C0596 - A Case of Idiopathic Non-Cirrhotic Portal Hypertension (INCPH) in a Patient With Klebsiella pneumoniae Pyogenic Liver Abscess (PLA)

Monday, October 24, 2022

3:00 PM – 5:00 PM ET

Location: Crown Ballroom

Presenting Author(s)

YK

Yassine Kilani, MD

Lincoln Medical and Mental Health Center
New York, NY

Yassine Kilani, MD¹, Dwayvania Miller, MD¹, Ebehiwele Ebhohon, MD, MPH², Mubarak Yusuf, MD³, Mohammad Aldiabat, MD¹, Albert Yao, MS⁴, Henry Ackerman, MS⁴, Anjana Pillai, MD⁵
¹Lincoln Medical and Mental Health Center, New York, NY; ²Lincoln Medical and Mental Health Center, Bronx, NY; ³NYCHHC/Lincoln Medical Center, New York, NY; ⁴New York Medical College, New York, NY; ⁵Morristown Medical Center, New Jersey, NJ

Introduction: Pyogenic Liver Abscess (PLA) remains rare in the United States (US). Higher incidences have been reported in Asian countries. Recent data shows that Klebsiella pneumoniae is becoming the leading bacteria in PLA internationally. Causes of bacterial seeding in the liver include biliary tract pathology, portal vein bacteriemia, systemic bacteremia, penetrating wounds or liver surgery. We describe a case of cryptogenic Klebsiella pneumoniae PLA, associated with an incidental finding of idiopathic non-cirrhotic portal hypertension (INCPH).

Case Description/Methods: A 55 year old Hispanic female, with no past medical history, presented with a 7 day history of nocturnal fever, dry cough, mild back pain, and jaundice. On admission, the patient was febrile with tachycardia and jaundice. Abdomen was soft and non tender with a negative Murphy’s sign. Laboratory workup revealed a normocytic anemia, leukocytosis with neutrophilia, and evidence of cholestasis (table 1). Blood cultures were drawn. Imaging with liver ultrasound (USG), Computer Tomography (CT) and Magnetic Resonance Imaging (MRI) showed a heterogeneous cystic mass in the left hepatic lobe, a heterogeneous hepatic parenchyma and a large stone in the fundus of the gallbladder without evidence of cholecystitis, along with signs of portal hypertension. CT guided drainage showed a purulent fluid. The patient was treated with antibiotics. Klebsiella pneumoniae was isolated in both blood and pus cultures. Further workup for etiology of liver disease was inconclusive. A CT-guided liver biopsy was performed and showed evidence of non-cirrhotic portal fibrosis (NCPF) (image 1). The diagnosis of INCPH was made.

Discussion: The incidental discovery of portal hypertension in patients with underlying liver disease should prompt a full diagnostic workup to rule out liver cirrhosis and towards finding etiology for liver disease. Diagnosis of INCPH requires clinical signs of portal hypertension (ascites, esophageal &/or gastric varices, splenomegaly/ hypersplenism, porto-venous collaterals), along with patent portal veins on imaging (doppler US or CT), exclusion of cirrhosis on liver biopsy, and exclusion of liver diseases that might cause either cirrhosis (Hepatitis B &/or C, non alcoholic steatohepatitis, hemochromatosis, Wilson’s disease, primary biliary cirrhosis) or INCPH (Congenital liver fibrosis, sarcoidosis, schistosomiasis). The management of INCPH, regardless of the cause, involves reducing the portal pressure to prevent variceal bleeding and death.

Figure: Image 1 - Normal liver parenchyma with a few chronic inflammatory cells within portal tracts (Image A, hematoxylin-eosin stain, see arrow), with mild portal fibrosis on trichrome stain (Image B, see arrow), and normal reticulin pattern of hepatic plates on reticulin stain (Image C, see arrow) indicating the absence of liver fibrosis (original magnification X100).

Laboratory test	Results (Normal ranges)
Complete Blood Count (CBC)	Hemoglobin 10.6 g/dL (12.0 - 16.0 g/dL) MCV 89.4 fL (80.0 - 99.9 fL) Leukocytes 12910 cells/uL (4.80 - 10.8 cells/uL) Neutrophils 86.8 % (44.0 - 77.0 %)
Liver Function Tests (LFT)	AST 35 (< = 32 U/L) ALT 49 (< = 33 U/L) ALP 301 (35 - 105 U/L) Total bilirubin 3.04 (0.20 - 1.20 mg/dL) Direct bilirubin 1.90 (0.00 - 0.30 mg/dL) Albumin 3.3 g/dL (3.5- 5.2 g/dL)
Basic metabolic panel	BUN 10.0 mg/dL (6.0 - 23.0 mg/dL) Creatinine 0.70 (0.50 - 0.90 mg/dL) Sodium 126 mmol/L (136 - 145 mmol/L) Potassium 3.7 mmol/L (3.5 - 5.1 mmol/L)
Lipid panel	Total cholesterol 91 mg/dL (< = 200 mg/dL) HDL cholesterol 19 mg/dL ( >= 50 mg/dL) LDL cholesterol 53 mg/dL (< = 129 mg/dL) Triglycerides 94 mg/dL (< = 150 mg/dL)
Iron studies	Iron 26 ug/dL (30-160 ug/dL) TIBC 171 ug/dL (220-430 ug/dL) Ferritin 243 ng/dL (15/150 ng/mL)
Bacterial cultures	Blood culture: Growth Klebsiella pneumoniae Abscess fluid culture: Growth Klebsiella pneumoniae
Viral hepatitis panel	Hepatitis B surface antigen (HBsAg) non reactive Hepatitis B surface antibody (HBsAb) non reactive Hepatitis C virus (HCV) antibody non reactive
Liver parasites serology	Schistosoma antibodies IgG negative Entamoeba histolytica serology negative Echinococcus antibodies IgG negative
Autoimmune workup	Antinuclear antibodies negative Anti-smooth muscle antibodies weakly positive 1:20 Anti-LKM antibodies negative < 20.1 Antimitochondrial antibodies negative < 1:20 Anti neutrophil cytoplasmic antibodies negative
Tumor markers	Alpha fetoprotein (AFP) negative Carcinogenic Antigen 19-9 (CA-19-9) negative

Table: Table 1. Initial laboratory workup

Disclosures:

Yassine Kilani indicated no relevant financial relationships.

Dwayvania Miller indicated no relevant financial relationships.

Ebehiwele Ebhohon indicated no relevant financial relationships.

Mubarak Yusuf indicated no relevant financial relationships.

Mohammad Aldiabat indicated no relevant financial relationships.

Albert Yao indicated no relevant financial relationships.

Henry Ackerman indicated no relevant financial relationships.

Anjana Pillai indicated no relevant financial relationships.

Yassine Kilani, MD¹, Dwayvania Miller, MD¹, Ebehiwele Ebhohon, MD, MPH², Mubarak Yusuf, MD³, Mohammad Aldiabat, MD¹, Albert Yao, MS⁴, Henry Ackerman, MS⁴, Anjana Pillai, MD⁵. C0596 - A Case of Idiopathic Non-Cirrhotic Portal Hypertension (INCPH) in a Patient With Klebsiella pneumoniae Pyogenic Liver Abscess (PLA), ACG 2022 Annual Scientific Meeting Abstracts. Charlotte, NC: American College of Gastroenterology.