BCS

Question 1

Definition

Answer 1

a clinical condition resulting from the obstruction of the hepatic veins and/or the inferior vena cava (IVC) that drains the liver. This obstruction may be due to thrombosis, stenosis, or compression

Question 2

Classification

Answer 2

1. Based on Etiology:

*Primary BCS: Caused by a primary thrombotic process within the hepatic veins or IVC, often associated with prothrombotic conditions such as:
-Polycythemia vera
-Antiphospholipid syndrome
-Factor V Leiden mutation
-Protein C/S deficiency
*Secondary BCS: Caused by external compression or invasion of hepatic veins or IVC due to:
-Tumors (e.g., hepatocellular carcinoma)
-Hydatid cysts
-Abscesses
-Congenital webs or membranes in IVC

Question 3

Classification

Answer 3

2. Based on Site of Obstruction:

*Hepatic vein thrombosis (classical BCS)

*Inferior vena cava obstruction

*Mixed type (both hepatic vein and IVC involvement)

Question 4

Classification

Answer 4

3. Based on Clinical Presentation:

*Fulminant BCS: Acute liver failure with encephalopathy and jaundice.

*Acute BCS: Rapid onset with abdominal pain, hepatomegaly, and ascites.

*Subacute BCS: Gradual onset with features developing over weeks or months.

*Chronic BCS: Long-standing disease with signs of portal hypertension and cirrhosis.

Question 5

A 56-year-old woman with myelofibrosis and agnogenic myeloid metaplasia underwent
allogeneic hematopoietic cell transplantation after a conditioning regimen of oral busulfan
(16 mg/kg with therapeutic drug monitoring), followed by intravenous cyclophosphamide
(120 mg/kg) and prophylactic ursodeoxycholic acid therapy. One week later, she became
jaundiced and had mild hepatic tenderness. She had gained approximately 10% of her initial
body weight. Liver enzyme levels did not change, and serum creatinine was stable (0.7 to 1.0
mg/dL).

Answer 5

A) What is the most likely diagnosis?
VOD
B) How is this disease diagnosed and how could it be prevented?

Question 6

VOD (Veno occlusive disese)

Answer 6

Volume gain (weight gain)
Obstruction
Drugs (bisphosphonate , cyclophosphamide)

Question 7

VOD….
Diagnosis

Answer 7

1-Baltimore Criteria:

Total bilirubin ≥ 2 mg/dL plus at least two of the following:
HAG هاج

Hepatomegaly or right upper quadrant pain

Ascites

Weight gain >5% of baseline

Question 8

VOD….
Diagnosis

Answer 8

Modified Seattle Criteria:

At least two of the following within 20 days post-transplant:

Bilirubin ≥ 2 mg/dL

Hepatomegaly or right upper quadrant pain

Weight gain >2% of baseline

Question 9

VOD
Prevention

Answer 9

1-Prophylactic ursodeoxycholic acid (as used in this case) can reduce incidence but does not eliminate risk.

2-Defibrotide is approved for both prevention in high-risk patients and treatment of VOD.

3-Therapeutic drug monitoring of busulfan (as was done here) helps reduce risk by maintaining levels within a safe range.

4-Avoid additional hepatotoxic medications when possible.

Question 10

Treatment of BCS

Answer 10

General Measures

1-Anticoagulation: First-line treatment in most cases to prevent thrombosis (e.g., warfarin, heparin).
2-Diuretics and sodium restriction: For managing ascites.
3-Management of underlying causes: Such as myeloproliferative disorders, infections, or malignancies.
4- Endovascular Interventions
Angioplasty with or without stenting: For short-segment venous stenosis.
5-Thrombolysis: In acute cases with fresh thrombus.
6-Transjugular Intrahepatic Portosystemic Shunt (TIPS)
Indicated if medical therapy and angioplasty are ineffective.
Decompresses hepatic congestion and controls portal hypertension.

7- Liver Transplantation

For patients with fulminant liver failure, decompensated cirrhosis, or when all other treatments fail.