ATS 2021 will feature presentations of original research from accepted abstracts. Mini Symposia and Thematic Poster Sessions are abstract based sessions.
Please use the form below to browse scientific abstracts and case reports accepted for ATS 2021. Abstracts presented at the ATS 2021 will be published in the Online Abstract Issue of the American Journal of Respiratory and Critical Care Medicine, Volume 203, May 3, 2021.
Search Tips:
- Use the keyword search to search by keyword or author's name.
- Filter your search results by selecting the checkboxes that apply.
- Click on "Clear" to clear the form and start a new search. .
Search results will display below the form.
Chronic Eosinophilic Leukemia Presenting as Asthma
Session Title
TP7 - TP007 PULMONARY AND ALLERGY CASE REPORTS
Abstract
A1366 - Chronic Eosinophilic Leukemia Presenting as Asthma
Author Block: J. Johnstone1, L. Thomas2; 1Department of Internal Medicine, The University of Kansas Medical Center, Kansas City, KS, United States, 2The University of Kansas Medical Center, Kansas City, KS, United States.
IntroductionChronic eosinophilic leukemia is a rare myeloproliferative disorder producing pronounced peripheral eosinophilia and end organ damage. Multisystem involvement is common and may involve the skin, gastrointestinal tract, lungs, heart, and central nervous system. We present a unique case of chronic eosinophilic leukemia manifesting as adult-onset moderate persistent asthma. Case Description: A 72-year-old man with a past medical history of hypertension presented with a 2-year history of progressive shortness of breath, cough, and paroxysmal wheezing. He is a farmer, never-smoker, and has no prior history of lung disease, including no childhood asthma. He was prescribed albuterol and fluticasone several months prior with moderate relief. Due to significant peripheral eosinophilia (70% of WBC 22.1), he received a hematologic workup and a diagnosis of FIP1L1-PDGFRA positive chronic hypereosinophilic leukemia. Prior to treatment with imatinib, spirometry displayed an obstructive pattern (FEV1/FVC 61%, FEV1 2.77L (86% predicted), FVC 4.55L (105% predicted)). CT imaging without consolidating infiltrates, fibrosis or bronchiectasis. He was prescribed inhaled fluticasone, montelukast, and albuterol. He was also initiated imatinib 400mg daily by hematology and at two months he had markedly decreased eosinophilia (6.5% of WBC of 7.4) with improved respiratory symptoms. Repeat spirometry demonstrated resolution of obstructive airway disease (FEV1 3.22L, 110% predicted). His wheezing and cough have nearly resolved with imatinib, inhaled corticosteroid and montelukast. DiscussionChronic eosinophilic leukemia (CEL) is a rare myeloproliferative disorder with pulmonary manifestations in 25-67% of patients. The broad family of hypereosinophilic syndromes has a prevalence of 0.3 to 6 cases per 100,000 people. Once peripheral eosinophilia is established, end organ damage is evaluated with serum chemistries, LFTs, echocardiography, pulmonary function testing, and computed tomography. Our case is unique in that symptoms were limited to moderate persistent asthma, however pulmonary fibrosis, emboli, lymphadenopathy, pleural effusions, and heart failure may occur. CT displays nonspecific scattered ground glass opacities and consolidations, with or without nodular opacities. Infectious etiologies should be investigated based on relevant exposure. For our gentleman from West Kansas without significant travel exposure this was limited to Strongyloides IgG. Serum tryptase, vitamin B12, and IgE are frequently elevated and guide specialized diagnostic testing. The myeloproliferative form is due to translocation or fusion of certain genes. Our patient was fortunate to carry the classic platelet-derived growth factor receptor alpha fusion (FIP1L1-PDGFRA fusion) as treatment with the tyrosine kinase inhibitor imatinib has an excellent prognosis. Nearly all patients achieve complete remission and remain on a decreased lifelong maintenance therapy.
Author Block: J. Johnstone1, L. Thomas2; 1Department of Internal Medicine, The University of Kansas Medical Center, Kansas City, KS, United States, 2The University of Kansas Medical Center, Kansas City, KS, United States.
IntroductionChronic eosinophilic leukemia is a rare myeloproliferative disorder producing pronounced peripheral eosinophilia and end organ damage. Multisystem involvement is common and may involve the skin, gastrointestinal tract, lungs, heart, and central nervous system. We present a unique case of chronic eosinophilic leukemia manifesting as adult-onset moderate persistent asthma. Case Description: A 72-year-old man with a past medical history of hypertension presented with a 2-year history of progressive shortness of breath, cough, and paroxysmal wheezing. He is a farmer, never-smoker, and has no prior history of lung disease, including no childhood asthma. He was prescribed albuterol and fluticasone several months prior with moderate relief. Due to significant peripheral eosinophilia (70% of WBC 22.1), he received a hematologic workup and a diagnosis of FIP1L1-PDGFRA positive chronic hypereosinophilic leukemia. Prior to treatment with imatinib, spirometry displayed an obstructive pattern (FEV1/FVC 61%, FEV1 2.77L (86% predicted), FVC 4.55L (105% predicted)). CT imaging without consolidating infiltrates, fibrosis or bronchiectasis. He was prescribed inhaled fluticasone, montelukast, and albuterol. He was also initiated imatinib 400mg daily by hematology and at two months he had markedly decreased eosinophilia (6.5% of WBC of 7.4) with improved respiratory symptoms. Repeat spirometry demonstrated resolution of obstructive airway disease (FEV1 3.22L, 110% predicted). His wheezing and cough have nearly resolved with imatinib, inhaled corticosteroid and montelukast. DiscussionChronic eosinophilic leukemia (CEL) is a rare myeloproliferative disorder with pulmonary manifestations in 25-67% of patients. The broad family of hypereosinophilic syndromes has a prevalence of 0.3 to 6 cases per 100,000 people. Once peripheral eosinophilia is established, end organ damage is evaluated with serum chemistries, LFTs, echocardiography, pulmonary function testing, and computed tomography. Our case is unique in that symptoms were limited to moderate persistent asthma, however pulmonary fibrosis, emboli, lymphadenopathy, pleural effusions, and heart failure may occur. CT displays nonspecific scattered ground glass opacities and consolidations, with or without nodular opacities. Infectious etiologies should be investigated based on relevant exposure. For our gentleman from West Kansas without significant travel exposure this was limited to Strongyloides IgG. Serum tryptase, vitamin B12, and IgE are frequently elevated and guide specialized diagnostic testing. The myeloproliferative form is due to translocation or fusion of certain genes. Our patient was fortunate to carry the classic platelet-derived growth factor receptor alpha fusion (FIP1L1-PDGFRA fusion) as treatment with the tyrosine kinase inhibitor imatinib has an excellent prognosis. Nearly all patients achieve complete remission and remain on a decreased lifelong maintenance therapy.
