Hypersplenism is a condition that may occur in conjunction with splenomegaly. In this condition, the spleen enlarges, increasing its functional effects more than necessary and causing difficulties.
What is hypersplenism?
Basically, the term hypersplenism refers to hyperfunction of the spleen. The synonymous term hypersplenism may also be used for the condition. In the majority of cases, hypersplenism forms as a result of an enlargement of the spleen. This condition is referred to as a complication of splenomegaly. In connection with the enlargement of the organ, its capacity is significantly increased. As a result, a larger number of blood cells accumulate inside the spleen. In addition, more blood cells are released from the organ. As a result, the blood cells decrease, which is also called pancytopenia. Different types of blood cells such as leukocytes, erythrocytes and platelets are involved to different degrees. The organism tries to compensate for the pathological condition by increasing the bone marrow.
Causes
Several causes exist for the development of hypersplenism. As a rule, certain underlying diseases are responsible for the formation of hypersplenism. Very often, these are blood-related diseases, rheumatoid arthritis, or high pressure in the portal veins. Basically, a distinction is made between primary and secondary hypersplenism. Primary hypersplenism occurs, for example, in connection with Banti’s disease. Secondary hypersplenism is possible in several diseases that imply an enlargement of the spleen. In addition, various infections are possible causes, such as malaria, kala-azar, or Felty syndrome. In addition, some malignant lymphomas, liver cirrhosis or various autoimmune diseases, such as erythematosus, lead to the formation of hypersplenism. In addition, various storage diseases, for example Gaucher syndrome or thesaurismoses are capable of causing hypersplenism. In principle, primary hypersplenism is not caused by any other underlying disease. Secondary causes are, for example, diseases of the gall bladder or liver such as viral hepatitis or cholangitis. Hematogenous causes include hemolytic anemias, leukemias, or Hodgkin’s disease. Possible storage diseases include hemochromatosis or glycogenoses. Infectious diseases that may cause hypersplenism include AIDS, typhoid fever, mononucleosis, and leptospirosis. Toxoplasmosis, Bang’s disease, rubella, and paratyphoid fever are also included.
Symptoms, complaints, and signs
Hypersplenism is associated with a number of typical symptoms and complaints. The enlargement of the spleen causes increased blood cells to be released from the organ. These are primarily erythrocytes, leukocytes, and platelets. As a result, pancytopenia, a so-called cell deficiency, develops. The different types of blood cells are involved to varying degrees. If anemia develops, frequent blood transfusions may be necessary. Thrombocytopenia increases the tendency to bleed, while leukocytopenia makes the affected patient more susceptible to infectious diseases. In response to the enlargement of the spleen, hypertrophy of the bone marrow responsible for blood formation occurs. If the spleen is particularly enlarged, it may cause pain as adjacent organs are squeezed. General symptoms of hypersplenism include dry mucous membranes, signs of anemia, or an unusually strong craving for sweet foods. If the spleen is slightly enlarged, symptoms of endocarditis or typhoid fever are possible. Moderate enlargement manifests itself, for example, in leukemia, hepatitis, or cirrhosis of the liver. If the spleen is particularly enlarged, affected patients sometimes suffer from a feeling of pressure in the upper abdominal region on the left side of the body. In principle, the functions of the spleen increase according to the degree of its enlargement. The consequences are cytopenia, anemia or thrombocytopenia. Hyperplasia of the bone marrow is also possible.
Diagnosis and course of the disease
If a person suffers from one or more typical symptoms of hypersplenism, a physician should be consulted.In the first step of the examination, the attending physician analyzes the patient’s medical history. Following the anamnesis, various clinical examinations are performed. For example, the spleen is palpated, which is significantly enlarged in hypersplenism. An ultrasound examination clearly shows the enlargement of the spleen and indicates the disease. The suspected diagnosis of hypersplenism is confirmed by a blood test. Here, the decreased percentage of blood cells becomes apparent. Laboratory tests also reveal the increased bone marrow. A definite diagnosis can be made by scintigraphy.
Complications
In most cases, hypersplenism leads to enlargement of the spleen. Severe pain for the patient occurs. If hypersplenism is not treated, various symptoms may develop in the long run. As a rule, the patient is more susceptible to infections and inflammations and thus becomes ill more often as well. The affected person feels weak and weary and no longer takes an active part in life. The enlargement of the spleen can also displace or squeeze other organs, leading to complications in the affected organs. As the disease progresses, it leads to cirrhosis of the liver and, in the worst cases, leukemia. The affected person also suffers from unpleasant feelings of pressure on the left side of the body, which make everyday life more difficult for the affected person and greatly reduce the quality of life. Without treatment, the worst case scenario for the patient can be death. In many cases of hypersplenism, the spleen is completely removed from the patient’s body. Since this organ is not vital, no further complications occur. However, in many cases, the symptoms can also be limited with the help of blood transfusions, so no surgical intervention is necessary either.
