The so-called CNS lymphoma is an extremely rare form of tumor in the lymphatic tissue; the tumor develops in the central nervous system. Doctors differentiate between primary CNS lymphoma (also known as PZNSL) and secondary CNS lymphoma. Primary CNS lymphoma occurs first in the central nervous system; the secondary represents a spread (so-called metastases) of lymphomas that arise in other regions of the body.
What is CNS lymphoma?
CNS lymphomas are tumors that have formed in the lymphatic tissue ; the designation CNS indicates that the tumor is located in the central nervous system. The CNS lymphomas are subdivided; there are primary CNS lymphomas and secondary CNS lymphomas. Primary lymphoma is the one that first forms in the central nervous system. For meaning of mesenteric lymphadenitis in English, please visit sportingology.com.
Secondary lymphoma arises as a spread from other lymphomas that first appear in other parts of the body. The secondary CNS lymphomas occur less frequently; This is because the patients are treated in advance, so that resettlement can often be prevented. Since the HIV or AIDS epidemic at the beginning of the 1980s, there have been significantly more cases of CNS lymphoma.
This is because there is an extremely strong link between primary CNS lymphoma and what is known as immunosuppression. CNS lymphoma is the most common tumor in patients suffering from AIDS. However, not only AIDS patients are affected; even immunocompetent people are often affected. 5 to 29 percent of all patients suffering from the so-called systemic non-Hodgkin lymphoma also develop secondary CNS lymphoma. This is where settlements in the brain take place again and again.
Causes
So far there are no indications or explanations as to why CNS lymphomas develop. However, physicians have recognized any risk factors that very well promote the development of such a lymphoma: These include immunosuppression, EBV infections or diseases of the so-called rheumatic type. Histogenetic investigations have also shown that the mutation of the protoocogene “BCL-6” can also play a role. However, whether and to what extent there is actually a connection here that ultimately leads to the occurrence of a CNS lymphoma cannot yet be said with 100 percent.
Symptoms, Ailments & Signs
If the patient is suffering from a primary CNS lymphoma, he subsequently suffers from personality disorders, disorientation, cognitive disorders and also from a psychosomatic slowdown. Patients often have diffuse and multifocal masses; since accompanying edema occurs, symptoms can often be obscure and not automatically indicative of CNS lymphoma.
Other possible symptoms are hemiparesis, meningitis, hemiesthesia, visual field defects, aphasia, visual impairment and endocrine disorders if the tumor is located in the hypothalamus. Around 50 percent of patients develop increased intracranial pressure over the course of the disease; almost all those affected also develop the classic symptoms of emesis and nausea.
If the temporal lobe is affected, more and more frequent seizures follow. However, the symptoms can vary in severity depending on the location of the lymphoma, stage, medical condition and age of the patient.
Diagnosis & course of disease
As part of the diagnostics, doctors rely on imaging methods. Around half of the patients have multifocal involvement; that is evident in the parietal and frontal lobes. An avascular structure is evident on angiography; the CT shows isodense or hyperdense, contrast medium-storing and ventricular results
An MRI can also give an indication of whether it is a CNS lymphoma or not. Subsequently, lumbar punctures are possible; sometimes a brain biopsy can also provide a result as to whether it is a CNS lymphoma or not. The biopsy is mainly used for certainty, so that process is chosen purely as a diagnostic confirmation.
If there is CNS lymphoma, around 50 percent of those affected live longer than four years after the diagnosis was announced. A large proportion of those patients who have not yet passed the age of 60 can actually be cured as part of chemotherapy. 50 percent of those patients who also have AIDS die after five months. As a rule, there is no hope or any chance of recovery for people suffering from AIDS; Here, doctors primarily try to alleviate the symptoms or prolong life by several months.
Complications
CNS lymphoma is a very malignant and rapidly growing tumor of the lymphatic system. Left untreated, the disease always leads to fatal complications. With the therapy, which consists of radiotherapy and chemotherapy, very good healing successes are achieved. However, this only applies to patients with a healthy immune system.
However, the tumor is inoperable because it is diffuse and not confined to a specific location in the brain. However, a weakened immune system after organ transplants or in the case of AIDS is often the cause of CNS lymphoma. These people have the most complications, which are quickly fatal in over 50 percent of cases. The therapy can help patients with a weakened immune system to prolong their lives.
