Hypereosinophilic syndrome (HES) is a very rare disease (0.5% of autopsy series) affecting the cardiovascular system leading to myocardial damage, heart failure, thrombosis and death. At present, there are only 30 published cases internationally. Given its rarity, it is often discovered on postmortem and the diagnosis is delayed. There is paucity of published data regarding this uncommon entity hence we aim to report 4 cases of Filipino patients with HES focusing on their cardiovascular presentation, work up, management and clinical outcomes.Design and Method:
This is a decriptive study reporting 4 cases of HES admitted in 10 years at 2 tertiary institutions in the Philippines.Sampling Method:
Convenience Sampling Design.Statistical Analysis:
While HES is an extremely rare myelopoetic disorder, 4 Filipino cases were documented in the Philippines. The cardiovascular system can be involved in various ways. Eosinophilic myocarditis (EM) from eosinophilic infiltration is the more common clinical presentation (75%). EM symptomatology, electrocardiographic, echocardiographic findings and increased cardiac enzymes may mimic acute coronary syndrome, making its diagnosis elusive. There is a robust role of cardiac imaging modalities in the diagnosis as typical imaging findings consistent with myocarditis can be detected. Exhaustive search for other causes of myocarditis should be employed. HES can promote a hypercoagulable state through invasive tissue damage and tissue factor exposure leading to a very rare presentation of acute upper limb ischemia. After exhaustive search for other causes of hypercoagulable state and documentation of persistent eosinophilia, there was dramatic improvement in upper extremity blood flow and resolution of peripheral eosinophilia with corticosteroids. Timely initiation of high-dose corticosteroid and antihistamine therapy improves clinical outcomes without waiting for the final histologic diagnosis.Conclusions:
To our knowledge, this is the first Filipino case series, the largest of all international case series, of documented HES and its varied cardiovascular manifestations.