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Hypereosinophilic Syndrome (HES) Roadmap

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Hypereosinophilic Syndrome (HES) Roadmap Hypereosinophilic Syndrome A Guided Workow for Improved National Jewish (HES) Roadmap: Health Diagnosis and Treatment in HES START HERE 1, 2, 3 DIAGNOSIS AND CLASSIFICATION Absolute eosinophil count ≥ 1500/µl A 1 AND/OR Tissue eosinophilia? Consider other causes Table 1 no 1, 4 Monitor for progression Table 1 Secondary causes of eosinophilia and hypereosinophilia Category with examples (not inclusive) yes Allergic disorders Immunologic disorders •Asthma, atopic dermatitis, allergic rhinitis •Immunodeciency: hyper-IgE syndrome, Drug hypersensitivity Omenn’s syndrome, DOCK8 deciency •Autoimmune diseases: sarcoidosis, Infection inammatory bowel disease, IgG4 disease, Initial workup •Helminthic/Ectoparasite other connective tissue disorders 2 •Protozoan •Fungal Miscellaneous •Bacterial •Radiation exposure •Viral, including HIV infection •Cholesterol emboli •Hypoadrenalism Neoplasms •IL-2 therapy •Leukemia History Physical exam Lab Tests Studies •Lymphoma • Onset, magnitude, temporal • Sinus disease • CBC with di • ECG •Adenocarcinoma associations of eosinophilia • Mucosal ulcers • Peripheral smear • Echocardiogram • Comorbid conditions • Adventitious lung sounds • Serum tryptase • PFTs • Exposures, travel • Heart murmurs, rubs • Serum B12 • Pulse oximetry • Medications • Lymphadenopathy • Stool ova & parasites • CXR • Infection • Liver/spleen enlargement • Liver/kidney tests • Family history of eosinophilia • Abdominal tenderness • Amylase/lipase • Symptoms: Organ specic, • Joint/muscle abnormality • Troponin Additional workup to evaluate for evidence of end-organ fever, constitutional 3 • Skin rash, edema • ANA damage or dysfunction • Neuropathy, weakness • ANCA • HIV serology • Serum immunoglobulins Cardiac GI Vascular • Cardiac MRI • Abdomen & pelvis CT • Angiography • Strongyloides IgG • ESR/CRP • Endomyocardial biopsy • Endoscopy with biopsy • LDH Pulmonary Dermatologic Neurologic • Urinalysis • Chest CT (high resolution) • Skin biopsy • Brain MRI or CT with contrast • Bronchoalveolar lavage • EEG • Lung biopsy Renal • Nerve conduction studies/EMG • 24 hour urine test • Nerve biopsy Evidence of end-organ Hematologic • Kidney biopsy 4 damage or dysfunction? • Bone marrow biopsy with cytogenetics • Peripheral FISH/RT-PCR for FIP1L1-PDGFRA • Lymphocyte phenotyping (ow cytometry) • T- and B-cell rearrangement studies • PET scan Secondary causes of yes no • Lymph node biopsy hypereosinophilia excluded? 5 no Hypereosinophilia yes Consider other causes Table 1 • Consider HEUS 2 • Consider subspecialty referral HYPEREOSINOPHILIA: • Monitor for progression C INITIAL TREATMENT APPROACH B Absolute eosinophil DETERMINE HES CLINICAL VARIANT 1 count ≥ 1500/µl 1, 2, 4, 5 Table 2 Symptoms? 2 HES CLINICAL VARIANT ETIOLOGIES/CHARACTERISTICS Clinically-dened MHES, FIP1L1/PDGFRA positive, or chronic Myeloproliferative variant Clinical manifestation of eosinophilic leukemia no myeloid neoplasm? no HEUS Lymphocyte population with aberrant phenotype by ow cytometry OR clonal T cell population by PCR analysis; yes Consider close monitoring Lymphocytic variant o therapy; obtain family history lymphocytes secrete IL-5 or other cytokines that drive eosinophilia Proceed as if symptomatic Family history of documented persistent eosinophilia. yes Familial eosinophilia Autosomal dominant. Mutations associated with IL-5 overexpression Incomplete criteria OR apparent