Hepatosplenomegaly is the medical term for a simultaneous enlargement of the spleen and liver. A wide variety of causes may underlie this symptom combination, including infectious associations or hereditary storage diseases. Treatment of the symptoms depends on the primary disease.
What is hepatosplenomegaly?
Hepatosplenomegaly is not a disease itself. Rather, it is the symptom of various diseases. Hepatosplenomegaly is always referred to when a patient manifests simultaneous swelling and enlargement of the spleen and liver. Thus, the symptom is actually two different symptoms or a combined symptom complex of an enlarged liver in the sense of hepatomegaly and an enlarged spleen in the sense of splenomegaly. The simultaneous presence of these symptoms has clinical relevance, as it may provide the physician with a clue to the larger framework of the primary disease in light of the history. Hepatosplenomegaly is acquired in most cases and, as an acquired symptom complex, is usually due to infection. The congenital form of both symptoms in the infant often has storage diseases or lymphatic systemic diseases as its cause. Thus, to diagnose for a primary disease, the age of the affected person is most relevant.
Causes
Hepatosplenomegaly can be due to a variety of causes. The acquired form is often associated with viral infections such as Epstein-Barr virus or cytomegalovirus. A connection with bacterial infections is also conceivable in the acquired form. The symptom combination thus characterizes, in addition to brucellosis, for example, ornithosis, parasitosis, malaria and schistosomiasis or leishmaniasis. If the acquired form occurs without infections, tumors may be the cause. The congenital form of both symptoms points mainly to storage diseases such as amyloidosis or mucopolysaccharidosis. Diseases of the lymphatic system are also possible causes of congenital splenic-liver enlargement. Other causative associations may include neuroblastoma, sarcoidosis, syndromes such as Hurler syndrome or Schnitzler syndrome, Farber disease, and preductal aortic isthmic stenosis. Apart from these, the combined symptoms are often present in hepatolienal diseases such as right heart failure or liver disease. In addition, blood disorders such as polycythaemia vera have been associated with the phenomenon. Identifying the cause is all-important for developing appropriate therapeutic steps.
Symptoms, complaints, and signs
The symptoms of hepatosplenomegaly depend greatly on the cause in each individual case. In principle, enlargement of the spleen and liver usually does not cause pain and, in isolation, may even be entirely asymptomatic. Often the swellings are visible to the naked eye. Depending on the extent of the swelling, the phenomenon may compress neighboring organs of the liver and spleen. In such a case are usually associated with loss of function of the compressed organs. In the case of extremely massive swellings, pain also presents. The present accompanying symptoms of splenic-liver enlargement are one of the most important clues for the physician to the actual cause of the disease. For example, infectious diseases are often associated with inflammation, redness and local pain or cough, hoarseness and cold. In addition, fever is a common accompanying symptom of infectious hepatosplenomegaly. When cardiac congestion is responsible for the enlargement of the two organs, upper abdominal pain is the most common accompanying symptom. To enumerate all possible accompanying symptoms of hepatosplenomegaly at this point is beyond the scope and makes little sense, as the clinical picture can be characterized by just about any symptom, depending on the cause.
Diagnosis and course
The first suspicion of hepatosplenomegaly usually comes to the physician by visual diagnosis and palpation. Physical examination in the form of palpation and percussion reveals enlargement of the liver. Splenic palpation with the patient in the right lateral position provides evidence of splenic enlargement. Sonography confirms the diagnosis. In addition to determining size, ultrasound also helps to determine the internal and surface structure of the two organs.Sonographic examination of the arteries and veins can detect or rule out causes such as hepatic vein congestion or portal vein dilatation. As an alternative to ultrasound, a CT scan can be performed. Laboratory values are particularly relevant for causal diagnosis, especially metabolic parameters, immunological parameters and, if necessary, tumor markers. Blood tests can also be used to determine or exclude infectious causes. For further cause investigation, procedures such as liver puncture or bone marrow puncture may be required.
Complications
Because hepatosplenomegaly is a complex symptomatic condition that should be considered an indicator of serious health problems, the nature of treatment is aimed at eliminating the particular cause rather than the hepatosplenomegaly itself. Therefore, the complications that occur in the course of therapy depend on it and are accordingly diverse in nature. In the case of untreated hepatosplenomegaly, various complications can occur. These include internal bleeding and the appearance of blood in the stool and vomit. Organs that are in close proximity to the respective enlarged spleen and liver may become compressed and thus impaired in function. In addition, liver insufficiencies and even liver failure may occur, which in turn may result in jaundice or blood clotting disorders, as well as changes in consciousness. These range from mood swings and sleep disturbances to states of confusion and coma. Furthermore, leg edema as well as congested neck veins can be observed as complications. In addition, there may be a disturbance of fine motor skills, which manifests itself in severe trembling of the hands. Another serious complication that can occur as a result of hepatosplenomegaly is encephalopathy, which is a pathological change of the entire brain. This is based on a disruption of brain cells that can no longer adequately perform their respective functions; this dysfunction may or may not be permanent.
When should you see a doctor?
