In hepatic vein occlusive disease, also called sinusoidal obstruction syndrome, blood backs up in the hepatic veins. Symptoms of the disease include pain in the right upper abdomen, abdominal dropsy, and yellowing of the skin.
What is hepatic vein occlusive disease?
In hepatic vein occlusive disease, damage occurs within the liver to tiny blood vessels called hepatic sinusoids. Because of this disorder, blood can no longer circulate properly within these small venous vessels. The result is venous outflow obstruction. Depending on the degree of congestion, the central veins may be involved. Blood stasis in the liver leads to destruction of liver tissue and, consequently, impaired liver function.
Causes
In most cases, hepatic vein occlusive disease occurs after treatment with chemotherapeutic agents. Presumably, the disease is caused by the combination of high doses of various cytostatic drugs. In particular, therapy with the cytostatic drugs busulfan, cyclophosphamide, actinomycin D, and thioguanine increases the risk of developing hepatic vein occlusive disease. The combination of cytostatic drugs and total body irradiation for cancer is also a risk factor. In many cases, the disease occurs in conjunction with preparation for hematopoietic stem cell transplantation. Such transplants are performed to treat leukemias. The disease can also be caused by the consumption of certain teas. Tea mixtures with plants that have a high content of pyrrolizidine alkaloids are particularly suspected in this regard. Pyrrolizidine alkaloids are present in fluctuating amounts in, for example, borage, water astragalus, coltsfoot or comfrey. If the harvest of other tea plants or of cereals is contaminated with such plants, epidemics of liver closure disease may occur. However, these are extremely rare, especially in Western countries, and are more likely to be found in developing countries.
Symptoms, complaints, and signs
Occlusion of the small and possibly the large hepatic veins causes blood congestion within the liver. Consequently, the liver swells. This is called hepatomegaly. The swelling causes stretching of the liver capsule, as a result of which the affected person feels mild to moderate pressure in the right upper abdomen. Within the liver, tiny bile ducts run. These merge into larger bile ducts while still inside the liver and eventually flow into two large bile ducts, which merge into one large bile duct outside the liver. The congestion of blood in the liver compresses the bile ducts and the bile canaliculi, so that in addition to the congestion of blood, there is also bile stasis, a condition called intrahepatic cholestasis. The bile contains the yellowish pigment bilirubin, a breakdown product of the red blood pigment hemoglobin. Due to bile stasis, some of this bile pigment leaks into the bloodstream. Above a certain concentration of bilirubin in the blood, the skin turns yellow. The yellowing of the skin as a result of the increased concentration of bilirubin in the blood is also known as icterus. Icterus shows up particularly early in the sclera of the eyes. The longer the bile stasis persists, the more yellow the skin appears. Hepatic veno-occlusive disease is also noticeable by weight gain. Due to the congestion of blood drainage, fluid leaks from the blocked blood vessels into the abdomen. This fluid accumulates in the abdominal cavity. Patients gain weight and their abdominal girth increases. There may be bloating and a noticeable protrusion of the abdomen. As a result of this abdominal dropsy (ascites), hepatorenal syndrome may occur as a complication. The exact mechanism of the disease is still unclear. What is certain, however, is that the renal vessels contract, making it impossible for the kidneys to maintain their filtering function. The result is renal insufficiency with kidney failure.
Diagnosis and course of the disease
Initial indications of hepatic vein occlusive disease are provided by symptoms typical of the liver, such as jaundice, abdominal dropsy, pain in the right upper abdomen, and weight gain. However, the symptoms may vary in severity.If this symptom complex occurs in conjunction with chemotherapy or radiation therapy or during preparation for stem cell transplantation, the diagnosis of hepatic vein occlusive disease is very likely. Nevertheless, exclusion of other causes is necessary. To do this, an ultrasound examination of the liver and other abdominal organs is first performed. The diagnosis can also be made by other imaging techniques such as computed tomography or magnetic resonance imaging. Doppler ultrasonography reveals an attenuation of blood flow in the hepatic veins. At the same time, a reversal of the so-called portal vein flow is visible. In this case, the blood in the portal vein no longer flows toward the liver but toward the systemic circulation. Normally, the portal vein receives blood from the unpaired abdominal organs and delivers it to the liver for purification and metabolism. In many cases, a liver biopsy is also required to make a diagnosis. Examination of the tissue sample reveals subsidence of cell clusters. Blood congestion in the hepatic veins can also be detected in this way. If the liver biopsy is performed via the jugular vein, the hepatic vein occlusion pressure can also be measured at the same time. If this pressure is above 10mmHG, this clearly indicates hepatic vein occlusion disease.
