Background:Malignant and benign hepatic diseases may both present with systemic symptoms and necrotic liver cavities. Although rare and reported predominantly in East Asia, pyogenic liver abscess (PLA) can represent the initial manifestation of hepatocellular carcinoma (HCC), as necrotic tumors may mimic infectious abscesses. Case Presentation:A 63-year-old Italian man presented with a 2-week history of fever and right upper quadrant pain. Computed tomography revealed a multiloculated lesion with irregular margins and a solid component in the right hepatic lobe, suggestive of a liver abscess, and empirical tigecycline therapy was initiated. Low-titer Entamoeba spp. IgG antibodies were detected, and percutaneous aspiration yielded material initially mischaracterized as "anchovy paste," raising suspicion for amoebic liver abscess. However, the patient failed to improve despite appropriate antiprotozoal therapy. Subsequent percutaneous drainage led to cavity resolution and symptom relief; all microbiological and parasitological tests were negative, and repeat Entamoeba serology became negative. Concurrent biopsy of the solid component revealed well-differentiated pseudoglandular HCC. Magnetic resonance imaging demonstrated a biliary fistula associated with the neoplastic lesion, supporting a diagnosis of PLA secondary to HCC. The patient underwent right hepatectomy, complicated by intraoperative abscess rupture and postoperative intra-abdominal abscesses caused by vancomycin-resistant Enterococcus faecium. Discussion:PLA presenting as the initial manifestation of HCC is associated with poor prognosis, largely due to diagnostic delays. Limited clinical experience outside high-prevalence regions and nonspecific clinical, laboratory, and imaging findings contribute to underrecognition of malignancy. Positive microbiological or serological results and transient clinical improvement during antimicrobial therapy do not exclude an underlying neoplasm. Liver biopsy and close radiologic follow-up are essential in atypical or treatment-refractory cases. Conclusion:PLA as a presenting feature of HCC is unusual, especially in Europe, yet poses substantial diagnostic and therapeutic challenges. Increased clinical awareness and early histopathological evaluation are crucial to avoid delayed oncologic diagnosis.
Isolated polycystic liver disease (PLD) is rare and may cause debilitating mass-effect symptoms despite relatively preserved hepatic synthetic function. Because the Model for End-Stage Liver Disease (MELD) score does not capture nutritional, functional, and mechanical disease burden, affected patients may have low calculated MELD scores despite advanced symptomatic disease. Updated MELD exception guidance broadened consideration for PLD to include malnutrition and sarcopenia. We describe a patient with isolated PLD who was initially considered too well for transplantation but subsequently developed progressive weight loss, sarcopenia, and functional decline, prompting repeat evaluation, wait-list registration, and MELD exception approval. Several weeks after listing, he sustained traumatic hepatic cyst rupture, resulting in fatal hemoperitoneum and abdominal compartment syndrome. This case highlights the evolving clinical course of symptomatic PLD, the importance of longitudinal reassessment, and the potential severity of rare cyst-related complications.
Background:Herb-induced liver injury (HILI) represents an increasing diagnostic challenge owing to the widespread use of herbal and dietary supplements (HDS), which are frequently consumed without reliable information regarding their composition or safety. Mischaracterized products may obscure causal attributes and mimic primary herbal hepatotoxicity. Methods and Results:We report a case series of five patients with suspected HILI at a tertiary university hospital in Bahia, Brazil. Clinical causality was prospectively assessed using the updated Roussel Uclaf Causality Assessment Method (RUCAM, 2016). Most patients present with hepatocellular injury following exposure to commercially available HDS, frequently in the presence of metabolic comorbidities. Analytical authentication of the consumed products revealed substantial label-composition discordance, including negligible amounts of turmeric-derived constituents in a Curcuma longa formulation and multiple undeclared synthetic drugs in a weight-loss product. The integration of structured clinical assessment with product verification refined causal attribution and, in selected cases, supported the reclassification of suspected herbal hepatotoxicity as probable adulteration-related drug-induced liver injury. Conclusion:Product authentication may substantially improve the diagnostic accuracy of suspected HILI and reduce the etiological uncertainty. Incorporating the verification of consumed supplements into hepatology evaluation may help distinguish true herbal hepatotoxicity from adulteration-related liver injury, thereby improving patient safety and pharmacovigilance.
