LAUSR

Dear Editor, Hepatosplenic schistosomiasis (HSS) is an underdiagnosed complication of chronic schistosomal infection which can lead to severe portal hypertensive complications if left untreated. We present a case of thrombocytopenia and splenomegaly secondary to undiagnosed schistosomal infection in an African migrant, illustrating the importance of recognising HSS early in the diagnostic process. A 31-year-old previously well South Sudanese man who migrated to Australia fifteen years prior was referred to a haematology service for an incidental thrombocytopaenia of 71 × 109/L (150–450 × 109/L) with no other blood-based haematological abnormalities including normal eosinophil count. Liver function tests (LFTs) were mildly deranged with a bilirubin of 26 μmol/L (< 20 μmol/L) and gammaglutamyltransferase (GGT) of 91 U/L (< 60 U/L). Biochemical hepatic synthetic function was preserved, but a raised total protein of 84 g/L (60–80 g/L) with increased polyclonal serum IgG of 29.4 g/L (7.5–15 g/L) was noted. Abdominal ultrasound showed moderate splenomegaly (858 mL) with a borderline enlarged portal vein, 12 mm (< 13 mm). Infectious causes of splenomegaly were assessed, and a 1:2560 Schistosoma antibody titre was detected, with S. mansoni isolated from faecal specimens. Two doses of praziquantel, 20 mg/kg, were prescribed, with subsequent negative faecal specimens and a downtrend in serum IgG to 18.8 g/L. Ultrasound 9 months later showed reduction in splenomegaly (528 mL), with a portal vein diameter of 14.3 mm. Magnetic resonance cholangiopancreatography (MRCP) demonstrated extensive periportal (pipestem) fibrosis with a lobulated liver contour. Additionally, widened hepatic fissures, peripheral perihepatic vessels, splenomegaly, splenic siderotic nodules, and portal and splenic vein enlargement were noted (Fig. 1a). Imaging features were pathognomonic of HSS. Retrospective review of a previous abdominal ultrasound showed periportal fibrosis seen as a “bull’s eye” on crosssection, also pathognomonic of HSS, which had initially been missed (Fig. 1b). Schistosomiasis is a trematode infection that affects over 250 million people across Africa, parts of Asia, South America, and the Middles East [1]. HSS manifests in 5–10% of cases, and is characterised by periportal fibrosis with ensuing peripheral portal vein branch occlusion, pre-sinusoidal obstruction, and subsequent portal hypertensive complications including splenomegaly [1]. HSS can often be misdiagnosed particularly in non-endemic countries and can present with thrombocytopenia, splenomegaly, or features suggestive of parenchymal liver disease with associated portal hypertension [2]. Pipestem fibrosis on ultrasound and MRI is highly specific and can differentiate HSS from portal hypertension [2]. The antihelmintic therapy, praziquantel, is highly effective, and serum IgG and ultrasound are useful indicators of treatment response [3]. Time to diagnosis in non-endemic countries remains suboptimal. In a European multicentre cohort study, the median diagnostic delay of schistosomiasis from time of arrival was 31 months in new migrants [4]. Delays in diagnosis are likely due to a lack of awareness of HSS, easily missed clinical and radiological features and presentations which can mimic parenchymal liver disease and haematological malignancy. In our case, the time from initial referral to diagnosis and treatment was 9 months. Given the ease and efficacy of anti-schistosomal therapies, there should be strong impetus to considering of schistosomiasis and other communicable diseases when assessing migrants from highendemicity populations. * Timothy Phan [email protected]