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Washington University Experience | VASCULAR | Infarct - Embolic | 9A0 Case 9 History
Case 9 History ---- The patient was a 42 year old woman with a past medical history of diabetes, hypertension, obesity, megaloblastic anemia, obstructive sleep apnea and weakness. She presented to an OSC on March 5 with left shoulder weakness. Workup showed no abnormality on head CT. WBC count was 50,000 with 48% eosinophils (24,000/m3). Weakness progressed to left hemiparesis and then to quadriparesis with mental status changes. Bone marrow biopsy demonstrated trilineage hematopoiesis with no cause for the patient's eosinophilia. By March 20 she had progressive shortness of breath requiring intubation and transfer to BJC BMT service. There she was treated with high dose IV steroids. The neuromuscular service was consulted and EMG/NCS demonstrated reduced and absent CMAPS as well as milder sensory involvement. Their differential diagnosis included myositis, eosinophilia related myalgia, neuromuscular junction disease and myosin loss myopathy. However, they note that these conditions would not explain the mental status changes. The patient had a declining course with persistent fever and yeast UTI. Extensive laboratory testing was unrevealing. FISH examination for CHIC2 deletion, a surrogate marker for FIP1L1-PDGFRA fusion, which occurs in some eosinophilia syndromes and predicts response to imatinib mesylate therapy was within normal limits. She developed thrombocytopenia and HES was entertained as a diagnosis. A followup head CT revealed indistinct hypodensities in the centrum semiovale bilaterally, pons, and right thalamus. She developed hypokalemic hyperglycemia requiring an insulin drip. Early on 4/17 she had bradycardia followed by asystole. ACLS protocol brought her back to a sinus tachycardia rhythm requiring multiple pressors. She begun on empiric therapy for sepsis but later that day her family withdrew care. ---- Autopsy revealed platelet-fibrin emboli and infarcts involving cerebral hemispheres, deep gray, brainstem, cerebellum and spinal cord. Microvascular engorgement with eosinophils in the CNS likely reflects an idiopathic hypereosinophilic syndrome (IHS) with no evidence of an allergic, neoplastic or parasitic etiology. Some patients with IHS develop Loeffler’s endocarditis which includes patchy endocardial fibrosis. Imatinib mesylate has proven effective in the treatment of hypereosinophilic syndrome (HES) and chronic myeloproliferative disorders that are associated with rearrangement of the PDGFRbeta gene, which this patient did not have. Although she had areas of frank myocardial infarction, numerous endocardial fibrin deposits overlay intact, non-infarcted myocardium. The extent of platelet fibrin microemboli in various non-CNS organs suggests an embolic source for the infarcts in the brain, both recent and subacute. CNS vasculitis was not identified. The recanalization of rare brain microvasculature in intimate association with subacute infarcts suggests that the process was not periterminal. In this patient’s case, she had large numbers of involved thromboembolized vessels, an underlying microvasculopathy due to diabetes and hypertension, and large numbers of eosinophils with the parenchymal microvasculature, of which the thromboemboli are thought to have been the most substantial process producing numerous, often microscopic, areas of brain infarction.