Background:Drug-induced liver injury (DILI) refers to hepatotoxicity caused by conventional chemical drugs or xenobiotics, whereas herb-induced liver injury (HILI) is attributed to herbal and dietary supplements. Both these conditions pose diagnostic challenges, particularly when concurrent etiologies such as acute viral hepatitis are present. Hurler syndrome (mucopolysaccharidosis Type I) causes hepatocyte and Kupffer cell vacuolization and can predispose to DILI. Early diagnosis is critical given the high fatality rates associated with DILI. Case Report:We present a case of a 28-year-old male with Hurler syndrome who presented with acute onset of nausea, vomiting, and jaundice. Liver function tests (LFTs) revealed markedly elevated liver enzymes. Serological workup identified newly acquired acute hepatitis C virus (HCV) infection. The patient had recent amoxicillin use and was taking hibiscus tea daily. Causality assessment using the updated Roussel Uclaf Causality Assessment Method (RUCAM) of 2016 yielded a score of 6 (probable DILI). Liver biopsy confirmed DILI. The patient showed clinical improvement with N-acetylcysteine and corticosteroids, with progressive normalization of liver enzymes. Conclusions:This case highlights the importance of differentiating DILI from acute viral hepatitis: strong clinical suspicion, temporal relation with offending drug, liver biopsy, and treatment response assessment. Clinicians should have a high index of suspicion for DILI even in the presence of concurrent acute HCV infection, especially in patients with underlying hepatic dysfunction such as Hurler syndrome in our case.
Background:Abiraterone acetate is widely used in metastatic castration-resistant prostate cancer and is generally considered safe. Hepatotoxicity is a known adverse effect, but fulminant liver failure remains a rare and potentially fatal complication. Case presentation:An 80-year-old man with metastatic prostate cancer was admitted with high-grade fever and general deterioration three weeks after initiation of abiraterone in addition to ongoing androgen deprivation therapy. He presented with jaundice, hypotension, and altered mental status. Laboratory investigations revealed severe acute liver injury with markedly elevated transaminases, coagulopathy, hyperbilirubinemia, acute kidney injury, metabolic acidosis, and hypoglycemia. Computed tomography demonstrated marked periportal hepatic edema and mild hilar lymphadenopathy, consistent with severe acute liver injury. No focal hepatic lesions or malignancy were identified. Despite discontinuation of abiraterone and supportive care, there was a progression to multiorgan failure, and the patient died within 48 h of admission. Postmortem investigations excluded acute hepatitis A and cytomegalovirus infection. Epstein-Barr virus serology was consistent with past infection, excluding acute infection. Although histological confirmation was not obtained, the clinical course and temporal association were highly suggestive of drug-induced liver injury. Conclusion:This case highlights a rare but catastrophic adverse reaction to abiraterone. Regular monitoring of liver function and early consideration of hepatotoxicity in patients presenting with systemic symptoms during treatment are essential.
Pruritus is a common, often debilitating symptom of liver disease. While most commonly seen in the setting of cholestasis and biliary obstruction, intrahepatic portosystemic shunts (PSSs) may also present with pruritus. The pathophysiology of intractable pruritus is not well understood and often requires multimodal management. We present a rare case of an 81-year-old woman with severe medically refractory pruritus with elevated serum bile acids in the absence of chronic liver disease. Antipruritics and plasmapheresis produced minimal relief, leading her to contemplate medical assistance in dying (MAID). Imaging during workup revealed two intrahepatic shunts, anomalously connecting the right portal and hepatic veins. Endovascular shunt embolization led to normalization of her serum bile acids and produced remarkable symptomatic relief. This highlights a rare but treatable cause of pruritus in an adult without chronic liver disease or biliary obstruction, while showcasing embolization as a safe and effective treatment strategy with significant improvement of the patient's quality of life.
