Transjugular Liver Biopsy Research Articles

Porto-sinusoidal vascular disorder (PSVD): Application of new diagnostic criteria in a multicenter cohort of patients

Background and aimThe term porto-sinusoidal vascular disorder (PSVD) was recently proposed to replace that of idiopathic non-cirrhotic portal hypertension (INCPH) to describe patients with typical histological lesions in absence of cirrhosis, irrespective of the presence/absence of portal hypertension (PH), and new diagnostic criteria were defined. The study aimed to compare the applicability between the diagnostic criteria of PSVD and those of INCPH. Materials and methods53 patients affected by PSVD were enrolled. Biochemical, clinical, ultrasound and histological data, the presence and type of associated diseases were recorded in a database. According to the new criteria, histological data and signs of PH were divided into specific and non-specific. Percutaneous and transjugular biopsies were compared to establish the usability of the two methods for diagnostic purposes. ResultsIn 85% of the patients the diagnosis of PSVD was obtained by applying the first criterion (25 had specific histological signs with specific signs of PH); one patient presented with specific histological signs but no PH. In 8 patients the diagnosis was obtained by applying the second criterion. 19% of patients had portal vein thrombosis. Finally, the prevalence of the various histological lesions was similar between the patients submitted to percutaneous and transjugular liver biopsy. ConclusionsThe study confirms that the diagnostic criteria of PSVD lead to the inclusion of a greater number of patients than INCPH.

Liver Biopsy and Pressure Hemodynamics: IR Perspective

AbstractLiver biopsy is important for staging of liver disease and cirrhosis and can be performed by many techniques. While percutaneous biopsy is fast, safe, effective, and widely available, transvenous biopsy, most often performed from the transjugular approach, offers many advantages for selected patients. In the setting of suspected or confirmed portal hypertension, transjugular liver biopsy (TJLB), most commonly performed by interventional radiologists, can be used to simultaneously obtain core biopsies of the liver and measure hemodynamic pressures in the right atrium, hepatic vein, and portal vein to calculate a hepatic venous pressure gradient. TJLB can be performed safely when percutaneous biopsy is contraindicated, including in the setting of ascites, coagulopathy, anticoagulation, and other high-risk bleeding situations. The procedure can be performed in the outpatient setting in a short period of time for most patients and without the use of precious anesthesia resources. Hepatic venography also allows for variant anatomy evaluation that may be important in the diagnosis and planning of future procedures, such as transjugular intrahepatic portosystemic shunt.

Concordance between HokUS-10 Scoring and Transjugular Liver Biopsy for the Diagnosis of Veno-Occlusive Disease/Sinusoidal Obstruction Syndrome after Allogeneic Stem Cell Transplantation

The aim of this study was to evaluate the concordance between clinical diagnosis and pathologic findings of veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) in post-hematopoietic stem cell transplantation recipients and to investigate the accuracy of the HOKUS-10 score in diagnosing VOD/SOS. We included 13 patients who underwent transjugular liver biopsy for clinical suspicion of VOD/SOS and collected their clinical, laboratory, imaging, and pathologic data. Eleven patients were confirmed to have VOD/SOS by pathologic examination. The median HokUS-10 score and hepatic venous pressure gradient were 6 points (range, 0 to 10 points) and 13 mmHg (range, 7 to 24 mmHg), respectively. There was no significant difference between these scores in VOD/SOS and non-VOD/SOS cases; however, patients with lower HokUS-10 scores tended to have milder histologic features of VOD/SOS compared with severe cases. This study highlights the potential discordance between clinical diagnosis and pathologic diagnosis of VOD/SOS and emphasizes the importance of liver biopsy to optimize treatment.

Relapsed nodular lymphocyte-predominant Hodgkin lymphoma presenting as severe paraneoplastic hepatitis: a case report

