A Case of Hypereosinophilic Syndrome Presenting With Multiorgan Infarctions Associated With Disseminated Intravascular Coagulation

Abstract

Thromboembolism is one of the most critical complications of hypereosinophilic syndrome (HES). We report here a case of multi-organ infarctions related to HES. A 23-year-old woman was referred to our hospital with hemoptysis. Not only pulmonary, but also renal and splenic infarctions were detected on computed tomography images. Blood tests showed profound peripheral eosinophilia. She was diagnosed with HES with disseminated intravascular coagulation (DIC). We initiated infusion of corticosteroids, which effectively suppressed peripheral eosinophilia. However, consumptive coagulopathy did not improve and intracerebral hemorrhage related to thrombosis then developed. Addition of interferon-alpha resulted in the correction of the DIC associated with HES.

A Case of Idiopathic Hypereosinophilic Syndrome Presenting With Acute Respiratory Distress Syndrome

“…About 85% of patients on corticosteroid therapy reach partial or complete remission by 1 month 17. For patients not responding to corticosteroids, second-line therapies such as hydroxyurea, interferon-α, anti-IL5 antibodies, or anti-CD52 antibodies can be considered 18. In the present case, peripheral eosinophilia and involvement of the skin and lung were successfully treated by high-dose corticosteroid alone.…”

Idiopathic hypereosinophilic syndrome with multiple organ infarction: improvement of the skin condition masking the course of disease

“…These patients tended to have multiple organ involvement [8]. Recently a case similar to ours was published by SunMi Park et al [6]. This patient developed disseminated intravascular coagulation with cerebral hemorrhage and was treated with corticosteroids and interferon-alpha.…”

“…An overview of therapeutic approaches has recently been published [5]. In some cases of HES, multiple thromboembolic events have been described [6]. We report a patient with HES who developed multiple organ infarctions despite corticosteroid treatment.…”

Identification of a novel PDGFRA point mutation at p.P6L as a potential molecular target of imatinib in an eosinophilia patient showing genetic heterogeneity

“…Other reported complications include hepatitis-splenomegaly, eosinophilic gastroenteritis, and coagulation disorders. [2][3][4] The FIP1L1-PDGFRA fusion gene (F/P+ variant) is considered the benchmark molecular biomarker and has recently proven to be sensitive to treatment with tyrosine-kinase inhibitor drugs, such as imatinib (Gleevec and other brand names). 5,6 Since it has been difficult to identify a gene mutation or to prove eosinophil clonality in association with peripheral eosinophilia, it has been difficult to diagnose HES accurately.…”