Hereditary spherocytosis is an inherited red cell membrane disorder resulting in haemolytic anaemia. Recognised clinical manifestations include anaemia, jaundice, splenomegaly and gallstones. Here we describe the case of a 40-year-old male with hereditary spherocytosis presenting with severe hyperbilirubinaemia. Liver biopsy demonstrated features consistent with acute severe cholestasis. Despite extensive investigations for gallstone disease and other causes of liver pathology, no aetiology was identified. There are very few reports in the literature describing cases of profound unexplained jaundice in hereditary spherocytosis. Hereditary spherocytosis may be associated with idiopathic acute cholestasis. We report that the case was managed conservatively and spontaneously resolved.
Vanishing bile duct syndrome (VBDS) is a rare form of liver injury caused by ischemia, drug reactions, autoimmune diseases, infections, or malignancy. VBDS involves the progressive disappearance of intrahepatic bile ducts, causing cholestasis and biliary cirrhosis, with high mortality if untreated. VBDS can also present as a paraneoplastic syndrome in Hodgkin's lymphoma (HL). A 53-year-old female presented with jaundice, pruritus, diarrhea, and elevated liver function tests (LFTs). A liver biopsy demonstrated cholestasis with mild ductopenia, and imaging revealed enlarged para-aortic lymph nodes. A bone marrow biopsy confirmed HL, and chemotherapy normalized symptoms and LFTs.
Fibrolamellar hepatocellular carcinoma (FLHCC) accounts for less than 1% of all primary liver cancers. Due to an absence of known risk factors, most individuals are diagnosed at advanced stages of disease. Subsequently, these tumors carry a 30%-50% mortality rate and a 33%-100% recurrence rate in those treated with curative intent. Here, we describe an incidental discovery of an FLHCC tumor in a young male patient, who following surgical resection has remained recurrence free for over 11 years. This exceptionally long recurrence-free survival highlights the advantage of early-stage detection, the benefit of complete tumor resection, and further underscores the importance of regular postresection cancer surveillance.
Hepatic epithelioid hemangioendothelioma (HEHE) is a rare tumor of vascular origin with an incidence of < 0.1/100,000. The disease is easily misdiagnosed. The aim of this article is to increase public awareness and vigilance of HEHE. We report two cases presenting with fever, abdominal discomfort, abnormal liver function, and jaundice. Computed tomography (CT) and magnetic resonance imaging (MRI) of the abdomen suggested hepatic stasis, venous compression, and ascites, and liver biopsy was initially misdiagnosed as hepatic veno-occlusive disease (VOD), which did not improve with treatment. In the first case, a male patient, after liver transplantation, pathological and immunohistochemical (IHC) analyses revealed hyperplasia of blood vessels in the liver tissue with dilated lumen and heterogeneous cells. Immunohistochemistry was performed and showed CD34 positivity, confirming the diagnosis of HEHE. The second female patient had liver bruising and ascites on imaging, and the first hepatic puncture was reported to be VOD, which did not improve with treatment. Repeat hepatic puncture was performed, and the diagnosis of HEHE was confirmed after a second repathology with additional immunohistochemistry for HEHE. These misdiagnosis cases highlight the challenge of diagnosing HEHE. This is the first report of misdiagnosis of HEHE as VOD. This article analyzes the underlying causes of misdiagnosis of HEHE and emphasizes the causes of imaging misdiagnosis and the importance of repeated hepatic puncture biopsy and immunohistochemistry in the diagnosis of HEHE.
Isolated gallbladder tuberculosis (GBTB) is a rare disease, even in endemic areas, and is often misdiagnosed due to its nonspecific clinical and imaging findings. Histopathological evaluation, demonstrating granulomatous inflammation with caseous necrosis, remains the only definitive method of diagnosis. Thus, maintaining a high index of suspicion and routine histological assessment of resected specimens are vital for its timely management, especially in patients with a history of tuberculosis. We report a similar case in a 50-year-old male patient with imaging suggestive of gallbladder perforation and chronic cholecystitis, which later revealed a cryptic GBTB dug up during histopathological examination.