BackgroundHematological malignancies are an infrequent but important cause of liver dysfunction. There are several mechanisms by which this can occur, including direct malignant infiltration of the hepatic parenchyma and/or vasculature, vanishing bile duct syndrome, and paraneoplastic hepatitis. Paraneoplastic hepatitis is an extremely rare mechanism by which a hematological malignancy can cause liver dysfunction, and we present the first case, to our knowledge, of paraneoplastic hepatitis caused by nodular lymphocyte-predominant Hodgkin lymphoma in the literature.Case presentationA 28-year-old Caucasian male presented with 3 weeks of fatigue, epigastric pain, and jaundice. His medical history was significant for early stage nodular lymphocyte-predominant Hodgkin lymphoma in the cervical region in remission for 5 years after primary treatment with involved-field radiotherapy. Liver biochemistry was normal at the time of treatment for lymphoma and there was no known liver disease before the current presentation. On physical examination, there was scleral icterus and ecchymoses, but no evidence of hepatic encephalopathy, other stigmata of chronic liver disease, or lymphadenopathy. A computed tomography scan of his neck, chest, abdomen, and pelvis showed heterogeneous enhancement of the liver, multiple enlarged upper abdominal lymph nodes, and an enlarged spleen with multiple rounded lesions. Portal and hepatic veins were patent. Initial workup for viral, autoimmune-, toxin-, and medication-related hepatitis was negative. A transjugular liver biopsy was performed with histology showing a predominantly T-cell mediated hepatitis with very extensive multiacinar hepatic necrosis, but no evidence of lymphoma within the liver. Retroperitoneal lymph node biopsy revealed nodular lymphocyte-predominant Hodgkin lymphoma. The patient’s symptoms, bilirubin, and transaminases improved significantly after treatment with oral prednisolone and a staged introduction of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone chemotherapy.ConclusionsNodular lymphocyte-predominant Hodgkin lymphoma may cause paraneoplastic hepatitis. Physicians should be aware of the possibility of this life-threatening presentation and the importance of early liver biopsy and treatment before acute liver failure occurs. Interestingly, paraneoplastic hepatitis did not occur when nodular lymphocyte-predominant Hodgkin lymphoma was first diagnosed and confined to the cervical region, but was the presenting feature of the recurrence below the diaphragm.

FRI-389 - With-in patient comparison of single-cell versus single-nucleus sequencing on human transjugular liver biopsies

FRI-389 - With-in patient comparison of single-cell versus single-nucleus sequencing on human transjugular liver biopsies

Cemiplimab-Associated Sinusoidal Obstruction Syndrome

A 58-year-old woman developed new-onset recurrent ascites after the recent initiation of cemiplimab for the treatment of advanced basal cell carcinoma. A comprehensive serological workup for viral, metabolic, and autoimmune causes was unrevealing. Transjugular liver biopsy demonstrated parenchymal changes consistent with a diagnosis of sinusoidal obstruction syndrome. While this is a condition commonly observed in patients after hematopoietic stem cell transplantation or use of chemotherapeutic agents, it should also be considered in patients who develop new-onset liver dysfunction after the initiation of checkpoint inhibitors.

Abstract No. 223 Intravascular Ultrasound-Guided Transvenous Biopsy of Retroperitoneal Lymph Nodes: Efficacy and Safety Compared with Percutaneous CT-Guided Biopsy

Intravascular ultrasound-guided transvenous biopsy (IVUS-TVB) is a recently described technique using intracardiac echocardiography catheter visualization to sample targets adjacent to the IVC or iliac veins using a transjugular liver biopsy kit. Although IVUS-TVB has been shown to be technically feasible, the efficacy and safety have not been directly compared with conventional approaches such as CT-guided percutaneous needle biopsy (CT-PNB) for similar targets. Therefore, the purpose of this study was to compare diagnostic accuracy and adverse event rates for IVUS-TVB versus CT-PNB for retroperitoneal (RP) lymph nodes. A single-institution, retrospective study comparing IVUS-TVB to CT-PNB was conducted. From December 17, 2020, to August 10, 2022, 53 IVUS-TVBs were performed, of which 32 were of RP lymph nodes, a non-overlapping sample from that previously reported. For comparison, a search of CT reports from January 1, 2013, to January 1, 2022, using the terms “retroperitoneal and lymph and needle and biopsy,” was performed, yielding 191 CT-PNB procedures. Patient demographics, lymph node size/location, needle passes, procedure time, diagnostic accuracy, and adverse events were compared via t-tests for quantitative variables and chi-squared or Fisher’s exact tests for qualitative variables. The diagnostic accuracy and adverse event rate for IVUS-TVB were compared with CT-PNB as the reference standard using the Farrington-Manning non-inferiority test assuming a 10% non-inferiority margin. There was no significant difference in age, Charlson comorbidity score, or number of needle passes between groups. IVUS-TVB targets were slightly smaller (2.4 vs 2.7 cm, long-axis, P = 0.04, 1.7 vs 2.0 cm, short-axis, P = 0.04) and primarily precaval (34%) or aortocaval (47%) in location (vs predominantly left periaortic (80%) for CT-PNB, P< 0.001). Procedure time was approximately 33% longer for IVUS-TVB (64 vs 48 minutes, P< 0.001). Diagnostic accuracy, defined as diagnostic of malignancy or a clinically verifiable benign result, was 94% for IVUS-TVB compared with 88% for CT-PNB. Overall adverse event rate for IVUS-TVB was 3.1% compared with 3.7% for CT-PNB. IVUS-TVB was non-inferior to CT-PNB for both diagnostic accuracy (P = 0.02) and adverse event rate (P = 0.04). This study demonstrated non-inferiority of IVUS-TVB compared with CT-PNB with respect to diagnostic accuracy and adverse event rate for biopsy of RP lymph nodes adjacent to the IVC. These data suggest that IVUS-TVB is as safe and effective as a conventional biopsy approach such as CT-PNB for similar targets.

