Project Title

Hyper eosinophilia with cardiomyopathy as manifestation of Churg Strauss syndrome

Authors' Affiliations

hakam khazrik ,department of internal medicine , medical oncology ,ETSU, johnson city ,TN

Faculty Sponsor’s Department

Internal Medicine

Additional Sponsors

Koyamangalath Krishnan MD

Type

Oral Non-Competitive

Classification of First Author

Post-doctoral Fellow

Project's Category

Healthcare and Medicine, Medicine

Abstract Text

Hyper eosinophilia with cardiomyopathy as manifestation of Churg Strauss syndrome

Hakam Khazrik MD1, Purva Sharma MD1

Division of Hematology/Oncology, Dept of Internal Medicine, East Tennessee State University.

Eosinophilia (≥500 eosinophils/micro-L) and hyper eosinophilia (≥1500 eosinophils/micro-L) could be caused by many conditions including allergic, infectious, inflammatory and neoplastic disorders. Patient may present with severe organ involvement require hospitalization and urgent intervention.

A 39-year-old female with history of asthma, nasal polyps presented with worsening dyspnea, lower extremity edema, acute left heart failure and non-ST elevation myocardial infarction with significant troponin elevation (31 ng/ml). Cardiac catheterization revealed no stenosis, echocardiogram showed severely reduced ejection fraction 20%. She was managed medically with presumptive diagnosis of viral myocarditis. One month later she represented with similar symptoms associated with mild facial malar rash and worsening peripheral eosinophilia (absolute count 6400cells/micro-L), leukocytosis (WBC 11k/ul norma differential except eosinophilia mild anemia Hg: 10g/dl, normal platelet(304k/ul), normal renal and liver functions, mild elevated tryptase 16. CT chest revealed bilateral hilar lymphadenopathy with no pulmonary infiltrate. Parasite, fungal,, tuberculosis, HIV, hepatitis infections were ruled out. Bone marrow biopsy revealed hypercellular marrow for age, mild increased marrow eosinophilic precursor, normal cytogenetics, FISH studies for MDS, eosinophilia, and BCR-ABL as well as molecular MPN panel were unremarkable. No lymphoproliferative disorder or increased blasts noted. Subcarinal lymph node biopsy with normal lymph node tissue. Extensive rheumatological workup was unremarkable except for elevated rheumatoid factor, ESR, CRP, anti-myeloperoxidase and anti-protease- 3. Eosinophilic granulomatosis with polyangiitis, (EGPA)was diagnosed. Patient started on long taper prednisone with improvement in her symptoms and her eosinophilia. Given severe disease with cardiac involvement she was started on cyclophosphamide to improve her prognosis.

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss), is multisystem disorder characterized by chronic rhinosinusitis, asthma and prominent peripheral blood eosinophilia. Asthma is the cardinal feature. Skin and neurological involvement is usually common but cardiac involvement is one of the more serious manifestations of EGPA accounting for approximately one-half of deaths attributable to EGPA. Two sets of diagnostic criteria are commonly used: the American College of Rheumatology (ACR) criteria and the Lanham criteria. Main diseases to consider in the differential diagnosis of EGPA are aspirin-exacerbated respiratory disease, the eosinophilic pneumonias, allergic bronchopulmonary aspergillosis, hyper eosinophilic syndrome, granulomatosis with polyangiitis and microscopic polyangiitis.

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Hyper eosinophilia with cardiomyopathy as manifestation of Churg Strauss syndrome

Hyper eosinophilia with cardiomyopathy as manifestation of Churg Strauss syndrome

Hakam Khazrik MD1, Purva Sharma MD1

Division of Hematology/Oncology, Dept of Internal Medicine, East Tennessee State University.

Eosinophilia (≥500 eosinophils/micro-L) and hyper eosinophilia (≥1500 eosinophils/micro-L) could be caused by many conditions including allergic, infectious, inflammatory and neoplastic disorders. Patient may present with severe organ involvement require hospitalization and urgent intervention.

A 39-year-old female with history of asthma, nasal polyps presented with worsening dyspnea, lower extremity edema, acute left heart failure and non-ST elevation myocardial infarction with significant troponin elevation (31 ng/ml). Cardiac catheterization revealed no stenosis, echocardiogram showed severely reduced ejection fraction 20%. She was managed medically with presumptive diagnosis of viral myocarditis. One month later she represented with similar symptoms associated with mild facial malar rash and worsening peripheral eosinophilia (absolute count 6400cells/micro-L), leukocytosis (WBC 11k/ul norma differential except eosinophilia mild anemia Hg: 10g/dl, normal platelet(304k/ul), normal renal and liver functions, mild elevated tryptase 16. CT chest revealed bilateral hilar lymphadenopathy with no pulmonary infiltrate. Parasite, fungal,, tuberculosis, HIV, hepatitis infections were ruled out. Bone marrow biopsy revealed hypercellular marrow for age, mild increased marrow eosinophilic precursor, normal cytogenetics, FISH studies for MDS, eosinophilia, and BCR-ABL as well as molecular MPN panel were unremarkable. No lymphoproliferative disorder or increased blasts noted. Subcarinal lymph node biopsy with normal lymph node tissue. Extensive rheumatological workup was unremarkable except for elevated rheumatoid factor, ESR, CRP, anti-myeloperoxidase and anti-protease- 3. Eosinophilic granulomatosis with polyangiitis, (EGPA)was diagnosed. Patient started on long taper prednisone with improvement in her symptoms and her eosinophilia. Given severe disease with cardiac involvement she was started on cyclophosphamide to improve her prognosis.

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss), is multisystem disorder characterized by chronic rhinosinusitis, asthma and prominent peripheral blood eosinophilia. Asthma is the cardinal feature. Skin and neurological involvement is usually common but cardiac involvement is one of the more serious manifestations of EGPA accounting for approximately one-half of deaths attributable to EGPA. Two sets of diagnostic criteria are commonly used: the American College of Rheumatology (ACR) criteria and the Lanham criteria. Main diseases to consider in the differential diagnosis of EGPA are aspirin-exacerbated respiratory disease, the eosinophilic pneumonias, allergic bronchopulmonary aspergillosis, hyper eosinophilic syndrome, granulomatosis with polyangiitis and microscopic polyangiitis.

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