Leriche’s syndrome and Löffler endocarditis in a 30-year-old patient presenting with hypereosinophilic syndrome

Dear Editor, Here, we report a severe complication of hypereosinophilic syndrome (HES), the onset of Leriche’s syndrome, in a 30year-old patient. A 30-year-old man was referred to our institution due to acute onset of severe pain and symmetric sensory neuropathy of both legs. Contrast enhanced computed tomography showed occlusion of aortic bifurcation (Leriche’s syndrome) (Fig. 1a). Immediate thrombectomy secured reperfusion of the lower limbs. Histopathologic workup confirmed the presence of thrombotic material. Three weeks prior to admission, the patient had been diagnosed with a hypereosinophilic syndrome and Loffler’s endocarditis. At that time, he had presented with nonspecific abdominal pain accompanied by diarrhea and progressive general weakness that he had observed for more than 2 months. Diagnostic workup showed an abnormal eosinophil count 7.5 (10/l) with a corresponding myeloproliferative bone marrow. Fluorescence in situ hybridization (FISH) analysis for rearrangements of the platelet-derived growth factor receptors alpha and beta (PDGFRα/β) were evaluated as negative (LSI 4q12 Tri-Color Rearrangement probe set; Poseidon Repeat Free, 5q33, Break probe). Echocardiography indicated the presence of Loffler endocarditis. Intracardial thrombotic formations had been detectable. Oral anticoagulation with phenprocoumone had been started, and an INR between 2 and 3 was targeted. When Leriche’s syndrome was diagnosed, the INR was 2.0. The patient had been treated with prednisolone (1 g) for 2 days with a temporary drop of eosinophilis to 1.8 (10/l). At admission, hematologic workup confirmed massive increase of eosinophils 3.6 (10/l). Screening for causes of reactive eosinophilia was negative with regard to an allergic reaction and autoimmune, parasitic, or helminthic diseases. Echocardiography confirmed Loffler’s endocarditis (Fig. 1b). Examination of the bone marrow showed a hypercellular marrow with marked increase of eosinophilic granulocytes and their progenitors (Fig. 1c). Standard cytogenetic characterization showed a normal karyotype. Using the FIP1L1Chic2-PDGFRα dual-color probe which is optimized to detect the Chic2 deletion at 4q12 FISH analysis found a deletion in chromosome 4, band q12 [del(4)(q12)] (Fig. 1d). Thomas Klag and Thomas Schnetzke have contributed equally to this work.