Lymphocytes, themselves subdivided into a variety of manifestations with different roles, are a subset of leukocytes. With few exceptions, they are part of the acquired immune defense, and their relative proportion of the total number of leukocytes is normally 25 to 45 percent of the leukocytes. If the relative proportion or absolute number falls below a certain level, lymphopenia is present.
What is lymphopenia?
Lymphopenia refers to a pathologically low absolute or relative number of lymphocytes in the blood. Thus, lymphopenia actually embodies lymphocytopenia. Lymphocytes, which are a subgroup of leukocytes, the white blood cells, can themselves be subdivided into a variety of different cell types with different tasks in the acquired immune defense. In principle, lymphopenia can be divided into an absolute and a relative form. Absolute lymphopenia occurs when the number of lymphocytes drops to a level of less than 1000 cells per microliter of blood. In relative lymphopenia, the proportion of lymphocytes within the group of leukocytes is less than 15 percent or, for other authors, less than 25 percent. A uniformly defined and binding standard value does not exist. Normally, the relative proportion of lymphocytes is about 20 to 40 percent of the total number of leukocytes. The absolute lymphopenia is more meaningful in terms of its physiological effect because the relative value depends on the total number of leukocytes. For example, if the absolute number of lymphocytes is within the normal range and the absolute number of the remaining leukocytes is pathologically elevated, lymphopenia could be mathematically inferred from this, although it is an increase in leukocytes, that is, leukocytosis.
Causes
Lymphopenias can develop because of a wide variety of complexes of causes. Lymphopenia is not always caused by a specific disease. For example, specific infections may cause a large increase in granulocytes, resulting in relative lymphopenia due to a greatly increased number of leukocytes. Absolute lymphopenias can be caused by a variety of different viral and autoimmune diseases, such as HIV, measles, and yellow fever, as well as multiple sclerosis (MS), celiac disease, Hodgkin’s disease, or rheumatoid arthritis. HIV infection is the most common cause worldwide for the development of infection-related lymphopenia. Typically, various forms of leukemia also cause lymphopenia. In many cases, the reduction in lymphocytes is caused by undesirable side effects of drugs such as immunosuppressants, cytostatics or glucocorticoids (cortisone preparations). Protein malnutrition, as is prevalent in some developing countries, is the most common cause of lymphopenia worldwide. In rare cases, it is a congenital immunodeficiency that causes a decreased number of lymphocytes from birth.
Symptoms, complaints, and signs
Lymphopenia is not accompanied by typical complaints or symptoms, but initially presents quite inconspicuously. This is also the reason why the disease is often discovered only during a blood test for other reasons. Accompanying symptoms, signs and complaints usually correspond to the causative disease, if lymphopenia is not based on other causes. For example, symptoms of hematologic disease that occur, such as eczema, dilated capillaries with bleeding, and other signs, are not caused by lymphopenia but by the causative hematologic disease.
Diagnosis and course of the disease
Absolute or relative lymphopenia can be diagnosed by laboratory chemistry using the blood count. However, because of a lack of specific symptoms, the examination usually does not take place because no cause is apparent. Frequently, the disease is discovered by chance only during an examination for other reasons. The situation changes only when the patient complains of frequently recurring infections. Congenital or acquired immunodeficiency may then be suspected. In these cases, a blood test can provide information on the absolute quantity and relative distribution of the various leukocytes and thus also of the lymphocytes.The course of the disease, which usually accompanies an underlying disease, depends on the course of the underlying disease. Lymphopenia can therefore develop in completely different ways. The course forms therefore cover a very different spectrum, ranging from harmless to severe – for example, in MS or leukemia.
Complications
In most cases, lymphopenia does not cause characteristic or special symptoms that are unique to the disease. It is recognized relatively late for this reason, so there is also a delay in treating the patient. As a rule, it can only be diagnosed during a blood test. The affected person may suffer from various bleeding episodes that occur for no particular reason. This does not result in restrictions or other complications in every case. Only rarely do the bleedings or swellings lead to pain or other restrictions in the daily life of the affected person. Furthermore, those affected suffer more often from infections and inflammations. However, the further course of lymphopenia depends very much on the causative underlying disease, so that a general course of the disease cannot usually be predicted. Treatment of the disease is primarily aimed at the underlying disease. It is also not possible to universally predict whether complications will occur. In many cases, however, the symptoms can be limited by stem cell transplantation so that there is no reduction in the patient’s life expectancy.
When should you see a doctor?
