Control of hypereosinophilic syndrome-associated recalcitrant coronary artery spasm by combined treatment with prednisone, imatinib mesylate and hydroxyurea

Abstract

Uncontrolled hypereosinophilic syndrome is frequently associated with cardiovascular consequences that cause significant morbidity and mortality. The present article reports on a patient with hypereosinophilic syndrome in whom recurrent, recalcitrant coronary artery spasm and associated cardiac arrest were the predominant cardiac manifestations. No valvular abnormalities, evidence of mural thrombi or other cardiac findings commonly associated with hypereosinophilic syndrome were detected, and cardiac function remained normal. The serum tryptase level was normal, cysteine-rich hydrophobic domain 2 (CHIC2) deletion analysis of bone marrow cells was negative and no evidence of mastocytosis or other hematological disorder was found in the bone marrow. To allow for the reduction of prednisone, interferon-alpha-2b was added to the patient's program, but caused aggravation of chest pain and was discontinued. However, a combination of reduced prednisone dosage, imatinib mesylate and hydroxyurea successfully controlled the eosinophilia, and thereafter, episodes of coronary artery spasm did not recur. The clinical features of the present case suggest that, in some patients, hypereosinophilia may manifest as resistant coronary artery spasm and that aggressive control of eosinophilia is necessary.

Keywords: Coronary artery spasm; Hydroxyurea; Idiopathic hypereosinophilic syndrome; Imatinib mesylate.

Figure 1)

The initial 12-lead electrocardiogram showed ST segment elevation in limb leads II, III and aVF and in precordial leads V₄ to V₆, consistent with transmural myocardial ischemia

Figure 2)

The coronary angiogram (top left) showed severe spasm (arrow) of the left anterior descending coronary artery. The left anterior descending coronary artery spasm resolved after administration of nitroglycerin (top right). The coronary angiogram demonstrated severe spasm (arrows) at multiple sites within the circumflex coronary artery (bottom left). The multifocal circumflex coronary artery spasm resolved after administration of nitroglycerin (bottom right)

Figure 3)

Total eosinophil counts, medication usage and clinical highlights. Freq Frequent; G Gleevec (Novartis International AG, Switzerland); IFN-α Interferon-alpha