Mantle cell lymphoma
| Classification according to ICD-10 | |
|---|---|
| C83.1 | Mantle cell lymphoma
Including: Malignant lymphomatous polyposis, centrocytic lymphoma ICD-10-GM-2018 |
| ICD-10 online (WHO version 2019) | |
The mantle cell lymphoma (. MCL, English mantle cell lymphoma ) is a malignant lymphoma and is one of the B-cell - non-Hodgkin's lymphoma .
nomenclature
Mantle cell lymphoma takes its name from the characteristic appearance ( cytomorphology ) of the lymphoma cells , which look similar to the (healthy) cells that are normally found in the mantle zone of the lymph nodes. The older term centrocytic lymphoma is practically congruent with mantle cell lymphoma.
Epidemiology
Mantle cell lymphoma is a rare disease. Its incidence is 2 / 100,000 per year, affecting four times as many men as women. The average age of onset is 60 years.
pathology
Mantle cell lymphoma is characterized by the reciprocal translocation t (11; 14) (q13; q32) between chromosomes 11 and 14, which increases the expression of cyclin D1 . This triggers the cell cycle and thus increases cell proliferation.
clinic
The disease is typically associated with swelling of the lymph nodes and enlargement of the spleen as the MCL cells accumulate in lymphoid tissue. The disease also often affects the bone marrow , where the MCL cells can displace the blood-forming cells. This manifests itself in anemia (lack of red blood cells), which leads to tiredness and decreased performance, leukopenia (lack of white blood cells), which increases the susceptibility to infections, and thrombopenia (lack of blood platelets), which increases the risk of bleeding . If there is a high degree of infiltration of the bone marrow by the mantle cell lymphoma, the MCL cells can also be found in the blood . There may also be so-called B symptoms (fever, night sweats and weight loss). The digestive tract ( gastrointestinal tract ) is also often affected by the disease (e.g. infiltration of the stomach or intestinal wall ).
diagnosis
To diagnose mantle cell lymphoma, an enlarged lymph node is surgically removed and examined histologically, immunohistochemically and genetically. The extent of bone marrow involvement is determined using bone marrow aspiration. To prove that the cells are MCL cells, the translocation t (11; 14) (q13; q32), which is pathognomonic for mantle cell lymphoma, is detected in the cells by means of FISH (fluorescence in situ hybridization) analysis. Alternatively, cyclin D1 overexpression in the cells can be detected by immunohistochemistry. The expression of CD5 is also characteristic of MCL cells, but in contrast to the likewise CD5-positive CLL cells, MCL cells are CD23-negative. To determine the prognostically relevant growth rate of the MCL cells, the Ki-67 rate is also recorded.
In order to determine the spread of MCL cells to other parts of the body (staging), computed tomography of the neck, chest, abdomen and pelvis is performed.
therapy
Intensive immunochemotherapy is sought for patients younger than 65 years. Established therapy options are R-CHOP / DHAP (a combined therapy with the anti-CD20 antibody rituximab and the chemotherapeutic agents cyclophosphamide, (hydroxy) doxorubicin, vincristine (also known as oncovin) and prednisone, alternating with rituximab, dexamethasone, cytarabine and cisplatin) with subsequent autologous stem cell transplantation, or R-Hyper-CVAD (rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone alternating with methotrexate and cytarabine).
In older patients over 65 years of age, less intensive therapy with BR (the chemotherapeutic agent bendamustine and rituximab) or with R-CHOP (rituximab in combination with the chemotherapeutic agents cyclophosphamide, (hydroxy) doxorubicin, vincristine (also known as Oncovin) and prednisone). Maintenance therapy with rituximab follows.
A novel targeted medicine is Ibrutinib (Imbruvica) that since 13 November 2013 by the FDA , and since 23 October 2014 by the European Commission is approved for the treatment of mantle cell lymphoma in the United States or in the European Union. Other drugs are being tested in clinical trials.
forecast
Mantle cell lymphoma has the most unfavorable prognosis among non-Hodgkin lymphomas (NHL), as it shows the rapid growth of aggressive NHL and, like indolent NHL, is generally not curative. The prognosis of mantle cell lymphoma depends on individual tumor factors. To better assess the prognosis, mantle cell lymphoma is therefore divided into three different risk groups using the MIPI (MCL International Prognostic Index). The MIPI takes into account the factors age, general condition of the patient according to the ECOG score , the level of the LDH value and the number of leukocytes in the blood.
Individual evidence
- ↑ a b c Herold, Gerd .: Internal Medicine: a lecture-oriented presentation: taking into account the subject catalog for the medical examination: with ICD 10 key in the text and index . 2016th edition. Herold, Cologne 2016, ISBN 978-3-9814660-5-8 .
- ^ Siegenthaler, Blum: Klinische Pathophysiologie , 9th edition, Thieme 2006; ISBN 3-13-449609-7
- ↑ Margit Klier: Influencing the expression of cyclin D1 in mantle cell lymphoma cell lines by lentivirally produced siRNA .
- ↑ a b c Martin Dreyling: Mantle Cell Lymphoma: Biology, Clinical Presentation, and Therapeutic Approaches . In: American Society of Clinical Oncology Educational Book . tape 34 , p. 191–198 , doi : 10.14694 / edbook_am.2014.34.191 ( asco.org [accessed on February 22, 2018]).
- ^ Andre Goy: Are we improving the survival of patients with mantle cell lymphoma: if so, what is the explanation? . In: Leukemia & Lymphoma 2011 Oct; 52 (10): 1828-30. . August.
- ↑ Ibrutinib Has 'Unprecedented' Impact On Mantle Cell Lymphoma. Announcement at Sciencedaily.com dated December 14, 2012.
- ↑ FDA approves Imbruvica for rare blood cancer. US Department of Health and Human Services, November 13, 2013, accessed November 13, 2013 .
- ^ Community register of medicinal products for human use: Imbruvica. European Commission, 23 October 2014, accessed on 24 November 2014 .