Abstract No. 575 Safety and Success of Transjugular Liver Biopsies in Children: A Single Tertiary Care Center Experience

Abstract No. 575 Safety and Success of Transjugular Liver Biopsies in Children: A Single Tertiary Care Center Experience

Advances in Endoscopic Ultrasound (EUS)-Guided Liver Biopsy.

Recent years have seen the emergence of endoscopic-ultrasound-guided liver biopsy (EUS-LB) as an effective alternative to traditional (percutaneous or transjugular) liver biopsy techniques. Comparative studies have demonstrated that both endoscopic and non-endoscopic approaches are similar in terms of diagnostic adequacy, accuracy, and adverse events; however, EUS-LB offers the advantage of reduced recovery time. Additionally, EUS-LB enables the sampling of both lobes of the liver as well as the advantage of portal pressure measurements. However, EUS-LB may be argued to have a high cost, although this procedure can be cost-effective if bundled with other endoscopic procedures. Approaches utilizing EUS-guided liver therapy, such as the administration of chemotherapeutic agents and EUS elastography, are in development, and their optimal integration into clinical care is likely to emerge in the coming years. In the present review, we evaluate the available literature on EUS-LB indications, contraindications, variations in needle biopsy techniques, comparative outcomes, advantages and disadvantages, and future trends and perspectives.

Hepatic venous pressure gradient evaluates the Scheuer stage of liver fibrosis by transjugular liver biopsy: a multicenter study

BackgroundHepatic venous pressure gradient (HVPG) is the criterion for assessing sinusoidal portal hypertension. Using HVPG to assess the degree of liver fibrosis by transjugular liver biopsy (TJLB) is still being explored, as no data has been shown that portal hypertension may already be present in patients with advanced hepatic fibrosis (Scheuer stage ≥ S3). The objective of this study was to observe whether portal hypertension exists before progressing to cirrhosis (Scheuer stage = S4). MethodsFifty patients who underwent TJLB and HVPG were enrolled. The correlation between Scheuer stage and HVPG was analyzed using the Pearson correlation coefficient, and the ROC curve predicted the diagnostic value of HVPG in patients with hepatic fibrosis. ResultsThe Scheuer stage and HVPG significantly correlated (r = 0.654, p < 0.001). The AUC of HVPG in predicting advanced liver fibrosis was 0.896, and of cirrhosis was 0.810. There were 45 patients with portal hypertension (HVPG> 5 mmHg), 12 with S3, 29 with S4; There were 42 patients with CSPH (HVPG≥ 10 mmHg), 11 with S3, and 29 with S4. ConclusionHVPG is valuable in evaluating the Scheuer stage of liver fibrosis in patients with TJLB. Portal hypertension might already exist before the progression to cirrhosis in some patients.

