Follicular lymphoma
| Classification according to ICD-10 | |
|---|---|
| C82 | Follicular (nodular) non-Hodgkin lymphoma ICD-O M9690 / 3 ICD-O M9691 / 3 (Grade II) ICD-O M9695 / 3 (Grade I) ICD-O M9698 / 3 (Grade III) |
| ICD-10 online (WHO version 2019) | |
The Follicular lymphoma (or Follikelzentrumslymphom or follicular germinal center lymphoma , sometimes abbreviated FCL or FL ., English follicular lymphoma or follicle center lymphoma ) is a malignant lymphoma and is one of the B-cell - non-Hodgkin's lymphoma (B-NHL).
Frequency, clinic, causes
Overall, the FCL is a rare disease with around 6000 to 8000 diseases per year throughout Germany . However, it is the most common of all low-grade non-Hodgkin lymphomas and accounts for around 20–35% of all NHLs. It occurs mainly in older adults (the mean age of onset is around 60 years) and more frequently in men. Clinically, there are lymph node swellings , but other lymphatic organs such as the spleen or the lymphatic tissue in the throat or in the gastrointestinal tract can also be affected. Often there is also a more or less pronounced bone marrow involvement by the FL. Most patients do not have any major symptoms at the time of diagnosis, only lymph node swelling, which is not painful (as is the case with an acute bacterial infection, for example ). One speaks therefore of an indolent lymphoma (literally: "not painful", but what is meant is: a lymphoma that does not cause any major complaints). Sometimes, however, there are complaints, e.g. B. when swollen lymph nodes compress blood vessels , press on nerve cords and thereby cause pain or neurological failures or when higher-grade bone marrow involvement leads to blood formation disorders or immunodeficiency with a tendency to infection.
The exact causes of the FL are not known. Virtually all cases have a chromosome translocation t (14; 18) (q32; q21). As a result of this chromosome translocation , the BCL2 gene on chromosome 18 is transferred to the vicinity of the immunoglobulin heavy chain locus on chromosome 14 . This leads to a dysregulation of BCL2 and the gene is activated permanently. Since BCL2 normally plays an important role in the apoptosis of B cells , this is disturbed and the affected cells continue to multiply uninhibited and thus become tumor cells . This chromosome translocation is not hereditary, but "accidentally" occurred in the course of life through a "molecular accident". It is therefore usually not possible to clearly justify why FL occurs in a person in individual cases. One speaks of a "sporadic" occurrence of lymphoma.
Diagnosis
A lymph node removal with a subsequent histological examination is usually necessary for a reliable diagnosis . The assessment of the lymph node should always (or possibly additionally) in a reference center for lymph node pathology, i. H. a pathological institute with extensive experience in the classification of NHLs. Once the diagnosis is confirmed, staging must be performed; H. imaging methods ( CT , MRT , sonography , x-ray ) must be used to determine which lymph node stations are affected. A bone marrow aspiration must also be done to determine whether the disease is also affecting the bone marrow. This allows the degree of spread or the stage of the lymphoma to be determined. The staging is important because it provides prognostic information and can be used to decide whether and how the FCL should be treated.
histology
The FL corresponds histologically to the centroblastic-centrocytic (cb / cc) lymphoma of the Kiel classification. In the WHO classification of lymphomas, three "grades" of FL are distinguished. This grading takes into account the proportion of immature cells (centroblasts) and the growth pattern of the FL (predominantly follicular or predominantly diffuse).
| Graduation ( grading ) | definition |
|---|---|
| Grade 1 | 0–5 centroblasts per visual field * |
| Grade 2 | 6–15 centroblasts per visual field |
| Grade 3 | > 15 centroblasts per visual field |
* A (microscopic) field of view is defined as the area (0.195 mm 2 ) visible with a 40x objective and 18 mm eyepiece . At least 10 different fields of view should be viewed for correct assessment.
| Growth pattern | designation |
|---|---|
| > 75% follicular | follicular |
| 25-75% follicular | follicular and diffuse |
| <25% follicular | focal follicular |
| 0% follicular | diffuse |
Areas with> 15 centroblasts per visual field and a diffuse growth pattern are referred to as " Diffuse large-cell B-Non-Hodgkin lymphoma with follicular lymphoma (grade 1, 2 or 3) " according to the WHO classification .
In simple terms, it can be said: the lower the centroblast proportion and the more follicular growth pattern, the lower the malignancy of the FCL, and vice versa: the higher the centroblast proportion and the more diffuse the growth pattern, the more malignant the FCL. A grade 1 FCL is more similar to a low malignant NHL such as chronic lymphocytic leukemia , while a grade 3 FCL is more like a highly malignant NHL and needs treatment.
Staging, principles of treatment
The staging is based on the Ann-Arbor system . When the diagnosis is made, the stage is often at an advanced stage. In such a widespread stage, the FL can usually no longer be completely cured, but a slowed progression of the disease can also be achieved here.
Basically, the FL in the higher stage (like the other indolent lymphomas) is only treated if it causes symptoms to the patient. In fact, there is no evidence that previous treatment will benefit the patient, e.g. B. extended its survival time. Complaints can be:
- Large lymph node problems (displacement problems), e.g. B .:
- Lymph nodes on venous vessels press, making it to the venous thrombosis occurs
- Lymph nodes that press on the ureters so that the urine no longer flows out of the kidney properly ( urinary congestion )
- Lymph nodes draining the bile ducts press, causing it to Gallenaufstau comes.
- Severe disruption of blood formation due to a high degree of bone marrow involvement by the lymphoma
- Immune weakness with a strong tendency to infection (usually associated with a high degree of bone marrow involvement by the lymphoma)
- Pain, e.g. B. Press lymph nodes on nerves
- unspecific so-called "general symptoms" ( B symptoms ): unclear fever, heavy night sweats or unwanted major weight loss.
- Large lymph node problems (displacement problems), e.g. B .:
Treatment is by means of chemotherapy and possibly also (if there are localized problems such as space-occupying lymph node packages) with local radiation therapy . Allogeneic bone marrow or stem cell transplantation is only a possible treatment option in a minority of cases. Typical chemotherapy regimens are:
- CVP ( cyclophosphamide , vincristine , prednisone )
- Bendamustine
- CHOP (cyclophosphamide, vincristine, prednisone, adriamycin )
- FC ( fludarabine , cyclophosphamide)
u. a.
All modern chemotherapy regimens also contain the monoclonal antibody rituximab (e.g. R-CHOP). Treatment should always be carried out within the framework of clinical studies whenever possible . This is the only way to achieve further improvements in the treatment of patients with this disease. In addition, the quality of the treatment according to the latest standards is better guaranteed.
Individual evidence
- ↑ a b S. H. Swerdlow, E. Campo, NL Harris, ES Jaffe, SA Pileri, H. Stein, J. Thiele, JW Vardiman (eds.): WHO Classification of Tumors of Haematopoietic and Lymphoid Tissues, Fourth Edition . IARC Press, Lyon 2008, ISBN 978-92-832-2431-0 , p. 220: Follicular Lymphoma.
Web links
literature
- Elaine S. Jaffe, Nancy Lee Harris, Harald Stein, James W. Vardiman (Eds.): Pathology and Genetics: Tumours of Haematopoietic and Lymphoid Tissues . World Health Organization Classification of Tumors. IARC Press, 2001.
