Giant hepatic hemangioma in a patient with cirrhosis: challenging to manage

Giant hepatic hemangiomas are occasional in patients with cirrhosis. It remains a challenge to decide on the need for treatment and choose the most appropriate intervention. A 62-year-old woman was recently diagnosed with cirrhosis and complained of upper abdominal fullness, reduction in oral food intake, and weight loss of 6 kg over the last three years. Upper digestive endoscopy evidenced thin-caliber esophageal varices and significant extrinsic compression of the lesser gastric curvature. Abdominal computed tomography revealed an exophytic tumor in the left hepatic lobe, measuring 11.5 cm, which had progressive centripetal contrast enhancement from the arterial phase, compatible with hepatic hemangioma. Serum tumor markers were negative, and her liver function was unimpaired. The patient underwent surgical resection (non-anatomical hepatectomy of segments II and III) which had no immediate complications, and the histopathological evaluation confirmed cavernous hepatic hemangioma. Two weeks later, she was admitted to the emergency room with jaundice, signs of hepatic encephalopathy, and moderate ascites, and was further diagnosed with secondary bacterial peritonitis. As no perforations, abscesses, or fistulas were observed on subsequent imaging tests, clinical management was successfully carried out. This case highlights that giant hepatic hemangiomas may be symptomatic and warrant treatment. In the setting of cirrhosis and portal hypertension, physicians should be aware of the risk of hepatic decompensation following surgical resection, even in patients with Child-Pugh class A

ENDOCARDITE INFECCIOSA SECUNDÁRIA À DOENÇA DE WHIPPLE: UMA ENTIDADE RARA

Doença de Whipple é uma entidade infecciosa rara, causada pela bactéria Tropheryma whipplei, um bacilo gram positivo. A infecção pode ser assintomática ou cursar com quadro clínico multissistêmico, incluindo febre, poliartralgia, diarreia, emagrecimento e, em raros casos, endocardite ou alterações neurológicas. JP, masculino, 61 anos, com antecedente de artrite psoriática em uso de glicocorticoide e secuquinumabe. Procura atendimento devido perda ponderal não intencional, inapetência, disfagia, febre intermitente e monilíase esofágica com sorologias negativas. A admissão encontrava-se com caquexia, hipoalbuminemia, anemia, leucocitose e linfopenia. Não apresentava sintomas neurológicos ou sintomas articulares. Em investigação, realizada endoscopia digestiva alta que evidenciou duodenite crônica e linfangiectasia, sendo aventada hipótese de Doença de Whipple, confirmada posteriormente com coloração de PAS fortemente positiva. Realizada ainda tomografia de abdome, evidenciando hipodensidade sugestiva de infarto esplênico. Solicitado então ecocardiograma que demonstrou valva aórtica com imagem ecogênica aderida ao folheto coronariano direito medindo 7 × 4 mm, sugestiva de vegetação. Hemoculturas resultaram negativas. Instituído tratamento com Ceftriaxona, entretanto paciente veio a óbito por complicações associadas à internação prolongada, possivelmente associadas à síndrome de reconstituição imune. A endocardite associada à Tropheryma whipplei tem raros relatos na literatura, com provável subdiagnóstico em casos de endocardite de culturas negativas. A evolução com síndrome de reconstituição imune é mais prevalente nos pacientes que receberam tratamento para doença reumática presumida previamente ao diagnóstico de Doença de Whipple, cuja terapia foi descontinuada durante o tratamento, como no caso descrito. Por se tratar de uma doença rara de apresentação clínica variada, a baixa suspeição e o diagnóstico tardio podem contribuir para desfechos negativos

Autoimmune hepatitis and primary biliary cholangitis overlap syndrome after COVID-19

COVID-19 is commonly associated with high serum levels of pro-inflammatory cytokines, and the post-infection status can disturb self-tolerance and trigger autoimmune responses. We are reporting a 45-year-old male who was admitted with fatigue, jaundice, elevated liver enzymes (with cholestatic pattern), and acute kidney injury two weeks after recovering from a mild SARS-CoV-2 infection. Serologies for viral hepatitis and anti-mitochondrial antibody were negative, while anti-nuclear and anti-smooth muscle antibodies were positive. There were no signs of chronic liver disease, and a magnetic resonance cholangiography showed no dilatation of biliary ducts. Histologic evaluation of the liver evidenced numerous foci of lobular necrosis without ductopenia or portal biliary reaction. Considering the autoantibody profile and histologic changes, the medical team started oral prednisone, but there was a suboptimal biochemical response in the outpatient follow-up. Two months later, a second liver biopsy was performed and revealed non-suppurative destructive chronic cholangitis, extensive areas of confluent necrosis with hepatocytes regenerating into pseudorosettes, and numerous plasma cells. According to the Paris Criteria, the patient was then diagnosed with an autoimmune hepatitis-primary biliary cholangitis overlap syndrome (AIH-PBC-OS). After adding azathioprine and ursodeoxycholic acid to the treatment, there was a satisfactory response. This is the second worldwide report of an AIH-PBC-OS triggered by COVID-19, but the first case with a negative anti-mitochondrial antibody. In this setting, histologic evaluation of the liver by an experienced pathologist is a hallmark of achieving the diagnosis and correctly treat the patient