|Classification according to ICD-10|
|C82||Follicular (nodular) non-Hodgkin lymphoma|
|C83||Diffuse non-Hodgkin lymphoma|
|C84||Peripheral and cutaneous T-cell lymphomas|
|C85||Other and unspecified types of non-Hodgkin lymphoma|
|ICD-10 online (WHO version 2019)|
The malignant lymphomas (colloquially lymphoma , formerly lympho sarcoma ) are a group of malignant diseases of the lymphatic system . These include lymph nodes , adenoids ( tonsils ), spleen and bone marrow . But other organs can also be affected.
The disease is based on a malignant transformation of lymphatic cells at different stages of maturation and differentiation. Depending on which cell type is affected, different types of lymphoma develop, which differ in terms of manifestation, course, therapeutic susceptibility and prognosis . Traditionally, a distinction is made between Hodgkin's lymphoma and the heterogeneous group of non-Hodgkin's lymphomas (NHL). Today, the classification of malignant lymphomas by the World Health Organization is common. In addition to the original cell ( B-lymphocyte or T-lymphocyte ), this differentiates
- Indolent lymphomas (dolor = Latin pain; low grade , this means: slowly growing lymphomas that cause no symptoms for a long time)
- Aggressive lymphomas ( high grade , meaning: fast-growing lymphomas that quickly cause symptoms)
- Hodgkin's disease .
Due to their origin in the lymphatic system , some of the malignant lymphomas can be traced back to disorders of the immune system. With an increasing number of organ transplants and the subsequent necessary immunosuppression and immunodeficiencies in HIV , the incidence (number of new cases) of so-called post-transplant lymphoproliferative diseases has increased. In the case of congenital disorders of the immune system, such as Wiskott-Aldrich syndrome or Severe combined immunodeficiency ( SCID , English for "serious combined immunodeficiency"), increased incidence of lymphomas has been reported. The age peaks in Europe and the USA (not in the rest of the world) are around the age of 30 and 60, and men are affected somewhat more often than women.
In the treatment of malignant lymphomas, therapy with cytostatics ( chemotherapy ) plays a decisive role, sometimes supplemented by radiation therapy . Many lymphoma diseases are thus easily treatable. Some of the lymphomas are curable today.
In the case of lymphomas, a basic distinction is made between Hodgkin lymphomas, which make up around 25% of all lymphomas, and non-Hodgkin lymphomas, which make up around 75% of all lymphomas.
The Hodgkin's lymphoma (HL, Hodgkin's disease) is a malignant, monoclonal B-cell lymphoma. In histology, Sternberg-Reed cells (multinucleated giant cells) and mononuclear Hodgkin cells can usually be detected.
The non-Hodgkin's lymphomas (NHL) are a heterogeneous group of monoclonal neoplasms of progenitor cells of the B- or T-lymphocytes are derived.
- Chronic lymphocytic leukemia
- Plasmacytoma (multiple myeloma)
- Lymphoplasmocytic lymphoma (including Waldenström's disease )
- Follicular lymphoma
- MALT lymphoma
- diffuse large B-cell lymphoma (DLCBL)
- Burkitt lymphoma
- T / NK cell lymphoma
- NK cell leukemia
- T-cell prolymphocytic leukemia
- Mycosis fungoides
- Sézary syndrome
In addition to the lymphatic organs, the skin can also be the site of the disease. According to a classification by the European Organization for Research on Treatment of Cancer (EORTC), T-cell and B-cell lymphomas are differentiated among the primary cutaneous lymphomas and sorted according to the degree of malignancy .
Important T-cell lymphomas of the skin
The term mycosis fungoides is a somewhat misleading name, because there is no connection with fungal diseases ( mycoses ). Mycosis fungoides belongs to the low malignancy category and is the most common cutaneous lymphoma (around ⅓). The origin of the disease has not yet been clarified; connections with viral infections and occupational exposure are being discussed. Mycosis fungoides is characterized by a typical course with three skin stages, which can also be present at the same time before organ involvement occurs:
- Eczema stage: similar to psoriasis or eczema , often itching. The differentiation from parapsoriasis en plaques is often very difficult; the latter can be a precursor of mycosis fungoides.
- Infiltrate stage / plaque stage: severe itching, brownish plaques (vs. reddish infiltrates in stage 1)
- Tumor stage: with bulging, bulbous, z. T. fungal tumors that sit on the infiltrates, high risk of accompanying infections
The course is often chronic for years, complete regression is also possible in earlier stages. After entering the tumor stage, the prognosis is poor. Mycosis fungoides is treated rather cautiously in the early stages with corticosteroids , UV-A radiation and various immunomodulators.
Large cell CD30 + cutaneous T-cell lymphoma
from the group with low malignancy , about ten percent of cutaneous lymphomas, typical of the elderly. The tumor T cells express the antigen CD 30. One usually finds individual tumors and tumor nodules, which often ulcerate ; no extensive skin infestation. The prognosis is good and spontaneous remissions are possible. Therapeutically, tumor nodules are surgically removed, and interferon and immunosuppressants are used. Irradiation with UV-A and X-rays also promise success.
from the group with high malignancy , disease of the elderly, preferably men. Histologically , the same degenerate T cells are found as in mycosis fungoides; these can also be detected in the blood ("Sézary cells"). The reddened skin ( erythroderma ), possibly also hyperpigmentation , severe itching and excessive cornification of the palms and soles are typical . Treatment similar to mycosis fungoides, but with a poor prognosis.
Large cell CD30 cutaneous T-cell lymphoma
from the group with high malignancy . Plaques, lumps and tumors quickly develop on the skin. Later involvement of the entire integument and organs, poor prognosis. Individual herds are treated with radiation, otherwise poly chemotherapy .
Important B-cell lymphomas of the skin
Cutaneous germinal center lymphoma
from the group with low malignancy , about ¼ of all cutaneous lymphomas. Papulo - nodular appearances mainly on the hairy head, in the neck or on the trunk. The prognosis is very good after surgical removal and irradiation, treatment with monoclonal antibodies ( rituximab ) also promises success.
(also marginal zone lymphoma; from Latin margo , dt. 'the edge' - in the lymph nodes the B cells are usually located in the edge areas), from the group of low malignancies . A rather rare lymphoma in adult humans. Brownish-reddish plaques and lumps quickly develop, preferably on the extremities, and patients do not complain of any symptoms. The B cells produce immunoglobulins , but these can rarely be detected in the plasma .
Pathologists and clinicians
- Ingrid Moll: Dermatology. (= Dual row). 6th edition. Georg Thieme Verlag, 2005.
- NL Harris, ES Jaffe, J. Diebold et al .: World Health Organization classification of neoplastic diseases of the hematopoietic and lymphoid tissues: report of the Clinical Advisory Committee meeting-Airlie House . Virginia, November 1997 In: J Clin Oncol . 1999; 17, pp. 3835-3849. PMID 10577857 . (English)
- Competence network "Malignant Lymphoma" and search for treatment centers
- Study Group Indolent Lymphoma Frankfurt am Main
- Study group of low-malignant non-Hodgkin lymphomas
- WHO classification
- ↑ Ackermann H., et al .: Internal Medicine . In: AllEx Compendium of Clinical Medicine . Georg Thieme Verlag, 2014, ISBN 978-3-13-146952-6 , p. 616-628 .