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Clinical Case Presentation: A Case of Eosinophilia

Objective. Review presentation and differential diagnosis of eosinophilia. Case Patient History. 65 yo white female presents to ED with intermittent upper abd/epigastric pain over previous 2 weeks that has not responded to PPI/H2 blocker, bland diet4-8/10 pain, mild nausea and anorexia, yellowish green stool, no blood in stool, mildly constipated, gassiness present, no diarrhea, 3 pound wt loss in one weekUS done earlier in the day was normal.

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Clinical Case Presentation: A Case of Eosinophilia

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    1. Clinical Case Presentation: A Case of Eosinophilia Kristin Steffen, MD GIM Conference 02/13/08

    2. Objective Review presentation and differential diagnosis of eosinophilia

    3. Case Patient History 65 yo white female presents to ED with intermittent upper abd/epigastric pain over previous 2 weeks that has not responded to PPI/H2 blocker, bland diet 4-8/10 pain, mild nausea and anorexia, yellowish green stool, no blood in stool, mildly constipated, gassiness present, no diarrhea, 3 pound wt loss in one week US done earlier in the day was normal

    4. Case Patient Past Medical History Asthma for 25 years HTN MHA Pericarditis 3/07 Pulmonary nodules found on CT 3/07, unchanged but new nodules per improved CT technique 6/07, new subpleural nodule, increased soft tissue at pericardial margin, ? Inflammatory 9/07 Cataract surgery:OS 9/17, OD 10/1/07

    5. Case Patient Medications Advair Albuterol Singulair Astelin nasal Rhinocort Guaifenesin LA Premarin Vag cream Lisinopril Fish oil Simvastatin Centrum mvit Imitrex

    6. Case Patient Social and Family History Retired med tech/ administrator for State Lab of Hygiene Lives with husband rural SW Wisconsin Camped in Canada near Lake Superior 9/07, drank well water EtOH: 1 wine/d, nonsmoker, no illicit drugs

    7. Case Patient Admit Exam T 37, P78, R 12, 95% RA BP 138/82 Gen: NAD HEENT: post-op cataract surgery changes Abdomen: unremarkable at time of exam Lungs, Heart, Neuro, Skin, Extremities: WNL

    8. Case Patient Admit Labs WBC 23.4: 13,080 E, 7,740 N, 1470 L, 1060 M, 50 B Ca 9.8, t prot 7.9, alb 4.0 Troponin 0 Lipase 193 ESR 36 T bili 0.5 Alk phos 218 (35-130) AST 27, ALT 49 GGT 117 CT Abd: no new findings (unchanged pulm nodules, adrenal adenoma)

    9. DDx Eosinophilia Infectious Disease Helminthic parasitic Fungal (ABPA, Cocci) Scabies, myiasis Allergic Drug hypersensitivity Atopic diseases Immunologic Primary immune defic (Hyper IgE, Omenn syndrome) Graft vs host disease Malignancy Leukemia, lymphoma AdenoCA, SCCA, Large cell lung, transitional cell bladder Systemic mastocytosis Other Hypoadrenalism Irradiation Atheroembolsim Sarcoidosis

    10. DDX Eosinophilia, cont. Hypereosinophilia syndrome (HES) Myeloproliferative variant (fusion protein) Lymphocytic variant (clonal lymphocytes) Familial Undefined (Benign, Complex, Episodic) Overlap (ie eos gi diseases, eos pneumonia, eos myalgia) HES Associated Disorders (see next slide)

    11. DDX Eosinophilia, cont. HES-Associated Disorders Churg-Strauss syndrome Systemic mastocytosis Sarcoidosis HIV Inflammatory bowel disease

    12. Definition of HES Periph blood eosinophilia with abs eos>1500 for more than 6 months No other evident causes for eosinophilia, including allergic disease and parasitic infection Signs or symptoms of organ involvement by eosinophilic infiltration

    13. HES Organ Involvement Cardiovascular (58%) Cutaneous (54%) Neurologic (54%): Encephalopathy/UMN signs, polyneuropathy, mononeuritis multiplex, radiculopathies and related muscle atrophy Splenic (53%) Pulmonary (49%): chronic non-productive cough, NOT wheezing, diffuse and focal infiltrates, pleural effusion

    14. HES Organ Involvement, cont. GI (23%): esophagitis, gastroenteritis, colitis Ocular (23%)

    15. More on Churg-Strauss Syndrome Small-vessel necrotizing vasculitis with asthma (100%), lung infiltrates (38-77%), extravascular necrotizing granulomas and hypereosinophilia Allergic rhinitis/sinus polyps (70%) Peripheral neuropathy (50-78%) Cardiac (60%) GI (37-62%) Kidney (16-49%) Skin (40-75%)

    16. Treatment HES Prednisone Interferon alpha, hydroxycarbamide Cytotoxic agents Cyclosporine Imatinib (1/3 of idiopathic/nonmyeloproliferative cases respond Monoclonal antibody therapy Myeloablative and nonmyeloablative allograft under study

    17. Treatment Churg-Strauss Prednisone +/- preceding methylpred pulse dose Cyclophosphamide /azathioprine Monoclonal Ab (several types) Interferon alpha

    18. Case Patient: Further Diagnostics Flow cytometry normal Bone marrow biopsy normal BM AFB negative IgE 78 (0-180) C-ANCA, P-ANCA negative Histo, Blasto, AB negative Giardia Ag negative Galactomannan normal Tryptase normal HIV negative

    19. Case Patient: Clinical Course Toe paresthesia 3 days later-EMG neg Prednisone 60 mg daily, eos count nl within 10 days Zoster in trigeminal distribution with eye involved Transient global amnesia 3 weeks later-viral encephalitis ruled out by neuro work up Cardiac Echo normal Sural nerve biopsy pending (“highly informative” in Churg-Strauss)

    20. Case Patient Diagnosis Hypereosinophilia syndrome versus Churg-Strauss syndrome, favor Churg-Strauss

    21. References Sheikh J, Weller PF. Clinical overview of hypereosinophilic syndromes. Immunol Allergy Clin N Am 2007;27: 333-355. Pagnoux C, Guilpain P, Guillevin L. Churg-Strauss syndrome. Curr Opin Rheumatol 2007;19(1):25-32. Fletcher S, Bain B. Diagnosis and treatment of hypereosinophilic syndromes. Curr Opin Hematol 2007;14:37-42. Nutman TB. Evaluation and differential diagnosis of marked, persistent eosinophilia. Immunol Allergy Clin N Am 2007;27:529-549.

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