However, the disease in this group of people is not curable. However, the start of treatment is crucial for the chances of recovery in people with a healthy immune system. Lymphomas in the brain grow very quickly and can cause irreversible damage if therapy is not started immediately after diagnosis. In over 50 percent of patients, organic brain changes occur that are triggered by cerebral edema.
This leads to personality changes, disorientation, cognitive disorders and general psychosomatic slowdowns. In people with a weakened immune system, however, this organic brain damage and fatal progression of the disease are found much more frequently, even under therapy, than in people with a healthy immune system.
When should you go to the doctor?
A doctor is needed as soon as there is swelling of the lymph. If this swelling persists for a long time or if it increases in intensity and extent, a medical examination must be initiated as soon as possible. Since the disease leads to the premature death of the affected person if left untreated, an immediate diagnosis and a treatment plan are required. Personality changes, behavioral problems, or disorientation are causes for concern. If there are general cognitive disorders, a slowing down of movement processes or a diffuse occurrence, a doctor must be consulted.
A feeling of illness, a general malaise and a decrease in physical and mental performance must be presented to a doctor. If the person concerned reports symptoms of paralysis, sensory disturbances or a feeling of numbness, medical help is necessary. Headaches, a feeling of pressure in the head, tiredness and withdrawal behavior are alarm signals from the organism. A doctor’s visit is necessary so that the cause can be clarified.
General functional disorders, rapid fatigue despite a restful night’s sleep, and psychosomatic disorders must be examined. If the person concerned can no longer carry out everyday duties as usual or if his or her usual memory is impaired, a doctor should be consulted. In many cases, those affected are dependent on the cooperation of relatives because they cannot help themselves sufficiently due to the symptoms.
Treatment & Therapy
In the foreground of the treatments are chemotherapeutics that go through the brain – this includes, for example, methotrexate – and radiotherapy. The radiation dose is lower (40 to 50 Gy) than for other brain tumors. The chemotherapy is applied intrathecally (that means in the cerebrospinal fluid space); Doctors mainly rely on the lumbar puncture.
These therapy options are mainly used when it comes to patients who are suffering purely from CNS lymphoma. However, if it is an AIDS patient, physicians do without chemotherapy. In AIDS patients, only brain radiation is used.
Sometimes there is also the possibility of surgical removal. However, this possibility is only considered if the patient does not respond to either radiation or chemotherapy. If the lymphoma regresses, the doctor subsequently refrains from an operation. Immunocompetents in particular respond very well to the therapies (chemotherapy and radiotherapy).
Prevention
Since doctors cannot yet say with 100 percent certainty which causes are actually responsible for the development of CNS lymphoma, there are no preventive measures.
Aftercare
In the case of CNS lymphoma, those affected usually have only very few and only very limited direct follow-up measures available. For this reason, the person affected by this disease should ideally consult a doctor at an early stage and initiate treatment in order to prevent the occurrence of further complications and symptoms.
As a rule, CNS lymphoma cannot heal on its own, so a visit to a doctor is necessary. Other areas of the body should also be examined for tumors so that they cannot spread further. Most of those affected are dependent on radiation therapy.
The support and care of one’s own family is often very important in order to alleviate psychological problems in particular. Those affected should be examined regularly by a doctor in order to monitor the condition of the CNS lymphoma over the long term. Furthermore, contact with other people affected by this disease can also be very useful, since it is not uncommon for an exchange of information to take place, which can make the everyday life of the person affected significantly easier.
You can do that yourself
Strengthening and daily support of the immune system is of immense importance for CNS lymphoma patients. Although the disease cannot be cured, the person affected needs emotional and physical stability in order to successfully cope with the overall circumstances. A healthy and balanced diet is just as important as physical activity and an adequate supply of oxygen.
The zest for life should be promoted through various activities. All activities must be adapted to the possibilities of the organism so that situations of excessive demands are avoided. Sufficient periods of rest and recovery must be included so that there is enough space for regeneration. In addition, the consumption of harmful substances should be avoided. Nicotine, alcohol or drugs damage the body and can contribute to a worsening of the overall condition. The risk of infection should be minimized. Particular care must be taken in this regard, especially at times when the seasons are changing.
The risk of infection with bacteria, viruses or other germs can be reduced by taking precautionary and protective measures, as otherwise serious complications can occur. Sufficient hygiene and regular disinfection are helpful. To strengthen mental strength, stressors must be eliminated. Conflicts or persistent interpersonal disagreements can lead to states of mental stress and thus weaken the entire organism.