A 77-year-old female with dementia was transferred from her family clinic to our hospital with a 2-day history of appetite loss and was diagnosed with pneumonia and urinary tract infection upon admission. Laboratory investigation revealed hepatitis C virus antibody positivity and an elevated hepatitis C virus ribonucleic acid level of 3.6 Log IU/mL; therefore, direct-acting antiviral therapy was initiated. Although the patient requested treatment for hepatitis C, managing her medication was difficult because of dementia, as she lived alone and had no family, which required her to take medication under supervision, even on holidays. After discharge, the patient was treated with glecaprevir hydrate and pibrentasvir for 8 weeks by a hepatologist with biweekly visits to monitor adverse events. The hepatitis C virus ribonucleic acid test result was negative after 4 weeks of treatment, and we asked her family physician to confirm a sustained virologic response. Collaboration among multiple specialists, both within and outside the hospital, is essential for facilitating the treatment of such patients.
Acute liver failure (ALF) is a medical emergency characterized by hepatic encephalopathy in patients with recent-onset jaundice and coagulopathy. We present a case of a patient who developed ALF secondary to NK/T-cell lymphoma, with the diagnosis confirmed via histopathological analysis of the explanted after liver transplantation. A 50-year-old woman with no significant medical history was transferred to our institution with severe acute liver injury. On admission, she exhibited drowsiness, jaundice, and hepatosplenomegaly. The patient denied alcohol use but reported consuming horsetail tea (Equisetum spp). We hypothesized drug-induced liver injury as the main diagnosis, attributing to the use of horsetail tea. Despite intensive supportive care, her condition continued to deteriorate, prompting urgent deceased-donor liver transplantation. Histopathological analysis of the explant revealed hepatic parenchyma infiltration by atypical lymphoid cells positive for CD2, CD3, CD8, CD56, and CD38, with Ki-67 > 95% and positive EBV in situ hybridization. The graft biopsy and appendix showed identical findings, confirming a high-grade Stage IV extranodal NK/T-cell lymphoma (ENKTL). Given her critical condition, a modified chemotherapy regimen was initiated. Subsequent complications included severe chemotherapy-induced pancytopenia, febrile neutropenia, invasive aspergillosis, and multiorgan failure, culminating in death 33 days post-transplant. This case highlights the diagnostic challenges of rare non-Hodgkin lymphoma with extranodal presentations. Although horsetail tea is associated with liver injury, its contribution to ALF remains insufficiently defined, reinforcing the importance of excluding alternative causes. ENKTL and other malignancies should be considered in indeterminate ALF, even without imaging findings, atypical lymphocytes in peripheral blood/ascites, or overt clinical suspicion of malignancy.
Background:Acute liver injury is a severe disease in which a hepatic and later systemic inflammatory response is triggered, generally induced by paracetamol intoxication, undetermined causes, drugs, and hepatotropic and nonhepatotropic viruses. Case Summary:A 67-year-old immunocompetent male with severe acute liver injury secondary to Cytomegalovirus (CMV) infection presented with a 2-week history of anorexia, asthenia, adynamia, generalized weakness, myalgia, and jaundice. Laboratory tests revealed hyperbilirubinemia, hypertransaminasemia, coagulopathy, and acute kidney injury, and tests for hepatitis A, B, C, HIV, and autoimmune hepatitis were negative, while PCR was positive for CMV. Patient was treated with N-acetylcysteine, albumin, valganciclovir, and liver support therapy using the dual plasma molecular adsorption system (DPMAS). Fundus examination showed CMV retinitis, and liver biopsy confirmed acute hepatitis with CMV cytopathic changes and areas of hepatocyte regeneration. After 5 sessions of DPMAS and 4 weeks of antiviral therapy, he showed clinical and biochemical improvement with native liver recovery and was discharged with outpatient follow-up. Conclusion:This case highlights the successful early recognition and prompt initiation of appropriate treatment using antiviral therapy and liver support therapy in managing severe CMV-induced acute liver injury in an immunocompetent patient, potentially averting the need for liver transplantation.