When fever, malaise, and other signs of lymphopenia are noticed, medical advice is needed. Affected individuals should consult the doctor immediately and have the symptoms clarified, especially if symptoms such as jaundice, bleeding or skin changes are added. Inflammations, eczema and dilated capillaries must also be examined by a doctor in any case, as there may be a serious underlying disease. Lymphopenia usually develops gradually and only causes clear signs of illness in the late stages. Therefore, a doctor should be consulted at the first sign of the disease. Risk groups include cancer patients and people suffering from pneumonia or another bacterial or viral disease. Anyone who has had rubella or severe inflammation should talk to their family doctor if they show signs of lymphopenia. The latter can diagnose the condition and consult various specialists as needed, such as dermatologists for possible skin problems, internists for organ disorders, and neurologists and physical therapists for neurological and physical ailments.
Treatment and therapy
Effective therapy for lymphopenia is aimed at treating the underlying disease. This requires that the underlying disease has been diagnosed with certainty and is effectively treatable. In the case of lymphopenia caused by side effects of drugs, it may be sufficient to substitute the drug with other drugs containing alternative active ingredients. If congenital immunodeficiency results in chronic deficiency of immunoglobulins and frequent infections are the consequence, intravenous administration of gammaglobulins with antibody properties may be indicated. If infusion with gamma globulins does not have the hoped-for effect, hematopoietic stem cell transplantation remains as the last resort. Lymphopenia caused by protein malnutrition could be treated and cured very easily by feeding a protein-rich diet.
Prospect and prognosis
The prognosis of lymphopenia depends on the underlying disease present. If it is a viral disease, it is curable in many cases. In medical therapy, the present disease is treated and often completely cured within a few weeks or months. In parallel, the number of leukocytes normalizes on its own. In the case of a chronic viral disease, the prognosis worsens. In this case, long-term therapy is used, during which there is relief from the symptoms. However, recovery is not to be expected. In the present malnutrition, the affected person can achieve sufficient change independently. By changing the food intake, the conditions within the organism change. The production of leucocytes automatically brings the organism into balance.If the dietary change is successfully implemented throughout life, a permanent freedom from symptoms can be documented. In particularly severe cases, the patient requires a stem cell transplant. For some patients, this is the last treatment option. The organism is already significantly weakened and other treatment methods have not been successful. Transplantation is associated with various risks and side effects. Nevertheless, in case of chronic deficiency or congenital immunodeficiency, it can bring the desired changes. In spite of everything, the patient must optimize his lifestyle and adjust it to the needs of the organism in order to achieve permanent freedom from symptoms. Otherwise, there will be a recurrence of symptoms.
Prevention
Direct preventive measures that could prevent the occurrence of lymphopenia do not exist, because the disease usually appears as a concomitant of another underlying disease. However, simple preventive measures do exist for the two leading causes of lymphopenia worldwide. These are, on the one hand, the nutritional form caused by chronic protein deficiency and the lymphopenia caused by HIV infection. In the former case, dietary protein fortification would be preventive, and in the latter case, effective protection against HIV virus infection could be estimated to be preventive.
Follow-up
Because the symptoms are ambiguous, lymphopenia is often recognized late. Accordingly, treatment is also delayed until after the late diagnosis. Because the disease as well as the treatment are relatively complex, follow-up care applies to trying to find a good way to deal with the situation. People with the disease are more susceptible to inflammations and infections and have to adapt their everyday life to the disease. This can lead to psychological upsets, which should sometimes be clarified by a psychologist. Therapy or contact with other sufferers can help to better accept the disease. This can increase the sense of well-being, regardless of the fact that the treatment is still ongoing. A general course of lymphopenia cannot usually be predicted, as it depends on the underlying disease causing it. In order to treat lymphopenia, the underlying disease must first be identified and treated. Whether complications or discomfort will result from treatment can only be estimated on a case-by-case basis. In most cases, the symptoms can be limited by a transplantation of stem cells. The lifespan of patients with the disease is then not reduced.
This is what you can do yourself
In the case of lymphopenia, the patient’s options for self-help are relatively limited. In this case, the patient’s underlying disease must be treated first and foremost, and a general statement about the means of self-help is not possible or useful in this case. However, if the lymphopenia is caused by certain medications, these medications should be changed or discontinued. A change of medication should only be made after consultation with a physician in order to avoid complications. Often, however, lymphopenia can only be treated by stem cell transplantation. In this case, the affected person also has no options for self-help. In general, the affected person should take it easy with this disease in order to avoid further infections or illnesses. Unnecessary exertion should be avoided. If the lymphopenia leads to psychological complaints, discussions with parents or friends are very helpful. Likewise, contact with other people affected by lymphopenia can often have a good effect on the course of the disease, as it leads to an exchange of information. This can be helpful in everyday life and increase the quality of life of the affected person.
