hepatic veno-occlusive disease

Hepatic Veno-Occlusive Disease (VOD): A Rare and Serious Liver Condition

Hepatic veno-occlusive disease, also known as sinusoidal obstruction syndrome (SOS), is a rare and serious liver condition characterized by the blockage of small blood vessels in the liver. This leads to toxic injury to the hepatic sinusoidal capillaries, causing obstruction of the small hepatic veins [1].

Causes and Risk Factors

VOD can occur following stem-cell transplant, chemotherapy, and other treatments that affect the liver and immune system. It may also be a hereditary disorder, known as hepatic veno-occlusive disease with immunodeficiency (VODI) [6]. The exact cause of VOD is not fully understood, but it is believed to involve damage to the small blood vessels in the liver.

Symptoms and Complications

The symptoms of VOD can include:

• Hepatomegaly (enlarged liver)
• Ascites (fluid accumulation in the abdomen)
• Weight gain
• Jaundice (yellowing of the skin and eyes)

If left untreated, VOD can lead to serious complications, including liver failure, bleeding, and even death [3].

Prevention and Treatment

While there is no specific treatment for VOD, early detection and management are crucial in preventing further damage to the liver. Treatment may involve supportive care, such as fluid replacement and medications to manage symptoms [4]. In some cases, liver transplantation may be necessary.

Incidence and Prognosis

VOD is a rare condition, affecting up to 15% of adults after hematopoietic cell transplantation [9]. The prognosis for VOD patients varies depending on the severity of the disease and the effectiveness of treatment. With prompt and proper care, some patients may recover fully, while others may experience long-term liver damage or other complications.

References:

[1] A rare vascular liver disease characterized by toxic injury to the hepatic sinusoidal capillaries that leads to obstruction of the small hepatic veins and ...

[3] Hepatic veno-occlusive disease is a clinical syndrome characterized by hepatomegaly, ascites, weight gain and jaundice, due to sinusoidal ...

[4] Hepatic veno-occlusive disease (VOD) is a complication that affects the liver and can occur following stem-cell transplant. VOD may also be referred to as ...

[6] Hepatic veno-occlusive disease with immunodeficiency (also called VODI) is a hereditary disorder of the liver and immune system.

[9] Hepatic SOS, which is also called veno-occlusive disease (VOD), develops in up to 15 percent of adults after hematopoietic cell transplantation ...

Common Signs and Symptoms of Hepatic Veno-Occlusive Disease

Hepatic veno-occlusive disease (VOD) is a clinical syndrome characterized by several distinct signs and symptoms. These can vary in severity and may include:

• Weight gain: Due to fluid retention, which can manifest as edemas, ascites, and an increase in abdominal circumference [9].
• Painful hepatomegaly: Enlargement of the liver, often accompanied by tenderness, especially on the upper right side [1].
• Jaundice: Yellowing of the skin and whites of the eyes due to a buildup of bilirubin in the blood [5].
• Ascites: Fluid accumulation in the abdominal cavity, leading to swelling and discomfort [2, 3, 7].
• Hepatomegaly: Enlargement of the liver, which can be tender to the touch [1, 4, 7].
• Hyperbilirubinemia: Elevated levels of bilirubin in the blood, contributing to jaundice [9].
• Thrombocytopenia: Low platelet count, which can increase the risk of bleeding [9].

These symptoms can be indicative of hepatic veno-occlusive disease and should prompt further medical evaluation.

References: [1] General signs and symptoms of veno-occlusive disease (VOD) include: Pain and tenderness in the abdomen, especially on the upper right side, due to swelling of ... [2] Clinical manifestations include painful hepatomegaly, jaundice, and fluid retention that manifests by weight gain, edemas, and ascites. ORPHA:890. [3] by M Senzolo · 2007 · Cited by 105 — Hepatic veno-occlusive disease is a clinical syndrome characterized by hepatomegaly, ascites, weight gain and jaundice, due to sinusoidal congestion. [4] Jan 1, 2009 — This condition can lead to enlargement of the liver (hepatomegaly), a buildup of scar tissue (hepatic fibrosis), and liver failure. [5] Signs and symptoms include weight gain, yellowing of the skin and whites of the eyes, dark-colored urine, and increased liver size. [6] Symptoms and Signs of Sinusoidal Obstruction Syndrome​​ Other patients have recurrent ascites, portal hypertension, splenomegaly, and, eventually, cirrhosis. [7] Signs and symptoms​​ Features of hepatic veno-occlusive disease include weight gain, tender enlargement of the liver, ascites, and yellow discoloration of the ... [8] by S Kumar · 2003 · Cited by 254 — Other signs such as rash and diarrhea are often present in GVHD. Confirmation of the diagnosis of GVHD by skin or other tissue biopsy can help rule out VOD; ... [9] Apr 26, 2024 — Weight gain · Increase in abdominal circumference · Hepatomegaly · Right upper quadrant pain · Ascites · Hyperbilirubinemia · Thrombocytopenia.

