Document Type

Conference Proceeding

Publication Date

5-2-2025

Abstract

Introduction Hypereosinophilic syndrome (HES) is a rare condition characterized by eosinophil-mediated organ damage and a peripheral eosinophil count >1.5x10⁹/L, with varied etiologies classified as primary, secondary, idiopathic, or organ-restricted. Cardiac involvement, often termed Loeffler’s endocarditis (LE), is a rare but severe manifestation of HES. Neoplastic causes of HES are typically linked to myeloid neoplasms, with limited literature on an association with B-cell lymphomas. We report a unique case of HES and LE in a patient with diffuse large B-cell lymphoma (DLBCL). Case description A 78-year-old female with a past medical history of DLBCL, coronary artery disease, and cryptogenic strokes presented with acute dyspnea and wheezing for 18 hours before arrival. Diagnosed with DLBCL 20 years earlier, she had failed multiple therapies and was on lenalidomide and obinutuzumab at the time of presentation. Her other medications included clopidogrel and apixaban. On arrival, the patient’s blood pressure was 98/59 mmHg, heart rate 80 beats per minute, and oxygen saturation 90% on room air. Examination revealed diffuse wheezing, and laboratory studies showed leukocytosis with an absolute eosinophil count of 6.5 × 10⁹/L (0.02–0.52). Imaging ruled out pulmonary embolism but suggested congestive heart failure. Echocardiography identified a 2.5 × 3.3 cm laminar left ventricular thrombus with an ejection fraction (EF) of 55%. Persistent eosinophilia documented over the past four years raised suspicion for HES with LE. Management included IV methylprednisolone, heparin, and diuresis. Extensive workup for her eosinophilia which included molecular genetics testing was negative. The etiology was attributed to neoplastic HES secondary to DLBCL. Cardiac magnetic resonance imaging confirmed LE. In addition to steroids, hydroxyurea was started and her eosinophil count decreased to 3 x 10^9/L at discharge on day 16 with significant improvement in symptoms. A week after discharge (30 days from initial presentation), the patient was re-admitted with heart failure with mildly reduced EF of 45%. This hospitalization was complicated by pancytopenia and septic shock, and the patient's clinical status rapidly deteriorated despite appropriate therapy. Considering her advanced lymphoma and multiple comorbidities, the patient transitioned to comfort care and passed away 43 days after her initial presentation. Discussion The incidence of HES is reported to be 0.03-0.04 per 100,000 person-years. HES is organ damage attributable to persistent peripheral eosinophilia. While almost half of HES etiologies are idiopathic, many primary and secondary causes are attributable to myeloproliferative and lymphocytic variants respectively. The lymphocytic variant of HES typically refers to T-cell lymphomas, with rare case reports of B-cell lymphomas in the literature. To our knowledge, this is the first case report of HES manifesting as LE in a patient with DLBCL. LE often leads to heart failure from restrictive cardiomyopathy and intra-cardiac thrombi with a high mortality rate. This case underscores the rare association between DLBCL and HES, bringing into focus the presentation, diagnosis, and management of LE. A high index of suspicion with timely initiation of treatment is paramount to improve mortality, with close monitoring of complications.

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American College of Physicians Michigan Chapter and Society of Hospital Medicine Michigan Chapter Resident and Medical Student Day, May 2, 2025, Troy, MI

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