Clinical significance of measuring hepatic venous pressure gradient on transjugular liver biopsy for patients with pre-cirrhotic bridging fibrosis liver disease

PurposeTo demonstrate that patients with pre-cirrhotic bridging fibrosis (Meta-analysis of Histological Data in Viral Hepatitis, METAVIR stage F3) and clinically significant portal hypertension (CSPH, Hepatic Venous Pressure Gradient ≥10 mmHg) experience a higher rate of clinical decompensation than patients without CSPH. Materials and methods128 consecutive patients with pathology proven bridging fibrosis without cirrhosis between 2012 and 2019 were reviewed. Inclusion criteria were patients with HVPG measurement obtained during the same outpatient transjugular liver biopsy and clinical follow up of at least two years. Primary endpoint included rate of overall complication related to portal hypertension including evidence of either ascites, presence of varices on imaging or endoscopy, or evidence of hepatic encephalopathy. ResultsAmong 128 patients with bridging fibrosis (67 females and 61 males; average age 56 years), 42 (33%) and 86 (67%) were with and without CSPH (HVPG ≥10 mmHg). Median follow-up time was 4 years. Rate of overall complication (either ascites, varices or hepatic encephalopathy) in patients with and without CSPH was 36/42 (86%) and 39/86 (45%) (p < .001) respectively. Rate of developing ascites, varices and hepatic encephalopathy in patients with and without CSPH was 21/42 (50%) vs 26/86 (30%) (p = .034), 32/42 (76%) vs 26/86 (30%) (p ≤ .001) and 18/42 (43%) vs 12/86 (14%) (p = .001) respectively. ConclusionPatients with pre-cirrhotic bridging fibrosis and CSPH were associated with higher rates of developing ascites, varices and hepatic encephalopathy. Measuring HVPG during transjugular liver biopsy provides additional prognostic value in anticipating clinical decompensation in patients with pre-cirrhotic bridging fibrosis.

Combined heart-liver transplantation practices survey in North America: Evaluation and organ listing practices.

We conducted a web-based survey to characterize liver transplant (LT) evaluation and listing practices for patients being evaluated for combined heart-liver transplantation (CHLT), with a specific emphasis on patients with congenital heart disease (CHD), around transplant centers in North America. Very few protocols for liver evaluation and listing in patients undergoing combined heart-liver transplantation are published, and no guidelines currently exist on this topic. A subject of intense debate in the transplant community is the decision of which patients with CHD and liver disease benefit from CHLT compared with heart transplantation. A focus group from the American Society of Transplantation Liver-Intestine Community of Practice Education Subcommittee developed a web-based survey that included questions (1) respondee demographic information; (2) LT evaluation practices in CHLT; (3) liver organ listing practices in CHLT, and (4) 4 clinical vignettes with case-based scenarios in CHLT liver listings among CHD patients who underwent Fontan palliation. The survey was distributed to medical and surgical LT program directors of 47 centers that had completed at least 1 CHLT up to July 2021 in the US and the University of Toronto, Canada. The survey had an excellent 83% response rate (87% for centers that completed at least 1 CHLT in the past 5y). Total 66.7% used transjugular liver biopsy with HVPG measurements, 30% used percutaneous liver biopsy with no consensus on the use of a fibrosis staging system, 95% mandated contrasted cross-sectional imaging, and 65% upper endoscopy. The following isolated findings evaluation mandated CHLT listing: isolated elevated HVPG (61.5%); the presence of portosystemic collaterals on imaging (67.5%); the endoscopic presence of esophageal or gastric varices (75%), and the presence of HCC (80%), whereas the majority of centers did not feel that the presence of isolated splenomegaly (100%), thrombocytopenia (81.6%), endoscopic findings of portal hypertensive gastropathy (66.7%), or highly sensitized patients (84.6%) justified CHLT. In our survey of North American centers that had performed at least 1 CHLT in the past 5 years, we observed heterogeneity in practices for both evaluation and listing protocols in these patients.

Diagnostic outcome and safety of plugged liver biopsy in high-risk patients: a systematic review and meta-analysis.

Percutaneous liver biopsy is the gold standard test for the assessment of liver disease. In patients with coagulopathy, ascites, post-transplantation, and hypervascular tumors, biopsy is associated with increased risk of adverse events (AEs). Transjugular liver biopsy (TJLB) is recommended in these conditions but is expensive and unavailable at many centers. Plugged liver biopsy (PLB) provides an alternate diagnostic modality in these high-risk cases. To analyze the pooled diagnostic outcome and safety of PLB in high-risk cases. A literature search of various databases from January 1990 to July 2022 was conducted for studies evaluating the outcome of PLB in high-risk cases. The primary outcomes were pooled sample adequacy and AEs. Pooled event rates across studies were expressed with summative statistics. A total of 17 studies (2329 patients) were included in the meta-analysis. The pooled proportion of sample adequacy was 98.9% (95% confidence interval [CI]=98.2-99.6). Severe AEs, major bleeding, and minor AEs were seen in 0.7% (95% CI=0.1-1.3), 0.4% (95% CI=0.1-0.8), and 11.5% (95% CI=2.4-20.6) of the patients. There was only one reported mortality, giving a pooled incidence of 0.0002% (95% CI=0.0-0.0038). Compared to TJLB (5 studies, n = 336), there was no difference in either sample adequacy (odds ratio [OR]=2.34, 95% CI=0.83-6.58) or risk of serious AEs (OR=0.47, 95% CI=0.173-1.31). PLB can be safely performed on patients with coagulopathy and/or ascites with high sample adequacy rates and low incidence of AEs and mortality.

