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SCVP Unknown Case 17

  A 35-year-old woman presents to the emergency department with rapid onset of dyspnea, palpitations, chest pain, fatigue and peripheral edema.  She underwent endomyocardial biopsy and was transferred to ECMO shortly thereafter.

 

 

 

 

 

 

Giant cell myocarditis 


  • Although eosinophils are a dominant cell type in this image, the presence of the clear giant cell in the top left quadrant determines the diagnosis in this case of giant cell myocarditis.  
  • Giant cell myocarditis favors younger-to-middle aged adults and has loose associations with autoimmune disorders and thymoma/lymphoma (among others). Affected patients generally become symptomatically ill quickly. When this occurs, endomyocardial biopsy can be a helpful diagnostic tool to guide the clinical team (roughly half are diagnosed this way…with therapeutic implications). Histologically, one sees myocyte necrosis, giant cells, lymphocytes, and eosinophils,  without truly well-formed granulomas. Interestingly, it may well be the eosinophils that cause the majority of the damage in this disease, not the giant cell for which it is named.  
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  • In contrast, eosinophilic endomyocardial disease is uncommon and generally occurs in the setting of peripheral eosinophilia. As the eosinophils marginate in the ventricular chamber, they degranulate and cause localized (endocardial/subendocardial) damage to the myocardium.  This results in relative blood stasis leading to thrombus formation and eventually endocardial fibrosis (generally with restrictive physiology). Patients tend to (but not always) present in the thrombosis stage, wherein they may show an intracardiac mass-like clinical presentation, or a thromboembolic one. On histology, thrombosis, endocardial fibrosis and eosinophilic myocarditis are present, but it is usually devoid of giant cells.
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  • More on myocarditis here.

     

     

    - Melanie Bois

     

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