When should you go to the doctor?
If there is noticeable pallor in the face, internal weakness or lassitude, a doctor should be consulted. If there is heavy bleeding from minor injuries, this is considered unusual and must be checked. The affected person is threatened by an undersupply of the organism and thus a life-threatening condition due to the existing anemia. Dizziness, unsteadiness of gait, lack of strength and loss of energy are indications that should be investigated. If the affected person suffers from infections more frequently or is prone to inflammation, a follow-up visit to clarify the clues is advisable. Pain, labored breathing or a tightness in the upper body, should be investigated and treated. Dry mouth, dry mucous membranes or difficulty swallowing should also be medically clarified. If the affected person feels the need to eat more sweet foods, this is considered unusual. A doctor should be consulted if this urge for sweet foods continues abruptly for several weeks or months. If headaches, increased heart palpitations or reduced mental performance occur, a visit to the doctor is advisable. If there is decreased physical strength as well as increased fatigue, a doctor is needed. Lack of drive, withdrawal behavior and a melancholic mood are indications that should be followed up. If there are disturbances in attention or gait unsteadiness, a doctor’s visit is advised.
Treatment and therapy
Various options are available for the therapy of hypersplenism. One is regular blood transfusions to compensate for the lack of blood cells. This is especially the case in anemia or thrombocytopenia. In principle, however, the spleen is not an organ that is absolutely necessary for the viability of the human organism. For this reason, removal of the spleen (medical term splenectomy) is recommended in cases of serious symptoms.
Outlook and prognosis
Hyperplasia of the spleen may have various causes. These determine the prognosis of hypersplenism. If the complication occurs as a result of cancer, the prognosis is made depending on the prospect of cure, the time of diagnosis, the patient’s general health, and the effectiveness of therapy. With successful cancer therapy and completion of all follow-up treatments, hypersplenism may completely resolve.If the cancer has progressed to the point where a cure can no longer be achieved, the treatment plan is restructured. The aim is then to alleviate the symptoms of severe impairment and not to cure the hypersplenism. In the case of a chronic underlying disease, regression of the enlarged organ is not indicated. In a long-term therapy the organism is regularly supported with the necessary blood cells. Permanent regeneration of the spleen is rarely or never achieved. If the treatment plan foresees removal of the spleen, spontaneous healing of all symptoms associated with hypersplenism occurs. The organ, which is not vital, can be removed from the patient in a routine procedure if the pain is severe or symptom relief is hopeless. However, because surgery is inherently associated with risks and side effects, sequelae or complications may occur.
Prevention
Concrete methods and measures for the effective prevention of hypersplenism are currently not known or have not been sufficiently researched. It is therefore all the more important to consult a suitable physician at the first signs of hypersplenism and to have the symptoms examined. This is because a timely diagnosis has a positive effect on the prognosis.
Follow-up
Aftercare for hypersplenism depends on the severity of the disease and whether the spleen was removed in surgery. The condition generally cannot be prevented by patients’ own preventive measures. Therefore, medical therapy is essential for patients. During follow-up, it is important to recognize early signs of a relapse immediately and to examine them during a medical appointment. The sooner the diagnosis is made, the better the prognosis for those affected. Patients should also take it easy during the follow-up phase. Physically demanding activities and stress are a high burden and can have a negative impact on health. In general, patients receive regular blood transfusions. Consistent appointment scheduling is very important here. Especially in connection with anemia, doctors recommend close monitoring to detect any deficiency symptoms or other complaints in time. Special dietary supplements are available to counteract the typical deficiency symptoms in patients. The attending physician can assess which products are suitable for the individual case and how high the dosage should be. Therefore, the intake of such means should necessarily be in close consultation with the medical professional.
What you can do yourself
Hypersplenism can usually not be treated by means of self-help. Affected persons are thereby dependent on medical treatment in any case, so that it does not come to a reduced life expectancy. Patients suffering from this disease should take it easy and not expose themselves to strenuous activities or unnecessary stress. Since the symptoms can only be treated by regular blood transfusions, care should be taken to perform them regularly. Regular examinations are especially necessary in the case of anemia. Since some affected persons also suffer from deficiency symptoms, nutritional supplements can be taken in this case. However, the type and amount of these agents should always be discussed with a doctor first. In severe cases, sufferers of hypersplenism are dependent on the removal of the spleen. To avoid complications with this condition, a doctor should be consulted at the first signs. Frequently, contact and exchange of information with other sufferers also has a very positive effect on the course of the disease. In the case of psychological complaints, discussions with family members or friends are very helpful. In severe cases, a psychologist can also provide help.