Predicting bleeding after liver biopsy using comprehensive clinical and laboratory investigations: A prospective analysis of 302 procedures

BackgroundLiver biopsy carries a small risk of bleeding complications. No validated clinical or laboratory tool helps predict liver biopsy–related bleeding. ObjectivesTo determine whether global hemostasis tests and/or a clinical questionnaire could identify patients at risk of liver biopsy–related bleeding. Patients/MethodsConsecutive patients scheduled for liver biopsy with an overnight hospital stay were prospectively included. Before liver biopsy, routine hemostasis tests, Platelet Function Analyzer 100, thromboelastometry, thrombin generation assay, plasma clot lysis time, and a clinical questionnaire were performed. Bleeding was defined as a liver hematoma or new free fluid on a systematic ultrasound performed 24 h after liver biopsy or a decrease in hemoglobin level of 2 g/dL or more in patients with pre‐existing free fluid in the abdominal cavity. ResultsThree hundred two patients were included: 173 underwent percutaneous and 129 transjugular liver biopsy. There were 21 bleeding episodes (7%); 20 based on ultrasonographic criteria, 1 on laboratory criteria. None of the hemostasis tests and no item of the clinical questionnaire were associated with liver biopsy–related bleeding in the overall study group. Same results were obtained in subgroup analyses focusing on patients who underwent percutaneous liver biopsy, transjugular liver biopsy, or on patients with cirrhosis. Pain 2 h after liver biopsy was more frequent in patients with liver biopsy–related bleeding (55% vs. 23% p = .002). ConclusionsAn extensive hemostasis workup, including global hemostasis assays, does not improve prediction of liver biopsy–related bleeding. Pain 2 h after liver biopsy should alert the clinician to the possibility of procedure‐related bleeding.

Expert consensus on transjugular liver biopsy

The liver biopsy is one of the most commonly used and important diagnostic methods in clinical hepatology. Transjugular liver biopsy (TJLB) can safely used in patients with severe coagulopathy and/or prehepatic ascites, thereby expanding the indication of liver biopsy. However, there is currently no TJLB-specific procedure guidance, standard process for pathological sampling and tissue specimen processing in China. Therefore, the Chinese Society of Hepatology of the Chinese Medical Association invited experts in the relevant fields to compile a consensus regarding the indications, contraindications, procedure methods, pathological specimen collection, tissue process, and other issues with the aim to facilitate the more reasonable application of TJLB in clinical practice.