Primary biliary cholangitis is a progressive disease with complications such as liver cirrhosis and hepatocellular carcinoma, and the treatment goal is to delay its progression. One of the markers for treatment response is alkaline phosphatase levels. Baricitinib has been used in one randomized controlled trial involving two patients to improve outcomes in unresponsive primary biliary cholangitis. We present a case of primary biliary cholangitis who had incomplete response to ursodeoxycholic acid, obeticholic acid, and fenofibrate but showed complete response to baricitinib in terms of sustained normalized alkaline phosphatase levels.
Metastatic recurrence to the parotid gland following liver transplantation has not been previously reported. We describe a case of a 63-year-old man with hepatocellular carcinoma secondary to alcoholic cirrhosis who was treated with transarterial chemoembolization and microwave ablation, achieving radiographic downstaging within the Milan criteria. He underwent liver transplantation with an exception Model for End-Stage Liver Disease score of 27. Explant pathology revealed moderately differentiated multifocal tumors with negative margins. He remained recurrence-free for 31 months under protocol-based surveillance. He then developed a facial mass; two core needle biopsies were nondiagnostic. Surgical resection confirmed moderately to poorly differentiated metastatic hepatocellular carcinoma involving the parotid gland and zygomatic arch. Tumor markers rose only modestly at the time of recurrence. Despite targeted therapy and radiation, the disease progressed, culminating in widespread metastasis and death 13 months after recurrence. Immunotherapy was deferred due to the risk of graft rejection, and conversion to an mTOR-based regimen was not recommended. This case highlights the challenges in long-term posttransplant surveillance, diagnostic limitations of core biopsy in atypical lesions, and systemic treatment constraints in immunosuppressed patients.
The 3-month, once-weekly regimen of isoniazid plus rifapentine (3HP) is widely used for latent tuberculosis infection (LTBI) because of its shorter duration and favorable adherence compared to isoniazid monotherapy. However, 3HP is associated with hepatotoxicity as well as systemic drug reactions (SDRs), characterized by rapid-onset flu-like symptoms, fever, myalgias, and rash, which may complicate therapy. We describe a healthy 26-year-old male diagnosed with latent tuberculosis who developed acute SDR symptoms accompanied by hepatotoxicity after his third dose of 3HP. The liver injury was initially cholestatic and evolved into a worsening hepatocellular pattern despite discontinuation of 3HP, with gradual normalization over 4 weeks. Autoimmune serologies were briefly positive but resolved spontaneously without intervention. This case illustrates the potential for SDRs with evolving liver injury during 3HP therapy and underscores the importance of early recognition of adverse effects and individualized management.
Drug-induced liver injury (DILI) is a rare but significant complication of commonly prescribed medications. This case describes a 69-year-old female presenting with clinical and laboratory features of mixed type liver injury, bisalbuminemia, and pseudohyponatremia. An extensive evaluation for obstructive, autoimmune, and infectious hepatitis yielded inconclusive results, necessitating advanced imaging and a liver biopsy. The biopsy revealed chronic hepatitis of unspecified etiology. On further inquiry, the patient disclosed having used amoxicillin-clavulanate for sinusitis 4 weeks prior to presentation, implicating this medication as the probable cause of her symptoms. The patient's condition improved with supportive care, with normalization of liver function within 4 months. This case underscores the importance of obtaining a detailed medication history to avoid unnecessary and costly diagnostic evaluations. Physicians must remain vigilant about rare adverse effects of commonly prescribed drugs and recognize their characteristic clinical patterns. Early identification and management can prevent complications, reduce unnecessary interventions, and optimize patient outcomes.
Azathioprine, an antimetabolite drug interfering with purines synthesis, is one of the immunosuppressive maintenance therapy used in solid organ transplant or dysimmune disease. We report the case of a 5-year-old-girl with Stage 5 chronic kidney disease, who developed hepatic peliosis due to azathioprine used 9 months after kidney transplant.
This case involves a 46-year-old obese, prediabetic female with a complex medical history. It illustrates several diagnostic challenges, including differentiating between thyroid storm-related liver injury and underlying chronic liver disease. The gradual progression to a definitive diagnosis of vanishing bile duct syndrome required multiple interventions and extensive evaluations.
