Lymphatic cancer, bone marrow cancer – these are the common names for a whole range of malignant diseases of the so-called lymphatic system. What warning signs you shouldn’t ignore.
Unlike the more well-known forms of cancer, it is usually not just a single organ that is affected, as the lymphatic system, as part of our immune system, runs through the entire body.
The lymphatic system includes:
Depending on which cells the cancer originates from, it can be divided into different groups:
1. Hodgkin’s lymphoma (Hodgkin’s disease), lymph node cancer, around 2,400 new diagnoses per year in Germany. As the disease progresses, the spleen, bone marrow, lungs and liver can also be affected. This form of cancer was named after the British doctor Thomas Hodgkin (1798 to 1866), who was the first to describe this disease. Footballer Lainer is now also suffering from this form.
2. Non-Hodgkin’s lymphoma is the most common form, with over 17,000 new cases affected every year. It includes all other malignant diseases of the lymphatic system that are not classified as Hodgkin’s lymphoma. “It is a group of over 50 individual lymphomas, including the particularly aggressive Burkitt lymphoma,” reports Heidelberg lymphoma expert Professor Mathias Witzens-Harig. The hemato-oncologist researches non-Hodgkin’s lymphomas and has already published numerous scientific papers in this context.
3. Multiple myeloma, which occupies a special position in the group of non-Hodgkin lymphomas. The focus here is on damage to bones, bone marrow and kidneys. Over 6,000 men and women are newly affected in Germany every year.
Each of these diseases originates from specific cells in the lymphatic system. Depending on the cells of origin, these carcinomas are divided into three categories. In Hodgkin’s lymphoma and non-Hodgkin’s lymphoma, altered lymphocytes are present.
In most cases it is still unknown why the cells degenerate and lymphoma forms. Medicine assumes that the disease usually arises from random errors in cell division, as is also the case with other forms of cancer. “Only very few forms of non-Hodgkin lymphoma have a clear cause; they are associated with certain infections,” reports the expert. Various viruses and bacteria would play a role here, such as:
However, the typical risk factors for cancer, such as smoking, alcohol or obesity, only play a minor role in lymphoma.
The signs of Hodgkin’s and non-Hodgkin’s lymphoma are similar, common are:
If the bone marrow is involved, a deficiency of white and red blood cells as well as blood platelets (thrombocytes) can occur. Accordingly, the following signs appear:
In multiple myeloma, the disease originates in plasma cells that are located in the bone and bone marrow. This can lead to bone defects. “These so-called osteolyses are painful and can also lead to bone fractures,” reports the oncologist. In addition, if bone marrow is involved, abnormalities such as anemia and the other symptoms listed are possible.
There is also a risk of kidney failure with multiple myeloma. Professor Witzens-Harig explains the background: “The diseased plasma cells form a pathological protein, the paraprotein. The paraprotein or parts of it, the so-called light chains, lead to kidney damage.”
Kidney problems, but also bone pain without previous trauma, are therefore considered important symptoms and should be clarified immediately.
If Hodgkin’s or non-Hodgkin’s lymphoma is suspected, the diagnosis is made from a tissue sample, i.e. histologically. To do this, a lymph node, for example on the neck, is removed and examined. The result shows what form of lymphoma it is.
To identify multiple myeloma, some bone marrow is removed and examined histologically, and a blood sample or urine sample (detection of paraprotein and light chains) is evaluated. In addition, the entire skeleton is examined using imaging, for example computer tomography, to determine whether and where osteolysis occurs.
The chances of recovery “are very good for Hodgkin lymphoma,” says the lymphoma expert. With the combination of chemotherapy and radiation therapy, the prognosis is usually very positive. New treatment options may gradually complement chemotherapy. These are currently the antibody therapies. “The conjugated antibody Brentuximab, which is directed against CD 30, plays an important role here,” explains the professor in more detail. CD 30 refers to the target structure on the lymphoma cell, conjugated means that there is still a poison attached to this antibody that is supposed to destroy the cancer cell.
Checkpoint inhibitors are also used, i.e. PD1 antibodies such as pembrolizumab and nivolumab, “they also work well in Hodgkin lymphoma,” he reports. Checkpoint inhibitors are drugs that activate certain of the body’s own immune cells, the so-called T lymphocytes, and enable them to attack the tumor cells.
The therapy here depends on whether the patient has an indolent, i.e. low-grade, or aggressive, i.e., high-grade non-Hodgkin lymphoma.
“Indolent lymphomas can often be cured in the early stages with radiation,” says Professor Witzens-Harig. In the advanced stage, indolent lymphomas are usually no longer curable, but are usually very easy to treat. In this situation, treatment is only started if symptoms are present.
However, indolent non-Hodgkin’s lymphomas progress so slowly that some affected people can live with them without symptoms for many years, even without treatment.
If indolent lymphoma needs to be treated, chemotherapy is supplemented with new medications. These include the antibodies rituximab and obinutuzumab, which are directed against the target molecule CD 20, as well as immunomodulatory substances such as lenalidomide.
In special situations, treatments that are completely chemotherapy-free are even possible. For more advanced diseases, stem cell transplantation and, in the future, possibly also innovative CAR T-cell therapy are used in suitable patients.
Chemotherapy works particularly well here because almost all cancer cells are active, dividing and therefore easily vulnerable to the active ingredients. Similar to the treatment of indolent non-Hodgkin lymphomas, stem cell transplantation and, more recently, CAR T-cell therapy are used for advanced diseases.
“The most modern treatment is currently therapy with CAR T cells,” emphasizes Professor Witzens-Harig. The explanation is a bit complicated, but the mechanism is clever and extremely successful. Healthy lymphocytes are taken from the patient’s blood. These T lymphocytes receive gene therapy in the laboratory and receive an artificial T cell receptor via a virus. This receptor is directed against a tumor antigen (CD 19). In this way, the lymphocytes are equipped with an artificial T-cell receptor. These patient’s own, genetically modified T lymphocytes are called CAR-T cells. The CAR-T cells are now multiplied and given back to the patient as medication.
“It is an immunotherapy with the patient’s own T lymphocytes, which have been genetically modified and thereby activated. “They are able to destroy the patient’s tumor cells in a targeted manner,” summarizes the scientist. The effect of the new therapy is very good: patients for whom several other treatments had already failed became permanently disease-free in up to 40 percent of cases. This is an excellent result for patients who would otherwise have had a very serious prognosis, emphasizes the expert.
This is a disease that cannot yet be cured. The decision about the need for therapy for multiple myeloma is based on the criteria of the International Myeloma Working Group (IMWG). This includes, among other things, anemia, kidney and bone involvement and a pathological increase in blood calcium levels.
Therapy usually consists of a combination of several effective medications. This includes
In addition, numerous other promising therapeutic options are being tested in clinical studies. In multiple myeloma, this also includes CAR T cells but also so-called bispecific antibodies, which can cause a particularly strong activation of the patient’s immune cells.
So there are forms of lymphoma that need to be treated as quickly as possible; for others you can wait and watch. However, it cannot be distinguished based on the symptoms alone. The expert’s urgent request to anyone who registers symptoms such as swollen lymph nodes or night sweats for a long time, fever or weight loss, without any explanation:
Go to a doctor, preferably a hematologist, and get yourself examined. Aggressive non-Hodgkin lymphomas and often multiple myeloma must be treated immediately to avoid damage to your health.