Development of Henoch-Schoenlein purpura in a child with idiopathic hypereosinophilia syndrome with multiple thrombotic onset: A case report

Abstract

BACKGROUND

The incidence of pulmonary embolism (PE) in children is low, but its mortality is high. Hypereosinophilic syndrome (HES) is a group of diseases caused by an abnormal increase in eosinophilic granulocytes resulting in multiple-organ dysfunction. The urgent event of thromboembolism in the pulmonary region provoked by eosinophils in idiopathic HES (IHES) is relatively unusual. This article reports a case of IHES with multiple PEs and left leg venous thrombosis as the first manifestation. One month later, the patient developed Henoch-Schonlein purpura (HSP), which is very rare.

CASE SUMMARY

We report the case of a 12-year-old boy who was admitted to the hospital with dyspnea, left leg pain, and aggravation. He had bilateral PE and left leg venous embolism with mild eosinophilia. Low-molecular-weight heparin and urokinase were given. At the same time, the interventional department was contacted about filter implantation, followed by urokinase thrombolysis. The left leg thrombus was aspirated under ultrasound guidance. He was discharged from the hospital on rivaroxaban. One month later, he developed a rash on both legs and ankle pain consistent with HSP, with severe eosinophilia and motor and sensory disturbances. The patient was diagnosed with IHES with multiple embolisms complicated by HSP after excluding other causes of the eosinophil elevation. After glucocorticoid treatment, the symptoms were relieved, but the patient later developed purpura nephritis.

CONCLUSION

We report a rare and life-threatening case of IHES with multiple embolisms associated with HSP. A mild elevation of eosinophils early in the disease leads to difficulties in diagnosis and delayed treatment.

Keywords: Eosinophil, Hypereosinophilic syndrome, Henoch-Schoenlein purpura, Thrombosis, Case report

Core Tip: Pulmonary embolism (PE) in children usually occurs in the presence of an underlying condition, systemic disease, or other risk factors. Idiopathic PE accounts for less than 4% of these. The child has no risk factors for thrombosis other than obesity at the first hospitalization. A month later, he developed a purpuric rash on both legs, and pain in his ankles consistent with Henoch-Schönlein purpura (HSP) was accompanied by severe eosinophilia and motor and sensory impairments. Persistent eosinophilia in peripheral blood can lead to tissue infiltration and even organ damage. If end-organ damage occurs, hypereosinophilic syndrome (HES) can be diagnosed immediately. According to monist principles, patients are diagnosed with idiopathic HES with multiple embolisms complicated by HSP. After glucocorticoids, eosinophils quickly return to normal, neurological symptoms gradually improve, and the rash disappears. Eosinophils are only mildly elevated in PE, making clinical diagnosis more difficult.