Differential Diagnosis, Investigations and Diagnosis

At presentation, the differential diagnosis included occult hepatobiliary infection, autoimmune or inflammatory disease, malignancy, and non‐cirrhotic portal hypertension.

Laboratory and ancillary investigations are summarized in Table 1. The patient had marked inflammatory activity with leukocytosis (21.7 × 109/L), neutrophilia (85%), ferritin 1095 ng/mL, and C‐reactive protein > 200 mg/L. Liver biochemistry showed mild‐to‐moderate cholestatic‐hepatitic abnormalities: alkaline phosphatase 251 U/L, alanine aminotransferase 112 U/L, and aspartate aminotransferase 107 U/L. Blood and urine cultures were negative, viral and tumor markers were negative, and thrombophilia screening was unrevealing. The extractable nuclear antigen profile showed isolated anti‐Sjögren syndrome type B (SS‐B/La) positivity, which was not considered sufficient to explain the overall presentation.

Parameter | Value | Reference range
Total white blood cell count | 21.7 × 10 9 /L | 4–11 × 109/L
Neutrophils | 85% | 40%–80%
Serum ferritin | 1095 ng/mL | 30–400 ng/mL
C‐reactive protein | > 200 mg/L | < 10 mg/L
Lactate dehydrogenase | 680 U/L | 135–225 U/L
Alanine aminotransferase | 112 U/L | 10–49 U/L
Aspartate aminotransferase | 107 U/L | 8–48 U/L
Alkaline phosphatase | 251 U/L | 30–130 U/L
Thyroid‐stimulating hormone | 2.5 μU/mL | 0.5–5 μU/mL
Urea and electrolytes | Normal | Not applicable
Random blood sugar | 5.5 mmol/L | < 7.8 mmol/L
Blood culture and sensitivity | No growth | Not applicable
Urine culture and sensitivity | No growth | Not applicable
Mantoux test | 2 mm | < 5 mm
Viral markers (HBV, HCV, HIV 1, and 2) | Negative | Not applicable
ICT for malaria and kala‐azar | Negative | Not applicable
Extractable nuclear antigen profile | Positive (SS‐B/La) | Negative
D‐dimer | 0.5 μg/mL | Up to 0.5 μg/mL
Fibrin degradation products | 6 μg/mL | < 10 μg/mL
Protein C and S | Negative | Not applicable
Antithrombin III | Negative | Not applicable
Tumor markers (AFP, CA19‐9, PSA, and CEA) | Negative | Not applicable
Electrocardiogram | Normal | Not applicable
Echocardiography | Normal study | Not applicable
Chest X‐ray (posteroanterior view) | Normal | Not applicable
Colonoscopy | Normal | Not applicable
Transient elastography (FibroScan) | 4.5 kPa | 2–7 kPa
Duplex study of hepatobiliary system | Cavernous transformation of the portal vein with mild portal hypertension | Not applicable

Upper gastrointestinal endoscopy revealed Grade II‐III esophageal varices and portal hypertensive gastropathy (Figure 1), whereas transient elastography was normal (FibroScan 4.5 kPa), making cirrhosis less likely.

FIGURE 1: Upper gastrointestinal endoscopy. Panels A and B demonstrate Grade II–III esophageal varices in the distal esophagus. Panels C and D show portal hypertensive gastropathy with a mosaic mucosal pattern.

Earlier ultrasonography, performed during the febrile phase, demonstrated bile duct dilatation without an obvious obstructing stone or definite stricture, which delayed identification of the source. Magnetic resonance cholangiopancreatography (MRCP) subsequently demonstrated abnormal biliary features together with portal vein thrombosis and collateralisation suggestive of cavernous transformation (Figure 2). Contrast‐enhanced computed tomography (CECT) of the abdomen then confirmed portal vein thrombosis with periportal serpiginous collateral vessels, establishing chronic portal vein thrombosis with cavernous transformation and portal biliopathy secondary to occult cholangitis (Figure 3).

FIGURE 2: Magnetic resonance cholangiopancreatography (MRCP). Panels A–D show abnormal hepatobiliary imaging with features of portal vein thrombosis and cavernous transformation (portal cavernoma). Panel D highlights the abnormality with an arrow.

FIGURE 3: Contrast‐enhanced computed tomography (CT) of the abdomen. Panels A–I demonstrate portal vein thrombosis with periportal serpiginous collateral vessels, consistent with cavernous transformation. Red arrowhead: Thrombus in portal vein, Yellow arrowhead: Dilatation of CBD and intrahepatic biliary tree.