S3075 Massive Splenomegaly With Non-Cirrhotic Portal Hypertension

Introduction: A 42 year old male with a 12 year history of Common Variable Immune Deficiency (CVID) presented for management of “cirrhosis.” His only subjective complaint was mild non-specific abdominal pain. He noted no history of jaundice, weight loss or constitutional symptoms. He did not drink alcohol. He was receiving immune globulin (Gamunex-C) infusions every 28 days. Case Description/Methods: Physical exam revealed a middle aged male in no acute distress with stable VS with mild diffuse abdominal tenderness and “fullness." Remainder of exam was unremarkable. An abdominal CT scan was obtained that showed massive splenomegaly (Image 1), portal vein enlargement, no ascites. He had intact hepatic synthetic and excretory function, normal aminotransferases but an elevated alkaline phosphatase. An EGD done three years previously showed minimal esophageal varices. A transjugular liver biopsy was obtained that showed minimal hepatic fibrosis but features of nodular regenerative hyperplasia (NRH). (Figure) Discussion: CVID is the most common immunodeficiency disease with a prevalence of 1 in 25,000. It is a primary B-cell immunodeficiency disorder with hypogammaglobulinemia. It can occur at any age, without gender preference and can affect multiple organ systems to include the liver with hepatic fibrosis and portal hypertension. The most common hepatic dysfunction in CVID is nodular regenerative hyperplasia (NRH) occurring in 5-10% in this population. Depending on clinical practice patterns the gastroenterologist could be presented with this scenario de novo or in consultation with Allergy/Immunology or Infectious Disease subspecialties. NRH in this population has been divided into three categories: Category 1; non-progressive, Category 2; NRH with portal hypertension and splenomegaly (as in our case presentation) and Category 3; NRH with features of autoimmune hepatitis. Repeated hepatic cell injury with concurrent regeneration causing compression of hepatic parenchyma, or of the portal vein and central veins is thought to result in portal hypertension, esophageal varices and splenomegaly. The patient can present anywhere along this variable clinical spectrum of development. Biopsies typically show a lack of perinodular fibrosis or intrahepetic shunts. Category II patients despite immunoglobulin replacement, can have infectious complications due to neutropenia. Category III patients can be progressive. Awareness of this entity is essential to optimize treatment strategies.Figure 1.: Splenomegaly calculated volume of 2000 cc, engorged splenic vein.

S2939 Ashwagandha Toxicity: A Rare Case of Drug Induced Liver Injury (DILI)

Introduction: The pursuance of natural and herbal remedies by many has brought with it further knowledge of the many causes of Drug Induced Liver Injury (DILI). Many of these "natural" over-the-counter (OTC) supplements may not be benign due to the lack of scientific analysis and unregulated production. Ashwagandha is a supplement used for memory enhancement, management of anxiety, and general increase in vitality. We present a case of Ashwagandha DILI in a healthy 36-year-old man. Case Description/Methods: A 36-year-old male presented to our institution with 1 week of fatigue, jaundice, nausea and subjective fevers. Upon presentation he was hemodynamically stable and afebrile. Physical exam was notable for jaundice without other stigmata of liver disease or mental status changes. He was found to have acute liver injury with lab chemistries remarkable for AST= 1482 U/L, ALT= 1375 U/L, Tbili= 22.3 mg/dL, alkaline phosphatase= 202 U/L and an INR of 1.6. He denied alcohol and drug use, recent travel or sick contacts. Medications list was notable for cetirizine, diphenhydramine (both taken as needed), OTC testosterone supplements, OTC apple cider vinegar gummies BID and OTC ashwagandha gummies BID. Workup was negative for autoimmune, infectious, metabolic, obstructive and vascular causes of liver injury. Acetaminophen level and urine drug screen were negative. Trans-jugular liver biopsy revealed evidence of acute portal and lobular hepatitis and cholestasis, suggestive of DILI. Patient's liver enzymes continued to downtrend (AST=929, ALT=765, Tbili=22.5) after withdrawal of his supplements and was discharged (Figure). Discussion: Ashwagandha causing DILI has been infrequently reported in the literature. One case series of 5 patients in Iceland and a single case in Japan reported that ashwagandha is an uncommon culprit linked to acute liver injury. None of the reported cases necessitated liver transplant. Average time to resolution and normalization of LFTs was 3.5 months and R values usually ranged in the mixed range (R value 2-5). The case series also revealed that ashwagandha's toxicity could be dose dependent, as higher peaks in LFT's were tied to recent increases in ashwagandha dosing. Although ashwagandha has been a rarely reported cause of DILI, a thorough history of medication and OTC supplements in patients with undifferentiated acute liver injury may yield more cases. Further study and reporting of DILI in the ever-growing supplement market appears to be required to minimize potential injury.Figure 1.: Tranjugular Liver Biopsy Findings.

SEVERE COVID-19 COMPLICATED WITH HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS: A CASE REPORT

SEVERE COVID-19 COMPLICATED WITH HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS: A CASE REPORT

S3168 Sinusoidal Obstruction Syndrome: A Known and Often Missed Complication of Oxaliplatin Therapy

