Allergy Asthma Respir Dis.  2017 Jul;5(4):232-236. 10.4168/aard.2017.5.4.232.

Myeloid and lymphoid neoplasm with eosinophilia and abnormalities of PDGFRB presenting as congestive heart failure and hypereosinophilia

• Kwon JW
• Kwon JH
• Her AY

• KMID: 2387653
• DOI: http://doi.org/10.4168/aard.2017.5.4.232

Abstract

Hypereosinophilic syndrome (HES) is a heterogeneous disorder characterized by persistent hypereosinophilia with the evidence of organ dysfunction caused by eosinophilic involvement. HES can be induced by various secondary causes, including helminthic infections, adverse drug reactions, and allergic diseases. Primary/clonal bone marrow disease, including genetic mutations in platelet driven growth factor receptor alpha (PDGFRA), platelet driven growth factor receptor beta (PDGFRB), and fibroblast growth factor receptor 1 (FGFR1) could be its causes. Although corticosteroids are the mainstay of therapy in confirmed HES, imatinib is considered a definitive treatment for HES with these mutations. However, there have been few reports about HES with these genetic mutations in Korea. Here, we report a patient who presented with sudden onset of congestive heart failure and hypereosinophilia, proved to have PDGFRB rearrangement, and was controlled successfully with imatinib after left ventricle thrombectomy.

Keyword

Hypereosinophilic syndrome; PDGFRB; Imatinib; Eosinophilia

MeSH Terms

Adrenal Cortex Hormones
Blood Platelets
Bone Marrow Diseases
Drug-Related Side Effects and Adverse Reactions
Eosinophilia*
Eosinophils
Estrogens, Conjugated (USP)*
Heart Failure*
Heart Ventricles
Helminths
Humans
Hypereosinophilic Syndrome
Imatinib Mesylate
Korea
Receptor, Fibroblast Growth Factor, Type 1
Receptor, Platelet-Derived Growth Factor beta*
Thrombectomy
Adrenal Cortex Hormones
Estrogens, Conjugated (USP)
Imatinib Mesylate
Receptor, Fibroblast Growth Factor, Type 1
Receptor, Platelet-Derived Growth Factor beta

Figure

• 

  Fig. 1. Transthoracic echocardiography image showing cardiac involvement of hypereosinophilia. (A) Parasternal long-axis view demonstrates a hyperechoic mobile mass (2.0×0.6-cm size) suggesting thrombus (white arrow) at the postero-lateral myocardial wall. (B) Apical 4-chamber view demonstrates a large thrombus filling the left ventricular cavity (yellow arrows) and a hyper-mobile thrombus (white arrow) at the myocardial wall. This view also shows the endomyocardial fibrosis extend-ing along lateral wall of the ventricle (arrowheads). (C) The posterior mitral valve leaflet has suffered tissue injury and it has developed moderate to severe eccentric mitral regurgitation (color doppler).

• 

  Fig. 2. Clinical course and medications. LV, left ventricle.

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