Cancer: Overview of rare lymphomas

Lymph gland cancer can develop extremely rare forms

Our lymphatic system is spread all over the body. It consists of lymphatic organs such as tonsils (tonsils), spleen and lymph nodes as well as lymph channels in which fluids in the tissues (lymph) are transported. Among other things, the lymphatic system also forms the white blood cells (lymphocytes). If these grow and grow out of control, one speaks of malignant lymphomas or colloquially lymph node cancer.

Since the lymphatic system runs through the whole body, malignant lymphomas can also arise almost anywhere. Lymph nodes, lungs, liver, bone marrow and spleen are particularly often affected. Malignant lymphomas include around 40 different and sometimes very rare cancers. The most common malignant lymphomas are divided into three large groups:

  • Hodgkin's lymphoma: So-called Reed-Sternberg giant cells can be detected in Hodgkin's disease. Around 2,400 people in Germany develop this rare cancer each year. The lymphoma can appear at any age. Around one in ten sufferers is under 20 years of age.
  • Non-Hodgkin's lymphomas: There are no Reed-Sternberg giant cells in this more common cancer. The risk of these lymphomas increases with age. The average age of the sick is over 70 years. Around 17,000 people develop it every year.
  • Multiple myeloma: With this form, which is also known as plasmacytoma or colloquially as bone marrow cancer, plasma cells incorrectly settle in the bone marrow and multiply there. Around 6,800 new cases occur each year. The average age of those affected is around 70 years.

New information on rare lymphomas

The Malignant Lymphoma Competence Network has now provided new brochures that summarize the findings of lymphoma research over the past 20 years. The experts also provide information on particularly rare forms of malignant lymphoma.

Marginal zone lymphomas

These lymphomas originate from a subset of white blood cells, the B lymphocytes. If the genetic information of B lymphocytes changes by chance, this cancer can develop. How this change occurs has not yet been finally clarified, according to the competence network. Chronic infections, for example with Helicobacter pylori, Chlamydia psitacci or hepatitis C, as well as rheumatic autoimmune diseases such as Sjögren's syndrome or lupus erythematosus, seem to favor the development of marginal zone lymphomas. The disease usually progresses slowly.

Post-Transplant Lymphoma (PTLD)

The form only occurs after organ transplants in combination with immunosuppressive therapies. About two percent of all transplant patients develop this rare lymphoma as a result of a transplant. That is around 50 to 100 new cases each year.

T cell lymphoma

If T lymphocytes change malignantly, one speaks of a T cell lymphoma. There is often a genetic mutation. Since T lymphocytes are part of the immune system, they can multiply and spread freely. In Germany around 800 people develop this rare lymphoma each year.

Mantle cell lymphoma

Mantle cell lymphoma arises when the genetic information of a single lymphatic cell changes incorrectly during cell division. The faulty cell divides again and thus distributes the misinformation. This causes tumors to develop very quickly in the areas where the inactive mantle cell lymphoma cells divide. Around 90 percent of those affected not only develop individual tumors, but several, for example in lymph nodes, bone marrow or in the gastrointestinal tract. There are around 1,000 new cases every year in Germany. On average, the sufferers are 65 years old. (vb)

Author and source information

This text corresponds to the specifications of the medical literature, medical guidelines and current studies and has been checked by medical doctors.

Graduate editor (FH) Volker Blasek


  • Competence Network Malignant Lymphomas e.V .: 20 years of networked lymphoma research: New brochures and leaflets provide information about rare lymphoma diseases (accessed: October 11, 2019),
  • Competence Network Malignant Lymphomas e.V .: Lymphoma Diseases (accessed: October 11, 2019), brochures
  • German Cancer Research Center: Malignant Lymphomas - Warning Signs, Diagnosis and Treatment (accessed: October 11, 2019),

Video: If someone close to you has Hodgkin lymphoma or high-grade non-Hodgkin Lymphoma: A film for children (January 2022).