HVD stands for hepatic veno-occlusive disease. In hepatic veno-occlusive disease, also known as sinusoidal obstruction syndrome, blood builds up in the veins of the liver. Symptoms of the disease include pain in the upper right abdomen, ascites, and yellowing of the skin.
What is hepatic veno-occlusive disease?
A blockage causes blood to congest within the liver. See AbbreviationFinder for abbreviations related to HVD.
In hepatic veno -occlusive disease, the smallest blood vessels in the liver, the so-called hepatic sinusoids, are damaged. Because of this disorder, the blood can no longer circulate properly within these small venous vessels. The result is a venous drainage disorder. Depending on the degree of congestion, the central veins may be involved. The blood congestion in the liver leads to the destruction of liver tissue and thus to impaired liver function.
In most cases, hepatic veno-occlusive disease occurs after treatment with chemotherapy drugs. Presumably, the disease is caused by the combination of high doses of different cytostatic drugs. In particular, therapy with the cytostatics busulfan, cyclophosphamide, actinomycin D and thioguanine increases the risk of contracting hepatic veno-occlusive disease. The combination of cytostatics and whole-body radiation in cancer is also a risk factor.
In many cases, the disease occurs in connection with the preparation for a transplantation with hematopoietic stem cells. Such transplants are performed to treat leukemias. The condition can also be caused by the consumption of certain teas. Above all, tea mixtures with plants that have a high proportion of pyrrolizidine alkaloids are suspected.
Pyrrolizidine alkaloids are found in varying amounts in borage, coltsfoot, coltsfoot and comfrey, for example. If crops of other tea plants or grain are contaminated with such plants, epidemics of hepatic occlusive disease can occur. However, these are extremely rare, especially in western countries, and are more likely to be found in developing countries.
Symptoms, Ailments & Signs
The occlusion of the small and possibly also the large hepatic veins causes blood to congest within the liver. As a result, the liver swells. This is referred to as hepatomegaly. The swelling causes the liver capsule to stretch, causing those affected to feel mild to moderate pressure in the upper right abdomen.
Tiny bile tubules run inside the liver. While still inside the liver, these join together to form larger bile ducts and finally open into two large bile ducts, which unite outside the liver to form one large bile duct. The bile canaliculi and the bile ducts are compressed by the blood congestion in the liver, so that in addition to the blood congestion there is also bile congestion, a so-called intrahepatic cholestasis.
The bile contains the yellow pigment bilirubin, a breakdown product of the red blood pigment hemoglobin. Due to bile stagnation, parts of this bile pigment enter 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 jaundice. The icterus appears particularly early in the sclera of the eyes. The longer the bile build-up, the yellower the skin appears.
Hepatic veno-occlusive disease is also noticeable through weight gain . Due to the congestion of the blood flow, fluid escapes from the congested blood vessels into the abdominal cavity. This fluid collects in the abdominal cavity. Patients gain weight and their waist circumference increases. There may be flatulence and a noticeable bulging of the abdomen.
As a result of this abdominal dropsy (ascites), the hepatorenal syndrome can occur as a complication. The exact mechanism of the disease is still unclear. What is certain, however, is that the renal vessels contract, which means that the kidneys can no longer maintain their filtering function. The result is renal insufficiency with renal failure.
Diagnosis & course of disease
Symptoms typical of the liver, such as jaundice, ascites, pain in the upper right abdomen and weight gain, are the first indications of a hepatic veno-occlusive disease. However, the symptoms can vary in severity. If this complex of symptoms occurs in connection with chemotherapy or radiation therapy or in preparation for a stem cell transplant, the diagnosis of hepatic veno-occlusive disease is very likely.
Nevertheless, it is necessary to rule out other causes. First, an ultrasound scan of the liver and other abdominal organs is performed. The diagnosis can also be made by other imaging methods such as computed tomography or magnetic resonance imaging. Doppler sonography shows a weakening of the blood flow in the hepatic veins. At the same time, a reversal of the so-called portal vein flow is visible. The blood in the portal vein no longer flows in the direction of the liver, but in the direction of the systemic circulation.
Normally, the portal vein receives blood from the unpaired abdominal organs and carries it to the liver for purification and metabolism. In many cases, a liver biopsy is also required for diagnosis. Examination of the tissue sample reveals the loss of cell structures. Blood congestion in the liver veins can also be detected in this way. If the liver biopsy is performed via the jugular vein, the hepatic venous occlusion pressure can also be measured at the same time. If this pressure is above 10mmHG, this clearly indicates hepatic veno-occlusive disease.
Hepatic veno-occlusive disease is a serious disease and must therefore be treated by a doctor in any case. Otherwise, in the worst case, the patient may die if no treatment for this disease is initiated. Those affected usually suffer from pressure in the abdomen.