Hepatic veno-occlusive disease (VOD) is a clinical syndrome characterized by hepatomegaly, ascites, weight gain and jaundice, due to sinusoidal congestion [2]. Diagnostic tests for VOD/SOS include:

• Imaging studies: These may help confirm the diagnosis of VOD/SOS. Specifically:
  • Abdominal ultrasound (hepatomegaly and/or ascites) [4]
  • Doppler ultrasound imaging (reversal of portal venous flow) [4]
  • CT (computed tomography) scan or X-ray to rule out other causes of liver enlargement [3]
• Liver biopsy: This is often indicated, especially in cases where the diagnosis is unclear. Recent data support its indication and can help confirm the diagnosis of VOD/SOS [5].
• Blood work: In addition to imaging studies, blood tests may be conducted to rule out other causes of liver dysfunction.
• Ultrasound with Doppler evaluation: This has been studied extensively in the context of VOD/SOS, with numerous findings suggestive of the condition. However, its accuracy and validation are still being researched [7].

It's worth noting that the incidence, risk factors, clinical presentation, evaluation, and diagnosis of hepatic SOS differ between adults and children [8]. A baseline abdominal ultrasound examination before conditioning that includes gray-scale, color Doppler, spectral Doppler, and elastography evaluations would be useful in identifying patients at high risk for VOD/SOS [6].

References: [2] M Senzolo · 2007 · Cited by 105 [3] [4] [5] [6] by SS Chan · 2020 · Cited by 36 [7] by F Ravaioli · 2019 · Cited by 49 [8] Feb 22, 2024

Treatment Options for Hepatic Veno-Occlusive Disease (VOD)

Hepatic VOD, also known as sinusoidal obstruction syndrome (SOS), is a serious complication that can occur after hematopoietic stem cell transplantation (HSCT). While there are various treatment options available, the primary goal of therapy is to prevent or manage this condition and improve patient outcomes.

Approved Medications

• Defibrotide: This is the only medication approved by the US Food and Drug Administration for the management of severe VOD/SOS after HSCT. Defibrotide has been shown to improve survival rates in patients with hepatic VOD/SOS, with a 23% improvement in survival at day +100 post-HSCT [1][6].
• Ursodeoxycholic acid: This medication is used as a preventive treatment for VOD, starting from the beginning of conditioning until 90 days post-transplantation. It has been shown to significantly reduce the proportion of patients developing VOD/SOS [4].

Other Investigated Treatments

• Tissue plasminogen activator (tPA): This medication has been investigated in clinical studies as a potential treatment for VOD/SOS, although its efficacy is still being researched [3].
• Methylprednisolone: This corticosteroid has also been studied as a possible treatment option for VOD/SOS, but more research is needed to confirm its effectiveness.
• N-acetylcysteine: This antioxidant has been investigated in clinical studies as a potential treatment for VOD/SOS, although its efficacy is still being researched.

Key Takeaways

• Defibrotide is the only FDA-approved medication for treating severe VOD/SOS after HSCT.
• Ursodeoxycholic acid can be used as a preventive treatment to reduce the risk of developing VOD/SOS.
• Other medications, such as tPA, methylprednisolone, and N-acetylcysteine, are being investigated as potential treatments for VOD/SOS.

References:

[1] M Nauffal (2022) - Defibrotide is approved by the US FDA for managing severe VOD/SOS after HSCT. [2] Apr 26, 2024 - The US FDA has approved defibrotide for treating adult and pediatric patients with hepatic VOD. [3] PG Richardson (2017) - Medical treatments for VOD/SOS have been investigated, including tPA, methylprednisolone, and N-acetylcysteine. [4] M Mohty (2020) - Ursodeoxycholic acid has been shown to reduce the proportion of patients developing VOD/SOS when administered continuously until 90 days post-transplantation. [5] Apr 26, 2024 - Defibrotide is approved by the US FDA for treating veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS). [6] S Corbacioglu (2016) - Defibrotide has demonstrated efficacy and safety in treating VOD/SOS.

Hepatic veno-occlusive disease (VOD), also known as sinusoidal obstruction syndrome, has a differential diagnosis that includes several conditions that can present with similar clinical features.

Conditions to Consider:

• Antithrombin III Deficiency: A rare genetic disorder that affects the production of antithrombin, a protein that helps regulate blood clotting. This condition can lead to venous thrombosis and VOD (3).
• Budd-Chiari Syndrome: A rare condition characterized by the obstruction of the hepatic veins, which can cause liver enlargement, ascites, and jaundice (3).
• Cholestasis: A condition where bile flow is reduced or blocked, leading to a buildup of bile in the liver and potentially causing VOD-like symptoms (3).
• Gastrointestinal Bleeding: While not directly related to VOD, gastrointestinal bleeding can be a complication of certain conditions that may also present with VOD-like symptoms (3).
• Hyperacute Hepatic Graft-Versus-Host Disease (GVHD): A rare but serious condition that occurs when the immune system attacks the liver after a transplant. It can present with similar clinical features to VOD, making differential diagnosis critical (9).

Diagnostic Tools:

• Abdominal Ultrasound: Can assist in the differential diagnosis of clinically suspected VOD/SOS by providing baseline and serial measurements (4).
• Liver Biopsy: The definitive method of diagnosis for VOD, as it allows for histological examination of liver tissue (6).

It's essential to consider these conditions and use diagnostic tools like abdominal ultrasound and liver biopsy to accurately diagnose hepatic veno-occlusive disease. [1][2][3][4][5][6][7][8][9]