Complications
Hepatic vein occlusive disease is a serious condition and therefore must be treated by a physician in any case. Otherwise, in the worst case, this can also lead to the death of the affected person if no treatment of this disease is initiated. The affected person usually suffers from a pressure in the abdomen. They also experience severe pain, which can spread to other regions of the body. Bile stasis also usually occurs due to hepatic vein occlusion disease and significantly reduces the patient’s quality of life. In most cases, patients also gain weight due to the disease, although this gain is unexplained. Bloating and diarrhea may also occur due to this disease, making daily life difficult. Furthermore, without treatment, hepatic vein occlusion disease also leads to renal insufficiency, which can result in death. Those affected are then dependent on a donor kidney or dialysis. As a rule, hepatic vein occlusive disease cannot be treated. In most cases, those affected die. Only the symptoms can be limited. In many cases, the relatives are also affected by psychological discomfort and need psychological treatment.
When should one go to the doctor?
When swelling of the liver is noticed, a doctor should be consulted. Signs of hepatomegaly indicate hepatic vein occlusion disease, which should always be evaluated and treated by a doctor. Pain and feelings of pressure in the right upper abdomen, signs of bile stasis, and yellowing of the skin are also warning signs that indicate serious liver disease. Anyone who notices these symptoms should seek medical advice immediately. A visit to the doctor is necessary at the latest when digestive complaints are added or the quality of life decreases sharply as a result of the aforementioned complaints. Hepatic vein occlusion disease occurs mainly after radiation or chemotherapy. People who have recently undergone hematopoietic stem cell transplantation are also at risk and should present the described symptoms to a physician immediately. In addition to the primary care physician, the hepatologist or an internist may be consulted. In the event of a medical emergency, first responders must alert the emergency medical services. Subsequently, treatment and follow-up in the hospital is necessary in any case.
Treatment and therapy
No effective drug treatment is currently available for hepatic vein occlusive disease. In severe cases, the prognosis is rather unfavorable. Thus, up to 90 percent of patients then die.
Outlook and prognosis
If left untreated, hepatic vein occlusion disease is fatal. The congestion of blood in the human organism leads to impaired functioning of organs. Ultimately, there is a failure of organ activity and thus a premature death of the affected person. The later the diagnosis is made and the later the treatment, the less favorable the further course of the disease.Only with rapid and comprehensive medical care can the chances of survival of the affected person be increased. Currently, the mortality rate is over 90 percent of patients. Medical care is made more difficult by the fact that there is as yet no adequate treatment option for hepatic vein occlusion disease. Therefore, timely avoidance of liver toxic drugs is necessary for a good prognosis. Preventive measures are already an important element for recovery. The available treatment methods serve to reduce the symptoms and are also associated with various risks and side effects. Since a variety of complications develop during treatment, secondary diseases are often detectable. To improve their own health, patients must take comprehensive action themselves and refrain from ingesting harmful substances. Environments filled with nicotine or other toxic gases, as well as smoking or the consumption of alcohol, significantly worsen the prognosis. They elementarily shorten the expected life span.
Prevention
The best prevention is to avoid liver-toxic drug combinations before stem cell transplantation. Care should also be taken when combining different cytostatic drugs or combining cytostatic drugs and radiation treatment. To prevent blood stasis in the liver, heparin can be used as a preventive measure. Ursodeoxycholic acid is also frequently administered as a drug prophylaxis.
Follow-up
In most cases, follow-up care for hepatic vein occlusive disease proves relatively difficult or is not even available to the affected person. In this case, a physician must be consulted at a very early stage to prevent further compilations or other symptoms. Therefore, those affected with hepatic vein occlusive disease should ideally see a doctor at the first signs and symptoms of the disease. As a rule, self-healing is not possible. Direct treatment is usually not possible in this case. Those affected are primarily dependent on taking various medications that can alleviate the symptoms. However, a purely causal treatment is not available to those affected by hepatic vein occlusion disease. Patients are dependent on the help and support of family and friends in their daily lives. The support of the closest relatives in particular has a positive effect on the course of the disease, and depression or other psychological upsets can also be prevented as a result. It is also necessary to follow all the instructions of the doctor regarding the use of medications to relieve the symptoms well. However, hepatic vein occlusion disease usually always drastically reduces the life expectancy of the affected person.
This is what you can do yourself
Patients with hepatic vein occlusive disease suffer from a serious condition as well as a high risk of death. The options for self-help are relatively limited for hepatic vein occlusive disease and only help to improve the general health condition. Therefore, it is first important for patients with hepatic vein occlusive disease to undergo regular medical examinations to register changes in their physical condition. The basic problem with hepatic vein occlusive disease is that no suitable drug therapy exists to date. Therefore, the patients’ influence is limited to supporting the organism, including the immune system, through a lifestyle that is as healthy as possible. For example, it is advisable for people with hepatic vein occlusive disease to contact a medical nutritionist and jointly draw up a diet plan tailored to the disease. It is central to minimize the intake of liver-toxic substances, for example, through food. The same applies to medications, some of which pose a risk to liver health. For this reason, medications should always be prescribed with hepatic vein occlusive disease in mind. For patients with hepatic vein occlusive disease, it is of course essential to abstain from alcohol in order to avoid further stressing the liver and to influence the course of the disease as positively as possible. In addition, those suffering from the disease should also stop smoking if possible.
