Peliosis hepatis is a disease that occurs very rarely. Typical of peliosis hepatis are so-called lacunae, which are found in various organs of the human body. In peliosis hepatis, the lacunae are filled with blood and develop a cystic effect. In most cases, lacunae of peliosis hepatis develop in the area of the liver.
What is peliosis hepatis?
Peliosis hepatis affects the liver in the majority of cases, from which the name of the disease is derived. A characteristic feature of peliosis hepatis is the development of cysts that are filled with blood. Physicians call this particular form of cysts lacunae. They form as a result of the death of tissue in the liver. In other cases, a central vein in the liver lacuna sags, causing the blood-filled cysts of peliosis hepatis to form. In addition to humans, peliosis hepatis also affects certain types of animals, such as dogs and cats, as well as rats and cattle. In humans, the causative agent of peliosis hepatis is found in germs of the Bartonella henselea variety. These are the pathogens of cat scratch disease. Infection with these pathogens in humans usually leads to the development of the typical symptoms of peliosis hepatis. In addition, in some cases, peliosis hepatis develops as a consequence of tuberculosis. Treatment with certain medical agents, such as androgens or anabolic steroids, also sometimes promotes the development of peliosis hepatis. The lacunae of peliosis hepatis have a relatively small size of no more than three millimeters. In addition, they usually do not have a surrounding layer that delimits the cells. Instead, so-called hepatocytes surround the cysts.
Causes
The triggers as well as the exact processes of the pathogenesis of peliosis hepatis have not yet been conclusively investigated. Thus, only a few factors favoring the development of the disease have been identified at present. On the one hand, an infection with Bartonella henselae often leads to the affected individuals developing peliosis hepatis. Second, peliosis hepatis sometimes develops in association with tumors or tuberculosis. In addition, certain drugs lead to the development of peliosis hepatis. This is an undesirable side effect. Oral contraceptives and anabolic steroids pose a particular risk. In addition, in some patients, malignant blood diseases, AIDS, and alcohol dependence favor the development of peliosis hepatis. The cause of peliosis hepatis is thought to be damage to the sinusoidal cells. Basically, peliosis hepatis often occurs in connection with the administration of hormones. The medical agents vinyl chloride, tamoxifen and vitamin A also increase the risk of developing peliosis hepatis. Finally, some people develop peliosis hepatis after kidney transplantation.
Symptoms, complaints, and signs
Peliosis hepatis is characterized by cysts in the area of the internal organs. Typically, the lacunae, which are only a few millimeters in size, develop in the liver. In the majority of cases, peliosis hepatis is largely asymptomatic, so patients experience little discomfort and in some cases do not even notice the disease. However, in some individuals, the blood-filled cysts of peliosis hepatis burst. This causes intraperitoneal bleeding to develop, which is a serious complication. Thus, peliosis hepatis ends fatally for some of the patients as a result of the bleeding. In addition, some of the affected individuals develop manifest diseases of the liver as a result of peliosis hepatis, manifested, for example, by hepatomegaly or jaundice. Sometimes the function of the liver fails completely.
Diagnosis and course of the disease
Peliosis hepatis goes unnoticed in some cases because there are few symptoms. Clear symptoms sometimes develop only when cysts burst and subsequent hemorrhage 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 interviewing the patient, primarily uses imaging methods of examination. For example, the physician uses a CT scan and ultrasound methods to visualize the liver.Sonographic examination of the organ is also possible. This reveals the typical masses of peliosis hepatis. Sometimes patients are given contrast media before the examination to better distinguish the blood-filled cysts from the surrounding tissue. In addition, the physician 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 as well as venous occlusive liver disease.
Complications
Due to peliosis hepatis, the patient experiences a number of different complaints. As a rule, the further course and complications of this disease strongly depend on the organs affected, so that a general prediction cannot usually be made. Patients primarily suffer from cysts that develop in the internal organs. The liver in particular can be affected, resulting in cirrhosis of the liver in the worst case. In many cases, the disease is diagnosed late, when the cyst has already burst, so that early treatment is usually not possible. The bleeding of the cysts can be fatal for the affected person. The patients themselves also suffer from jaundice in this disease and further from a complete failure of the liver. Life expectancy is significantly reduced if treatment of peliosis hepatis does not occur. Surgical intervention can be used to remove the cysts. However, the affected person may have to undergo various interventions if not all cysts can be removed. No particular complications occur. A healthy lifestyle has a very positive effect on peliosis hepatis.
