Abstract 4142245: Eosinophilic Myocarditis and Duodenitis: A Unique Manifestation of Hypereosinophilic Syndrome

Description of Case: This case presents a 23-year-old man with history of sickle cell disease and allogeneic peripheral blood stem cell transplantation (SCT) who presented with symptoms of acute substernal chest pain, shortness of breath, abdominal pain, and vomiting. Methods: Performed a comprehensive retrospective analysis of one patient's medical history, clinical presentation, diagnostic assessment, therapeutic intervention, and clinical outcome. Results: Physical exam was remarkable for euvolemia. Electrocardiogram demonstrated sinus rhythm. Laboratory studies revealed a complete blood count significant for eosinophilia of 14,800. Troponins and BNP were elevated at 31 ng/mL and 98 pg/ml respectively. Echocardiogram showed normal biventricular systolic function. CT abdomen/pelvis with contrast showed findings suggestive of esophagitis and duodenal thickening. Biopsy of the duodenum revealed an increased number of eosinophils with scattered lymphocytes and plasma cells. Infectious work-up including stool ova and parasite, tryptase, vitamin B12, uric acid, and IgE were unremarkable. BCR-ABL, CHIC-2 deletion, and peripheral flow cytometry were negative. C-reactive protein was elevated. Cardiac MRI (CMR) findings demonstrated late gadolinium enhancement (LGE) of the mid-inferior segment of the left ventricle with moderate hypokinesis. Patient was treated with a short course of steroids. Repeat CMR 10 weeks later showed resolving LGE in the mid-inferior segment of the left ventricle. Discussion: CMR and duodenal biopsy demonstrated findings consistent with rare manifestation of HES with muti-organ inflammation as evident by left ventricular LGE, duodenal biopsy, and peripheral eosinophilia. Initial management involves prompt initiation of immunosuppressive therapy to mitigate eosinophilic infiltration and end-organ damage. Diagnostic modalities such as tissue biopsy, advanced imaging, and laboratory testing play crucial roles in confirming the diagnosis. Follow-up CMR can help follow the response treatment and rule-out confounding disease processes, as seen in our case. In conclusion, HES is a rare disorder with variable presentations. Eosinophilic myocarditis, a life-threatening sequala of HES, should be considered as a differential in patients that develop heart failure symptoms and eosinophilia after SCT.

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Journal
Circulation
Published
2024-11-12
DOI
https://doi.org/10.1161/circ.150.suppl_1.4142245
Primary Topic
Eosinophilic Disorders and Syndromes
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article
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Abstract 4142245: Eosinophilic Myocarditis and Duodenitis: A Unique Manifestation of Hypereosinophilic Syndrome

Yash Patel, Saurabh Agarwal, Arianne Sweeting, Edward Hulten et al.
Circulation
Eosinophilic Disorders and Syndromes
article

Abstract 4142245: Eosinophilic Myocarditis and Duodenitis: A Unique Manifestation of Hypereosinophilic Syndrome

Yash Patel, Saurabh Agarwal, Arianne Sweeting, Edward Hulten, Michael K. Atalay
article en

Abstract

Description of Case: This case presents a 23-year-old man with history of sickle cell disease and allogeneic peripheral blood stem cell transplantation (SCT) who presented with symptoms of acute substernal chest pain, shortness of breath, abdominal pain, and vomiting. Methods: Performed a comprehensive retrospective analysis of one patient's medical history, clinical presentation, diagnostic assessment, therapeutic intervention, and clinical outcome. Results: Physical exam was remarkable for euvolemia. Electrocardiogram demonstrated sinus rhythm. Laboratory studies revealed a complete blood count significant for eosinophilia of 14,800. Troponins and BNP were elevated at 31 ng/mL and 98 pg/ml respectively. Echocardiogram showed normal biventricular systolic function. CT abdomen/pelvis with contrast showed findings suggestive of esophagitis and duodenal thickening. Biopsy of the duodenum revealed an increased number of eosinophils with scattered lymphocytes and plasma cells. Infectious work-up including stool ova and parasite, tryptase, vitamin B12, uric acid, and IgE were unremarkable. BCR-ABL, CHIC-2 deletion, and peripheral flow cytometry were negative. C-reactive protein was elevated. Cardiac MRI (CMR) findings demonstrated late gadolinium enhancement (LGE) of the mid-inferior segment of the left ventricle with moderate hypokinesis. Patient was treated with a short course of steroids. Repeat CMR 10 weeks later showed resolving LGE in the mid-inferior segment of the left ventricle. Discussion: CMR and duodenal biopsy demonstrated findings consistent with rare manifestation of HES with muti-organ inflammation as evident by left ventricular LGE, duodenal biopsy, and peripheral eosinophilia. Initial management involves prompt initiation of immunosuppressive therapy to mitigate eosinophilic infiltration and end-organ damage. Diagnostic modalities such as tissue biopsy, advanced imaging, and laboratory testing play crucial roles in confirming the diagnosis. Follow-up CMR can help follow the response treatment and rule-out confounding disease processes, as seen in our case. In conclusion, HES is a rare disorder with variable presentations. Eosinophilic myocarditis, a life-threatening sequala of HES, should be considered as a differential in patients that develop heart failure symptoms and eosinophilia after SCT.

CirculationVol. 150(Suppl_1)
Rhode Island Hospital (US)
Good health and well-being
Openalex Percentile: Top 64%
Eosinophilic Disorders and Syndromes
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