Follicular lymphoma
The Follicular Lymphoma (or Follikelzentrumslymphom or follicular germinal center lymphoma, sometimes abbreviated FCL or FL, Eng. Follicular lymphoma or follicle center lymphoma ) is a malignant lymphoma and one of the B- cell non -Hodgkin's lymphoma ( B- NHL).
Frequency, clinical manifestations, causes
The FCL is a rare disease with a total of approximately 6,000 to 8,000 cases per year in Germany. However, it is the most common of all low -grade non -Hodgkin's lymphoma, accounting for approximately 20-35 % of all NHLs from. It occurs most frequently in older adults ( the average age at diagnosis is about 60 years ) and more frequently in men. Clinically, show lymph node swelling, but other lymphatic organs such as the spleen or lymphatic tissue in the throat or in the gastrointestinal tract may be affected. Often a more or less pronounced bone marrow involvement by FL is available. Most patients have no major complaints, only lymph node swelling, but they are not painful (such as in an acute bacterial infection) at diagnosis. We therefore speak of an indolent lymphoma ( literally: " not painful " meaning but: a lymphoma that no main complaints). Sometimes, however, there are problems, for example, if swollen lymph nodes compress blood vessels press on nerves, causing pain or neurological deficits or when it comes through the higher grade of the bone marrow to blood formation disorders or immunodeficiency with susceptibility to infections.
The exact causes of the FL are not known. Virtually all cases have a chromosomal translocation t (14, 18 ) ( q32, q21 ) to. As a result of 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. As BCL2 normally plays an important role in apoptosis of B-cells, this is disturbed and the affected cells proliferate and thus unimpededly to tumor cells. This chromosomal translocation is not inherited, but in the course of life " by accident " occurred through a "molecular accident". Why a FL in a single human case occurs is, therefore, not be clearly justified in the rule. One speaks of a " sporadic " occurrence of lymphoma.
Diagnosis
For reliable diagnosis of lymph node removal with subsequent histological examination is usually required. The assessment of the lymph node should ( optionally in addition respectively ) are always in a reference center for lymph node pathology, that is, a pathological institute with great experience in the classification of NHLs. When the diagnosis has been confirmed, a staging must be done, that is, using imaging methods (CT, MRI, ultrasound, x-ray ) should be investigated which lymph nodes are affected. Also, must be a bone marrow biopsy to determine whether the bone marrow is affected by the disease. Thus, the degree of spreading and the stage of the lymphoma is defined. The stadiums definition is important, as this prognostic information is obtained and it can be decided whether and how the FCL must 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 " degrees " of the FL can be distinguished. This grading takes into account the proportion of immature cells ( centroblasts ) and the growth pattern of FL ( predominantly follicular or predominantly diffuse).
* A ( microscopic ) field is defined as that with a 40x lens and 18 mm eyepiece visible area ( 0.195 mm2). Should be considered at least 10 different fields of view for the correct assessment.
Areas with > 15 centroblasts per high power field and diffuse growth patterns are set according to the WHO classification as " diffuse large cell B- non-Hodgkin lymphoma with follicular lymphoma (grade 1, 2 or 3)".
Simplified one can say: the smaller the Zentroblastenanteil and the more follicular growth pattern, the low - malignant is the FCL, and vice versa: the higher the Zentroblastenanteil and more diffuse growth pattern, the more malignant is the FCL. A FCL Grade 1 so is more like a low-grade NHL such as chronic lymphocytic leukemia, while a grade 3 FCL rather a highly malignant NHL and corresponds rather to be treated.
Staging, principles of treatment
Staging is performed according to the system of Ann Arbor. When diagnosis is often before an unfolded state. In such a spread-out stage, the FL rule no longer be cured completely, but slowed progression of the disease is also accessible here.
Basically, the FL in the higher stage (as well as other indolent lymphomas ) is only treated when it prepares the patient discomfort. Because there is no evidence that earlier treatment benefits the patient something that prolongs its survival, for example. Complaints can be:
- Caused by large lymph nodes ( displacement problems ), eg: Lymph nodes, the press venous vessels so that comes to venous thrombosis
- Lymph nodes pressing on the ureters, so that the urine from the kidney no longer drains properly ( urinary retention )
- Lymph nodes pressing on the draining bile ducts, so that it comes to Gallenaufstau.
Treatment is with chemotherapy and possibly (if localized problems such as space-occupying lymph nodes packages consist ) with local radiotherapy. Allogeneic bone marrow or stem cell transplantation occurs only in a minority of cases as a treatment option in question. Typical chemotherapy regimens are:
- CVP (cyclophosphamide, vincristine, prednisone)
- Bendamustine
- CHOP (cyclophosphamide, vincristine, prednisone, Adriamycin)
- FC ( fludarabine, cyclophosphamide)
And Others
All modern chemotherapy regimens also include the monoclonal antibody rituximab (eg R- CHOP). Treatment should in principle always be possible if in the context of clinical trials. The only way to further improvements in the treatment of patients achieve with this disease. In addition, ensure that the quality of the treatment according to the latest standards is better ensured.