (37–39°C) and fatigue over
two weeks. He had lost 25 kg weight in the past three months.
He had smoked 20 cigarettes per day for more than 20 years. His
past medical history was unremarkable with no similar disease
found in his family.
On examination, his body temperature was 37.2°C and blood
pressure was 103/60 mmHg. He had no other positive findings
except for a mild holosystolic blowing murmur with 2/6 degree
that was heard at the mitral valve area. The 12-lead ECG showed
Department of Cardiology, China–Japan Union Hospital of
Jilin University, Cardiovascular Institute of Jilin Province,
Changchun 130033, Jilin Province, China
Yu-Quan He, MD, PhD
Jin-Ming Zhu, MD, PhD
Ya-Liang Tong, MD, PhD
Hong Zeng, MD, PhD,
zenghong@jlu.edu.cnPing Yang, MD, PhD