restriction to specic Overlap tissues/organs (eg, eosinophil-associated GI disorders (EGID), chronic eosinophilic pneumonia, eosinophilic fasciitis); EGPA Life- no Benign (asymptomatic with no evidence of organ involve- 3 yes Idiopathic ment), complex (symptomatic with organ involvement but threatening? idiopathic), or episodic (cyclic angioedema and eosinophilia) Comprehensive evaluation: see algorithm for HES Peripheral eosinophilia ≥ 1500/µl associated with a dened Diagnosis and Classication diagnosis (eg, inammatory bowel disease, sarcoidosis, Associated autoimmune lymphoproliferative syndrome, other Initiate therapy secondary causes (Table 1)) based on clinical variant Table 3 1, 2, 4, 5 HES CLINICAL VARIANT TREATMENT Begin diagnostic workup Imatinib, a tyrosine kinase inhibitor, is rst line for known or but do not delay treatment; suspected PDGFR-associated myeloid neoplasms prioritize tests that are aected Myeloproliferative variant Add glucocorticoid if cardiac involvement or by glucocorticoid therapy insucient control with imatinib Short-term glucocorticoid is rst line 4 Hydroxyurea is preferred second line Lymphocytic variant Imatinib, interferon-α, pegylated interferon, methotrexate, Suspected or proven cyclosporine are other second line PDGFR-associated neoplasm? Monitor clinically if no symptoms or tissue organ damage Glucocorticoid if symptomatic and/or evidence of organ involvement Familial eosinophilia Immunomodulators such as hydroxyurea, interferon-α, or anti-IL5 therapy yes no Glucocorticoid and/or immunomodulators if symptomatic and/or evidence of tissue damage Swallowed glucocorticoid and/or diet modication for EGID Overlap Short-term glucocorticoid is rst line for EGPA Mepolizumab, cyclophosphamide, methotrexate are second line for EGPA Features of EGPA? Monitor clinically if no symptoms or tissue organ damage no yes Idiopathic Glucocorticoid if symptomatic and/or evidence of tissue damage Immunomodulators such as hydroxyurea, interferon-α, or anti-IL5 therapy Associated Identify and treat underlying cause of hypereosinophilia HES HES Imatinib +/- glucocorticoid High-dose High-dose glucocorticoid* (high-dose* if cardiac involvement) glucocorticoid* + cyclophosphamide Abbreviations EGPA, eosinophilic granulomatosis with polyangiitis HEUS, hypereosinophilia of unknown signicance *If potential exposure to Strongyloides at any time, treat empirically with concomitant ivermectin for 2 days. (formerly Churg-Strauss syndrome) MHES, myeloid hypereosinophilic syndrome HES, hypereosinophilic syndrome PDGFR, platelet-derived growth factor receptor gene References 1) Khoury P, Bochner BS. Consultation for elevated blood eosinophils: clinical presentations, high value diagnostic tests, and treatment options. J Allergy Clin Immunol Pract. 2018;6(5):1446-1453. | 2) Klion A. Hypereosinophilic syndrome: approach to treatment in the era of precision medicine. Hematology Am Soc Hematol Educ Program. 2018;2018(1):326-331. | 3) Schuster B, Zink A, Eyerich K. Medical algorithm: diagnosis and treatment of hypereosinophilic syndrome. Allergy. 2020;00:1-4. | 4) Klion AD. Eosinophilia: a pragmatic approach to diagnosis and treatment. Hematology Am Soc Hematol Educ Program. 2015;2015:92-97. | 5) Klion AD, Ackerman SJ, Bochner BS. Contributions of eosinophils to human health and disease. Annu Rev Pathol. 2020;15:179-209. This reference aid was developed as part of an educational activity supported by an educational grant from GlaxoSmithKline. © 2021 National Jewish Health.
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