Peliosis hepatis is a disease that occurs very rarely. So-called lacunae, which occur in various organs of the human body, are typical of peliosis hepatis. In peliosis hepatis, the lacunae are filled with blood and develop a cystic effect. In most cases, the lacunae of peliosis hepatis develop in the liver area.
What is peliosis hepatis?
Peliosis hepatis affects the liver in the majority of cases, from which the name of the disease is derived. Peliosis hepatis is characterized by the development of cysts that are filled with blood. Doctors call this special form of cysts lacunae. They form as a result of the death of liver tissue. In other cases, a central vein in the lobule of the liver bulges, forming the blood-filled cysts of peliosis hepatis. See foodanddrinkjournal for Hypertrophic Cardiomyopathy Dictionary Definitions.
In addition to humans, peliosis hepatis also affects certain types of animals, such as dogs and cats, rats and cattle. The trigger of peliosis hepatis in humans is found in germs of the Bartonella henselea variety. These are the pathogens of cat scratch disease.
In humans, infection with these pathogens usually leads to the development of the typical symptoms of peliosis hepatis. In addition, in some cases, peliosis hepatis develops as a result of tuberculosis. Treatment with certain medical agents, such as androgens or anabolic steroids, also partially promotes the development of peliosis hepatis.
The lacunae of Peliosis hepatis are relatively small in size, no more than three millimeters. In addition, they usually have no surrounding layer that delimits the cells. Instead, so-called hepatocytes enclose the cysts.
Causes
The triggers and the exact processes of the pathogenesis of peliosis hepatis have not yet been conclusively researched. At present, only a few factors favoring the development of the disease have been identified. On the one hand, an infection with Bartonella henselae often leads to the affected persons contracting peliosis hepatis.
On the other hand, peliosis hepatis sometimes develops in connection with tumors or tuberculosis. In addition, certain medications lead to the development of peliosis hepatis. This is an undesirable side effect. Oral contraceptives and anabolic steroids pose a particular risk. In some patients, malignant blood diseases, AIDS and alcohol dependence also promote the development of peliosis hepatis.
The cause of peliosis hepatis is believed to be damage to the sinusoidal cells. Basically, peliosis hepatis often occurs in connection with the administration of hormones. The medicinal substances vinyl chloride, tamoxifen and vitamin A also increase the risk of developing peliosis hepatis. Finally, some people develop peliosis hepatis after a kidney transplant.
Symptoms, Ailments & Signs
Peliosis hepatis is characterized by characteristic cysts in the area of the internal organs. The lacunae, which are only a few millimeters in size, typically develop in the liver. In the majority of cases, peliosis hepatis is largely asymptomatic, so that the patients hardly feel any symptoms and sometimes do not even notice the disease.
However, in some people, the blood-filled cysts of peliosis hepatis rupture. As a result, intraperitoneal bleeding develops, which is a serious complication. Thus, peliosis hepatis ends fatally for some of the patients due to the bleeding. In addition, some of the people affected develop manifest diseases of the liver as a result of peliosis hepatis, which manifest themselves, for example, in hepatomegaly or jaundice. Sometimes the liver fails to function completely.
Diagnosis & course of disease
In some cases, peliosis hepatis goes unnoticed because there are hardly any symptoms. Significant symptoms sometimes only arise when the cysts burst and the subsequent bleeding occurs. In addition, often only manifest diseases of the liver indicate peliosis hepatis.
The diagnosis of peliosis hepatis is made by a specialist who, after the patient consultation, primarily uses imaging methods of the examination. The doctor uses, for example, a CT scan and ultrasonic methods to visualize the liver. A sonographic examination of the organ is also possible. Here, the typical masses of peliosis hepatis appear.
Sometimes patients are given contrast media before the examination to better differentiate the blood-filled cysts from the surrounding tissue. In addition, the doctor always examines the liver values when diagnosing peliosis hepatis, so that conclusions can be drawn about the function of the organ. In the differential diagnosis, the specialist primarily considers cavernous hemangioma and venous occlusive liver disease.
Complications
Patients with peliosis hepatis experience a number of different symptoms. As a rule, the further course and the complications of this disease depend heavily on the affected organs, so that a general prediction cannot usually be made. The patients primarily suffer from cysts that develop on the internal organs.
The liver in particular can be affected, so that in the worst case it can lead to liver cirrhosis. In many cases, the disease is only diagnosed late, when the cyst has already ruptured, so that early treatment is usually not possible. The bleeding from the cysts can be fatal for those affected. The patients themselves with this disease also suffer from jaundice and further from complete failure of the liver.