When should one go to the doctor?
Peliosis hepatis should be evaluated by a doctor if pain or swelling occurs in the area of the liver. If there are problems with urination, medical advice is also needed. Peliosis hepatis is a serious disease, but it can be treated well if the triggering drug is discontinued or an infection is detected early. If the above symptoms 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 primary care physician if signs of secondary disease occur. Peliosis hepatis is treated by the family physician or a specialist in diseases of the liver. Severe disease must be treated as an inpatient. After initial therapy, the physician should be informed of any complications and symptoms that may indicate a return of peliosis hepatis. Side effects of prescribed medications must also be clarified and treated promptly.
Treatment and therapy
In peliosis hepatis, there is a fundamental risk that the blood-filled cysts will burst and cause life-threatening complications due to bleeding in the abdomen. For this reason, removal of the diseased areas is usually performed as part of surgical intervention.
Outlook and prognosis
A uniform prognosis cannot be given for the liver with blood-filled cysts called peliosis hepatis. The reason lies in the different triggers by which this disease can develop. In addition, it may be an unrecognized liver cancer. A careful clarification of the cysts and their cause is therefore indicated. Physicians also distinguish between three different forms of peliosis hepatis. The prognosis of the parenchymatous form may differ from that of the phlebectatic form. In addition, bacillary peliosis hepatis is still known to occur in humans and some animal species close to humans. Often, affected individuals are infected with cat scratch disease by corresponding pathogens. In many cases, peliosis hepatis sufferers are patients who must take immunosuppressants before or after transplantation. Peliosis hepatis also frequently affects patients taking anabolic steroids, oral contraceptives, or androgens. Some affected individuals suffer from Hodgkin’s disease or tuberculosis. If such underlying problems are present, this worsens the prognosis. However, peliosis hepatis occurs relatively rarely. Its course is usually asymptomatic. However, complications may occur.A life-threatening complication would be a ruptured cyst. The blood from the ruptured cyst spills into the abdominal cavity. Consecutive intra-abdominal hemorrhage occurs, requiring immediate surgical intervention.
Prevention
Specific prevention of peliosis hepatis is almost impossible. The relevant risk factors of peliosis hepatis, such as taking certain medications, should be avoided whenever possible. If it is urgently necessary to take the medication, regular medical check-ups are recommended. Any alcohol abuse should also be discontinued as soon as possible.
Aftercare
In most cases, only a few measures or options for aftercare are available to the person affected by peliosis hepatis. First and foremost, the disease should be diagnosed early to prevent other complications or further worsening of symptoms. In the worst case, the affected person will die from the consequences of peliosis hepatis if it is completely ignored and not treated. Sufferers of this disease are dependent on surgical intervention, which can permanently alleviate the symptoms. The surgery should be performed as soon as possible after diagnosis to prevent rupture and bleeding. After such an operation, the patient should in any case rest and take care of his body. Strict bed rest should be observed, refraining from exertion and from physical activities. Regular check-ups and examinations by a physician are also very important in peliosis hepatis, as this is the only way to permanently control the condition of the liver. Alcohol should likewise 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.
What you can do yourself
If peliosis hepatis has been diagnosed, the first thing to do is to stop the triggering medication. If the condition is based on a serious disease such as AIDS or cancer, this must be treated concomitantly with therapy for peliosis hepatis. If an alcohol or drug problem is the cause, therapy must be initiated. The affected persons are usually physically very weakened and must take care of themselves. The diet usually has to be changed to relieve the liver and improve overall well-being. It is best for those affected to consult a nutritionist in order to work out a suitable diet together. General measures such as sport and occupation are recommended as support. Since the condition is often associated with considerable pain, pain therapy may also be necessary. In individual cases, inpatient hospitalization 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 as inpatients if their health deteriorates. After hospitalization, rest and recuperation are again the order of the day. In addition, a complaints diary should be kept so that the symptoms can be precisely defined and specifically treated. If the complaints increase despite all measures, further examinations are necessary.