Severe pain also occurs, which can spread to other regions of the body. Bile congestion also usually occurs due to the hepatic veno-occlusive disease and significantly reduces the patient’s quality of life. In most cases, patients also gain weight as a result of the disease, although this increase is unexplained.
Flatulence and diarrhea can also occur as a result of this disease and make everyday life more difficult. Furthermore, if left untreated, hepatic veno-occlusive disease also leads to renal insufficiency, which can lead to death. Those affected are then dependent on a donor kidney or dialysis.
Usually, hepatic veno-occlusive disease cannot be treated. Those affected die in most cases. Only the complaints can be limited. In many cases, the relatives are also affected by psychological problems and need psychological treatment.
When should you go to the doctor?
If swelling of the liver is noticed, a doctor should be consulted. Signs of hepatomegaly indicate hepatic veno-occlusive disease, which must be medically clarified and treated in any case. Pain and feelings of pressure in the upper right abdomen, signs of bile stasis and yellowing of the skin are also warning signs that indicate a serious liver disease. If you notice these symptoms, it is best to see a doctor immediately.
A visit to the doctor is necessary at the latest when digestive problems occur or the quality of life decreases significantly as a result of the symptoms mentioned. Hepatic veno-occlusive disease occurs mainly after radiation or chemotherapy. People who have recently had a hematopoietic stem cell transplant are also at risk and should report the symptoms described to a doctor immediately. In addition to the family doctor, the hepatologist or an internist can be consulted. In the event of a medical emergency, first responders must alert the emergency services. Treatment and follow-up in the hospital are then necessary in any case.
Treatment & Therapy
No effective drug treatment is currently available for hepatic veno-occlusive disease. In severe cases, the prognosis is rather unfavorable. Up to 90 percent of patients die in this way.
Outlook & Forecast
If left untreated, hepatic veno-occlusive disease can be fatal. The accumulation of blood in the human organism leads to an impairment of the functionality of the organs. Ultimately, there is a loss of organ activity and thus the premature death of the person concerned. The later a diagnosis and thus treatment takes place, the more unfavorable the further course of the disease. The chances of survival for those affected only increase if they receive rapid and comprehensive medical care.
Currently, the mortality rate is over 90 percent of patients. Medical care is made more difficult because there is currently no adequate treatment option for hepatic veno-occlusive disease. Therefore, timely avoidance of liver-toxic drugs is necessary for a good prognosis. Precautionary measures are 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 large number of complications develop during treatment, secondary diseases can often be identified. In order to improve their own health, the patient must become fully active and refrain from taking in harmful substances. Environments filled with nicotine or other toxic gases, as well as smoking or consuming alcohol significantly worsen the prognosis. They shorten the expected lifetime elementarily.
The best prevention is to avoid hepatotoxic drug combinations before stem cell transplants. Caution should also be exercised with the combination of different cytostatics or the combination of cytostatics and radiation treatment. Heparin can be used preventively to prevent blood congestion in the liver. Ursodeoxycholic acid is also often administered as a prophylactic drug.
In most cases, follow-up care for hepatic veno-occlusive disease proves to be relatively difficult or is not even available to the person concerned. A doctor must be consulted very early on, so that further compilations or other complaints do not occur. Therefore, those affected by hepatic veno-occlusive disease should ideally consult a doctor at the first signs and symptoms of the disease.
As a rule, no self-healing can occur. Direct treatment is usually not possible. Those affected are primarily dependent on taking various medications that can relieve the symptoms. However, a purely causal treatment is not available to those affected with hepatic veno-occlusive disease. Patients depend on the help and support of family and friends in their everyday lives.
Above all, the support of the closest relatives has a positive effect on the course of the disease, whereby depression or other mental upsets can also be prevented. It is also necessary to follow all the doctor’s instructions regarding the taking of medication in order to relieve the symptoms well. However, hepatic veno-occlusive disease usually always drastically reduces the life expectancy of the person affected.
You can do that yourself
Patients with hepatic veno-occlusive disease suffer from serious illness and a high risk of death. The options for self-help are relatively limited in the case of hepatic veno-occlusive disease and only help to improve the general state of health.
It is therefore important that patients with hepatic veno-occlusive disease undergo regular medical examinations to register changes in their physical condition. The fundamental problem with hepatic veno-occlusive disease is that there is currently no suitable drug therapy.
Therefore, the influence of the patient is limited to supporting the organism including the body’s defenses through a lifestyle that is as healthy as possible. It is therefore advisable for people with hepatic veno-occlusive disease to contact a medical nutritionist and together draw up a nutrition plan tailored to the disease. It is crucial to minimize the intake of substances that are toxic to the liver, for example through food. The same applies to medicines, some of which pose a risk to liver health. For this reason, medicines should always be prescribed taking hepatic veno-occlusive disease into account.
For patients with hepatic veno-occlusive disease, it is of course essential to avoid alcohol in order not to further stress the liver and to influence the course of the disease as positively as possible. In addition, if possible, the sick people stop smoking.