The “truth” behind Budd-Chiari syndrome | case study

< /polyline>case data

male, 62 years old, recently diagnosed with Budd-Chiari syndrome (Budd– Chiari syndrome), was admitted to the liver ward of our hospital. No past medical history. Engaged in construction industry, retired for 6 months.

Although the patient had abstained from alcohol for 6 months, laboratory tests showed γ-glutamyltransferase (858 U/L ) and alkaline phosphatase (228 U/L) were elevated, and prothrombin time, transaminases, and bilirubin levels were normal. Physical examination revealed hepatomegaly and abdominal distention without evidence of cirrhosis.

Liver ultrasonography (Panel A) showed Budd-Chiari syndrome with thrombosis in the middle and right hepatic veins (white arrow), low content of ascites, and a round hypointense calcified mass (4cm in diameter) was seen at the junction of the hepatic vein on the top of the liver.

CT failed to determine the exact nature of the liver mass However, hypodense lesions with central calcification of the liver dome (white arrows in panels B and C), ascites due to portal hypertension (orange arrows in panel C), and damage to the middle and right hepatic veins (white arrows in panel B) were confirmed. ).

Serological results for acute viral hepatitis were negative. Levels of tumor markers (alpha-fetoprotein, carbohydrate antigen 19-9, and carcinoembryonic antigen) were normal. Hydatid serology was negative. Upper gastrointestinal endoscopy revealed portal hypertensive gastropathy without esophagogastric varices.

After the patient was admitted to our hospital, he underwent ultrasound-guided percutaneous liver biopsy.

Q: What is the patient’s most likely diagnosis based on imaging findings and negative laboratory findings?

answers revealed

Answer revealed: Brucella hepatica Abscess (also known as brucella tumor)

Histopathological analysis of the mass showed partial calcification and necrosis ( 2 asterisks in panel D) with a ring of epithelioid cells (asterisk) and few plasma cells (arrowheads), no giant cells.

hours after biopsy The patient presented with fever and abdominal pain. Blood cultures detected Brucella. Brucella serology showed high titers of IgG and negative for IgM (ELISA technique). Serum agglutination test (Wright) test was positive for Brucella ovalis antigen with a titer of 1/160.

Radiology, histology to rule out other diagnoses, and post-biopsy bacteremia (brucella spp. ), and the final diagnosis was hepatic brucellosis.

The patient was treated with doxycycline (200 mg/day) and gentamicin (3 mg/kg/d), fever and Abdominal pain symptoms subsided. After 7 days of intravenous therapy, gentamicin was replaced by rifampicin (900 mg/day), and the combination of rifampicin and doxycycline was continued for 6 months. At the most recent follow-up, 9 months after antibiotic treatment, the hallmark of disease evolution was the development of refractory ascites due to Budd-Chiari syndrome.

This case was presented at a multidisciplinary expert meeting and was indicated for hepatic vein angioplasty. After antibiotic therapy and endovascular therapy for portal hypertension, surgical resection of the liver mass should be considered. Hepatic brucellosis is a rare manifestation (less than 2%) of chronic infection with Brucella.

Knowledge Class

brucellosis (brucellosis) is one of the most widespread zoonotic diseases in the world. But because of its atypical clinical symptoms, it has become one of the seven diseases that are most easily overlooked. At present, 12 species of Brucella have been reported, among which the most common Brucella species that cause human infection include sheep, cattle and pigs, among which Brucella is the most virulent.

Common Diagnostic Methods

1. General laboratory examinations1. Blood picture: the white blood cell count is usually normal or low, the lymphocytes are relatively increased, and sometimes abnormal lymphocytes may appear, and a few cases of red blood cells , thrombocytopenia. 2. ESR: The ESR may be accelerated in the acute phase, and mostly normal in the chronic phase.

II. Immunological examination1. Plate agglutination test: tiger red plate (RBPT) or plate agglutination test ( PAT) results were positive and were used for primary screening. 2. Test tube agglutination test (SAT): the titer is 1:100++ and above or the titer is 1:50++ and above for the disease course for more than one year; or there is a Brucella vaccine within half a year Vaccination history with a titer of 1:100++ and above. 3. Complement fixation test (CFT): titer 1:10++ and above. 4. Brucellosis anti-human immunoglobulin test (Coomb’s): titer 1:400++ and above.

III. Pathological examination

blood, bone marrow, synovial fluid, Brucella was isolated from cerebrospinal fluid, urine, lymphoid tissue and other cultures. The positive rates of blood, bone marrow and synovial fluid were higher in the acute phase, and lower in the chronic phase.

Therapies

The principle of treatment is Early, combined, sufficient, and full course of medication, and if necessary, prolong the course of treatment to prevent recurrence and chronicity.

Tetracyclines and rifamycins are commonly used, and quinolones, sulfonamides, aminoglycosides and third-generation cephalosporins can also be used. During the course of treatment, pay attention to monitoring blood routine, liver and kidney function, etc.

References:

< p>1.Le Cosquer G, et al. An Unusual Case of Budd–Chiari Syndrome: Diagnosis and Treatment.Gastroenterology. 2022.

2. Liu Jia, Jiang Hai. Application and thinking of diagnostic methods for brucellosis in China. Chinese Journal of Epidemiology, 2021, 42(01).

3. Diagnosis and treatment of brucellosis Guidelines (Ministry of Health 2012 Trial Version)