Introduction: Sinusoidal obstruction syndrome (SOS) is caused by sinusoidal endothelial injury due to hematopoietic cell transplantation, liver irradiation or exposure to chemotherapy such as oxaliplatin and may occur days to weeks following exposure. SOS can be a life-threatening complication and its presentation may include jaundice, volume overload or hepatic failure. We present the case of a 32-year-old male with gastroesophageal adenocarcinoma who underwent oxaliplatin-based chemotherapy and developed SOS. Case Description/Methods: A 32-year-old male with no significant medical history presented with dysphagia and weight loss. Esophagogastroduodenoscopy showed a mass at the gastroesophageal junction. He underwent diagnostic laparoscopy which showed peritoneal metastasis. Tissue biopsy was consistent with stage 4 gastroesophageal junction adenocarcinoma. He was started on 5-fluorouracil (5-FU), oxaliplatin and nivolumab. The patient developed thrombocytopenia and moderate aminotransferase elevation after 6 cycles of chemotherapy. An abdominal ultrasound obtained after 10 of 12 cycles of chemotherapy showed portal hypertension. Subsequent abdominal imaging showed worsening ascites (serum ascites to albumin gradient > 1.1), anasarca and splenomegaly. Transjugular liver biopsy showed elevated portal hepatic venous pressure gradient, focal sinusoidal dilatation and centrizonal ischemic changes suggesting drug induced hepatopathy, and a diagnosis of oxaliplatin induced SOS was made. The patient was not a candidate for defibrotide and was treated conservatively. He continued to have worsening ascites with severe direct hyperbilirubinemia and moderately elevated aminotransferases without biliary obstruction (Figure). Discussion: Oxaliplatin-based chemotherapies are known to cause the potentially life-threatening complication of SOS which is oftentimes missed. Our patient developed signs and symptoms suggestive of SOS by cycle 10 of oxaliplatin therapy. These changes included thrombocytopenia, elevated liver associated enzymes, volume overload and portal hypertension. He went on to complete 12 cycles of oxaliplatin with eventual worsening of his portal hypertension related symptoms and worsening of his hepatic function. This case highlights the importance of having a high index of suspicion for the development of SOS with plans to pursue patient-centered discussions regarding alternative chemotherapeutic regimens, instituting prophylactic therapies and timely SOS screening protocols.Figure 1.: Timeline of events leading up to SOS diagnosis.

S2994 Fulminant Liver Failure Due to Budd-Chiari Syndrome Triggered by Strenuous Exercise

Introduction: This case illustrates the complexity of diagnosis of Budd-Chiari syndrome especially with an atypical presentation. Case Description/Methods: A 27-year-old man in his usual good state of health attended an all-day police academy fitness test. That evening he developed abdominal pain, nausea, vomiting, and diarrhea. He left work the next day due to these symptoms and progressive confusion. His family called EMS and he was transported to a local emergency department where he was found to be hypothermic, tachycardic, and hypoxemic. Labs were notable for acute kidney injury with creatinine 1.7, lactate 11.8, total bilirubin 1, AST 116, and ALT 95. He was begun on 2 vasopressors and empiric antibiotics. CT Abdomen noted a possible mesenteric thrombus; he was taken emergently to the operating room, but no bowel ischemia was found. His laboratory tests increased over the next 3 days reaching total bilirubin 3.0, AST 8560, ALT 5880, alkaline phosphatase 92, albumin 2.7, creatinine 3.1, INR 5.06, WBC 34.5, and ferritin 18,724. He was subsequently transferred to a tertiary center where his mental status worsened, and he was intubated. A transjugular liver biopsy demonstrated perivenular coagulative necrosis involving 80% of the hepatic parenchyma, and a hepatic venogram noted extensive portal vein thrombus. He had no history of taking any medications on a regular basis and did not take acetaminophen. An acetaminophen level was negative. Orthotopic liver transplantation was performed on the sixth day after his initial hospital presentation. During the surgery the patient was noted to have extensive thrombus involving the portal system including the main portal vein as well as the superior mesenteric vein, and thrombus in the hepatic veins. Testing for hypercoagulable state was positive for JAK2 V617F mutation, and he was begun on treatment with hydroxyurea. Discussion: His development of Budd-Chiari syndrome is felt to be due to this hypercoagulable state combined with dehydration from strenuous exertion. There was no hepatomegaly noted on imaging which had led to confusion regarding the diagnosis of Budd-Chiari syndrome, but the occlusion of the portal vein in this patient resulted in decreased blood flow to the liver and prevented the development of hepatomegaly. His initial development of lactic acidosis, leukocytosis, and elevated ferritin is felt to be the result of marked hepatic necrosis from the Budd-Chiari syndrome but had led to confusion over the possibility of septic shock or bowel ischemia.