https://www.atsjournals.org/doi/pdf/10.1164/ajrccm-conference.2024.209.1_MeetingAbstracts.A5307
Am J Respir Crit Care Med 2024;209:A5307. C32 ALL THAT WHEEZES (AND THEN SOME): CASE STUDIES / Thematic Poster Session / Tuesday, May 21/09:15 AM-04:15 PM / San Diego Convention Center, Area H (Hall H, Ground Level)
M. Mallozzi, J. F. Most
Precise diagnosis of systemic eosinophilic disorders can be difficult due to overlapping features but is important in guiding specific therapies 1 . Anti-IL-5 therapies have been studied in several eosinophilic disorders and may be one option in situations of uncertainty 2 . As frequent prescribers of anti-IL-5 therapies, pulmonologists are essential in the management decisions surrounding these medications.We present a case of a male presenting in his sixth decade of life with respiratory symptoms and peripheral eosinophilia responsive to anti-IL-5 therapies who recurrence of his disease when therapies were stopped surrounding a stem cell transplant (SCT). The patient first presented to our institution with Medical Oncology to evaluate eosinophilia (6,440 cells/microliter) associated with cough, pruritic rash, and sinusitis. A bone marrow biopsy showed a hypercellular marrow with increased eosinophils and degenerated mast cells with normal cKit, PDGFRA/B, and FGFR1. A diagnosis of systemic mastocytosis was made. He was treated at that time symptomatically with partial response. Bronchoscopy revealed 35% eosinophils with lymphocytic and eosinophilic interstitial inflammation. He received four cycles of cladribine for progressive eosinophilia. Mepolizumab 300mg every 4 weeks was later added by his outside pulmonologist for possible eosinophilic granulomatosis with polyangiitis. His eosinophil counts normalized, but he developed neutropenia. A bone marrow biopsy showed evidence of myelodysplastic syndrome, for which he received one cycle of decitabine plus cedazuridine prior to undergoing SCT. A post-transplant bone marrow biopsy showed no evidence of systemic mastocytosis despite a rise in the peripheral eosinophil count. On day 104 post-transplant, he was admitted to the hospital with severe cough, dyspnea, hypoxemia, peripheral eosinophilia, and diffuse pulmonary infiltrates. Bronchoscopy revealed 37% eosinophils with a negative infectious workup. Intravenous glucocorticoids were started followed by Benralizumab 30mg subcutaneously. His clinical status continued to worsen and he developed fatal respiratory failure and refractory shock. This case represents a pulmonary manifestation of a rare disease with diagnostic uncertainty and important therapeutic implications. Anti-IL-5 therapy presented a feasible therapeutic option for the pulmonary manifestations of an eosinophilic disorder in the absence of molecular markers to guide management. Continued research is needed to guide the use of anti-IL-5 therapies in patients with eosinophilic myeloproliferative disorders and those undergoing stem cell transplant. 1. Shomali W, Gotlib J. World Health Organization‐defined eosinophilic disorders: 2022 update on diagnosis, risk stratification, and management. Am J Hematol. 2022;97(1):129-148. doi:10.1002/ajh.26352 2. Roufosse F. Targeting the Interleukin-5 Pathway for Treatment of Eosinophilic Conditions Other than Asthma. Front Med. 2018;5:49. doi:10.3389/fmed.2018.00049
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