Thrombosis in the portal venous system caused by hypereosinophilic syndrome: A case report

Abstract

Rationale: Extensive thrombosis in the portal venous system caused by hypereosinophilic syndrome (HES) is rare, and there is no consensus on anticoagulant and thrombolytic treatments for arteriovenous thrombosis caused by HES.

Patient concerns: The clinical data of a patient with extensive thrombosis in his portal venous system (superior mesenteric, splenic, hepatic, and portal veins), renal artery thrombosis, and mesenteric thrombosis caused by HES with secondary gastrointestinal bleeding and intestinal necrosis were retrospectively analyzed. Before admission, his eosinophil count increased to 7.47 × 10/L, and HES had been confirmed via bone marrow cytology. The patient experienced fever, cough, abdominal pain, massive hematemesis, and hematochezia that developed in succession. Abdominal computed tomography showed portal vein and superior mesenteric vein thromboses.

Diagnosis: Hypereosinophilic syndrome; extensive thrombosis in the portal venous system; acute eosinophil-associated pneumonia; gastrointestinal bleeding; intestinal necrosis.

Interventions: The patient was first treated with methylprednisolone, plasma exchange/hemofiltration, and single or combined use of unfractionated heparin and argatroban for anticoagulation. He was also administered alteplase and urokinase, successively, for thrombolytic treatment. Once the thromboses finally disappeared, the patient underwent surgery to excise a necrotic intestinal canal.

Outcomes: The thromboses disappeared with these treatments, and the patient recovered after the necrotic intestinal canal was excised.

Lessons: The clinical manifestations of HES are complex and varied, and this condition can cause severe and extensive arteriovenous thrombosis. Anticoagulation therapy and thrombolysis are necessary interventions, and appear to be safe and effective.

Figure 1

Bone marrow cytological examination. Granulocyte proliferation was markedly high, and the proportion of eosinophils was significantly increased. The morphologies of the remaining granulocytes at various stages were normal, with erythroid hyperplasia inhibited slightly.

Figure 2

Whole abdominal enhanced computed tomography scan on July 21, 2017. The arrow in the figure refers to veins with extensive venous filling defects in the contrast-enhanced scan during the venous phase, suggesting venous thrombosis.

Figure 3

Whole abdominal enhanced computed tomography scan on July 25, 2017. Both axial and reconstructed images showed extensive venous filling defects, suggesting extensive venous thrombosis that was not significantly improved since July 21. The thrombosis was accompanied by partial small intestine expansion and intestinal wall edema.

Figure 4

The variation trend of fibrinogen degradation products and D-dimer in our patient's plasma after anticoagulant and thrombolytic treatments. The coagulogram in this patient was monitored since his admission. The abscissa in the figure indicated different time points.

Figure 5

Changes in the volume of ascites of our patient. The abscissa in the figure indicated the daily ascites volumes.

Figure 6

Changes in our patient's hemoglobin levels. The abscissa indicates the date when the hemoglobin level was measured, while the arrows show the amount of red blood cell transfusion on the indicated dates.

Figure 7

Bedside abdominal ultrasonography scan. (A) indicates intestinal cavity expansion and severe intestinal wall edema in this patient on July 21, 2017. (B) and (C) show that the intestinal wall still had significant edema on July 23, but the tension was reduced. (D) shows that the intestinal wall edema and tension were significantly reduced by July 25.

Figure 8

Whole abdomen enhanced computed tomography scan on July 31, 2017. Apart from the lack of significant improvement in the right hepatic vein thrombosis and small emboli in the left branch of the portal vein, the remaining veins were basically unobstructed. However, the small intestine showed expansion and effusion. A part of the small intestine was severely expanded with decreased perfusion, and intestinal necrosis could not be ruled out.