Life expectancy is significantly reduced if peliosis hepatis is not treated. The cysts can be removed with the help of surgical procedures. However, the person affected may need to undergo various interventions if not all cysts can be removed. There are no particular complications. A healthy lifestyle has a very positive effect on peliosis hepatis.
When should you go to the doctor?
Peliosis hepatis should be clarified by a doctor if pain or swelling occurs in the liver area. If there are problems with urination, medical advice is also required. Peliosis hepatis is a serious disease, but it can be treated effectively if the drug that caused it is stopped or if an infection is detected early. If the symptoms mentioned occur after contact with a possibly infected animal, peliosis hepatis may be present.
Tuberculosis and therapy with antibiotics or androgens are also associated with liver disease. Patients at risk should see their family doctor if signs of a secondary disease appear. Peliosis hepatis is treated by the family doctor or a specialist in liver diseases. A serious illness requires inpatient treatment. After initial therapy, the physician should be informed of any complications and symptoms that indicate a return of peliosis hepatis. Side effects of the prescribed medication must also be clarified and treated quickly.
Treatment & Therapy
With peliosis hepatis, there is always a risk that the blood-filled cysts will rupture and cause life-threatening complications due to bleeding in the abdomen. For this reason, the diseased areas are usually removed as part of surgical interventions.
Outlook & Forecast
A uniform prognosis cannot be given for the liver with blood-filled cysts known as peliosis hepatis. The reason lies in the different triggers that can cause this disease. It can also be an undiagnosed liver cancer. A careful clarification of the cysts and their cause is therefore indicated.
Doctors also distinguish between three different forms of peliosis hepatis. The prognosis of the parenchymatous form may differ from the prognosis of the phlebectatic form. In addition, a bacillary peliosis hepatis is known, which occurs in humans and some animal species related to humans. Those affected are often infected with cat scratch disease by corresponding pathogens.
Peliosis hepatis sufferers are often patients who have to take immunosuppressants before or after a transplant. Peliosis hepatis also often affects patients taking anabolic steroids, oral contraceptives or androgens. Some sufferers suffer from Hodgkin’s disease or tuberculosis.
If such basic problems are present, this worsens the prognosis. However, peliosis hepatis is relatively rare. Its course is usually asymptomatic. However, complications can arise. A life-threatening complication would be cyst rupture. The blood from the ruptured cyst spills into the abdomen. Consecutive intra-abdominal bleeding occurs, which requires immediate surgical measures.
Prevention
Targeted prevention of peliosis hepatis is hardly possible. The corresponding risk factors of peliosis hepatis, such as taking certain medications, should be avoided if possible. If intake is urgently required, regular medical check-ups are recommended. Any alcohol abuse should also be stopped as soon as possible.
Aftercare
In most cases, those affected with peliosis hepatis have only a few measures or options for aftercare. First and foremost, an early diagnosis of the disease should be made so that other complications or a further deterioration of the symptoms do not occur. In the worst case, the affected person dies as a result of peliosis hepatis if it is completely ignored and not treated.
Those affected are dependent on a surgical procedure for this disease, which can relieve the symptoms permanently. The procedure should be carried out as soon as possible after the diagnosis, so that it does not burst open and lead to bleeding. After such a procedure, the patient should definitely rest and take care of his body. Strict bed rest should be observed, with exertion and physical activity being avoided.
Regular checks and examinations by a doctor are also very important for peliosis hepatis, as this is the only way to permanently monitor the condition of the liver. Alcohol should also be avoided in peliosis hepatis, although a healthy lifestyle with a balanced diet generally has a positive effect on the further course of the disease.
You can do that yourself
If peliosis hepatis has been diagnosed, the triggering drug must first be discontinued. If the condition is due to a serious illness such as AIDS or cancer, this should be treated in addition to the therapy for peliosis hepatis. If an alcohol or drug problem is the cause, treatment must be initiated.
The people affected are usually very weak physically and have to take it easy. Diet usually has to be changed to relieve the liver and improve overall well-being. It is best for those affected to contact a nutritionist in order to work out a suitable diet together with them. General measures such as sport and employment are recommended as support. Since the condition is often associated with considerable pain, pain therapy may also have to be carried out. In individual cases, an inpatient stay in the hospital is necessary.
Persons suffering from peliosis hepatis should take care of the necessary organizational tasks at an early stage so that they can be treated quickly in the event of a deterioration in their state of health. After the hospital stay, rest and rest apply again. In addition, a complaints diary should be kept so that the symptoms can be precisely defined and treated in a targeted manner. If the symptoms increase despite all measures, further investigations are necessary.