A physician should be consulted as soon as unusual swelling can be detected in the area of the upper body. If there is pain, a feeling of pressure or an incomprehensible inner tension, a visit to the doctor is necessary. If the complaints begin to spread or increase in intensity, a check-up visit must be made. If circulatory disturbances, palpitations or circulatory problems occur, a medical examination is needed. Complaints such as fever, a general feeling of illness, dizziness or reduced performance must be clarified and treated by a doctor. In case of vomiting, loss of appetite or sleep disturbances, a visit to the doctor is necessary. Special care should be taken in case of repeated blood in urine or stool. A physician should be consulted as soon as possible so that the cause can be determined. The affected person requires immediate medical attention in case of confusion, drowsiness or disturbance of consciousness. It is advisable to call an emergency physician and provide first aid care until he arrives. Noticeable skin changes should also be clarified by a doctor. Discoloration of the skin, pimples or edema are considered unusual and should be examined. If swelling of the legs occurs that leads to motor problems or if the affected person suffers from lassitude or apathy for several days, a physician should be consulted.
Treatment and therapy
Hepatosplenomegaly is not treated symptomatically. All treatment measures are aimed at eliminating the cause and thus correspond to causal therapy. Treatment depends so much on the actual trigger of the organ enlargements. If infections have brought about the enlargement, the administration of antibiotics, for example, is an important step in the therapy. In the case of cardiac causes, such as congestion from the heart, drug therapy can also take place. However, surgical treatments may also be required as part of such causes, for example, heart valve replacement or therapeutic steps such as stenting. In the case of storage diseases and hereditary congenital syndromes, causal therapy is not possible. In this case, the cause of the organ enlargements cannot be eliminated. Depending on the clinical picture, such diseases are treated symptomatically.If the hepatosplenomegaly is due to a tumor, the focus of therapy is on excision of the tumor. Depending on the type of growth, additional treatments such as radiation therapy may follow.
Outlook and prognosis
The prognosis of hepatosplenomegaly is always dependent on the underlying disease. The combined enlargement of the liver and spleen is merely a symptom that indicates another disease. If the underlying disease is successfully treated, the hepatosplenomegaly also disappears. There are many causes. They may be viral infections, bacterial infections, parasitoses, tumors, storage diseases, diseases of the lymphatic system, and many other precipitating diseases. Thus, the prognosis of hepatosplenomegaly cannot be made separate from the prognosis of the individual underlying disease. There is a very good prognosis for certain infectious diseases. Among others, this applies to Pfeiffer’s glandular fever (Epstein-Barr virus). However, severe infections and parasitoses such as brucellosis, ornithosis, malaria, leishmaniasis or schistosomiasis often play a role. The prognosis of the individual diseases varies. The prognosis is even worse for tumors and diseases of the lymphatic system, although here, too, a differentiation must be made. The storage diseases, among others, are not curable and often lead to permanent hepatosplenomegaly. Many diseases are also genetic. Congenital causes of hepatosplenomegaly also include preductal and postductal aortic isthmic stenosis. This form of hepatosplenomegaly is very life threatening. The lethality is approximately 90 percent if no therapy is given. However, both forms of stenosis can be successfully treated by surgery.
Prevention
Hepatosplenomegaly can have so many causes that all preventive measures cannot be listed here. The causes with a genetic basis cannot be prevented. Some of the infectious causes can be prevented by vaccination. Congestion or underlying cardiac conditions can be prevented by healthy diet, exercise, and antihypertensive measures in moderation.
Follow-up care
In most cases of hepatosplenomegaly, no special or direct measures or options for aftercare are available to the affected person. In this case, it is primarily a matter of rapid and, above all, early diagnosis so that there is no further worsening of the symptoms or other complications. For this reason, the patient should seek medical attention at the first symptoms and signs of hepatosplenomegaly to prevent further complications or discomfort. In the treatment of hepatosplenomegaly, very many regular visits to a doctor are necessary. This involves regular examination of the internal organs to quickly identify the underlying disease. The disease itself is treated with the help of medication. The affected person should pay attention to a regular intake and to a correct dosage in order to alleviate the symptoms properly. If antibiotics are taken, it is advisable not to consume alcohol so as not to reduce the effect of the antibiotics. In many cases, patients are dependent on the support and help of friends and their own family for this disease. This can also prevent psychological upset or depression.
Here’s what you can do yourself
Medical treatment for hepatosplenomegaly focuses on correcting the causes. The patient can take some measures to alleviate the individual symptoms and complaints. In addition to medically prescribed drugs, gentle preparations such as St. John’s wort capsules or valerian tea also help against the typical sleep disturbances. Mood swings can also be alleviated by natural sedatives. Exercise and an overall healthy and balanced lifestyle also help to ensure that irritability, fatigue and other typical symptoms subside quickly. Should states of confusion develop, prudent action must be taken in everyday life to avoid accidents. Other symptoms such as blood clotting disorders or changes in consciousness require medical treatment. The patient should inform the physician of all symptoms and seek hospitalization if symptoms increase.Although the simultaneous enlargement of the spleen and liver is not life-threatening, the symptoms nevertheless represent a considerable burden for patients. Psychological support is therefore useful and necessary. Talking to other sufferers, for example on Internet forums or in a self-help group, makes it easier to cope with the disease and reveals new ways of dealing with it.