Lymphoma

The malignant lymphomas ( colloquially lymphoma, formerly lymphosarcoma ) are a group of malignant diseases of the lymphatic system. These include lymph nodes, tonsils ( tonsils ), spleen and bone marrow. However, other organs may be affected.

• 2.2.1 Cutaneous follicle center lymphoma
• 2.2.2 immunocytoma

Causes

The disease is due to a malignant transformation of lymphoid cells of different maturation and differentiation stages. Depending on which type of cell is affected, gives rise to various types of lymphoma, which differ in the manifestation, the course, the therapeutic intervention and prognosis. Traditionally, a distinction is made between Hodgkin 's lymphoma and the heterogeneous group of non-Hodgkin's lymphoma ( NHL). Nowadays binding is the classification of malignant lymphoma by the World Health Organization. This differs in addition to the cell of origin (B- lymphocyte or T- lymphocyte )

Due to their origin from the lymphatic system, a part of malignant lymphoma is due to disorders of the immune system. With an increasing number of organ transplants and the necessary after immunosuppression and immunodeficiency in HIV incidence (number of new cases ) has increased diseases of the so-called post-transplant Lymphoproliferative. Also in congenital disorders of the immune system such as the Wiskott -Aldrich syndrome, or Severe combined immunodeficiency (SCID, English for " severe combined immunodeficiency " ) reported increased incidence of lymphoma. The peak age in Europe and the U.S. (not in the remaining parts of the world ) are around 30 and 60 years of age, men are slightly more likely than women affected.

In the treatment of malignant lymphoma therapy plays with cytostatic drugs (chemotherapy ) play a key role, sometimes supplemented by radiotherapy. So many diseases are treatable lymphoma. Part of the lymphoma is curable today.

Lymphomas of the Skin

In addition to the lymphoid organs and the skin may be the location of the disease process. After a classification of the European Organisation for Research on Treatment of Cancer (EORTC ) T- cell and B -cell lymphomas can be distinguished among the primary cutaneous lymphomas and sorted according to the degree of malignancy.

Important T -cell lymphomas of the skin

Mycosis fungoides

The term mycosis fungoides is a somewhat misleading name, because there is no connection with fungal diseases ( mycoses). Mycosis fungoides belongs to the category low malignancy and is the most common cutaneous lymphoma ( about ⅓ ). The pathogenesis has not been elucidated, relationships with viral infections and occupational exposures are discussed. Mycosis fungoides is characterized by a typical course with three stages of skin that may be present simultaneously before an organ involvement occurs:

The course is often chronic for years, complete regressions are also possible in earlier stages. After admission to the tumor stage, the prognosis is poor. Mycosis fungoides is treated in the early stages rather cautious with corticosteroids, UV - A irradiation and various immune modulators.

Large-cell CD30 cutaneous T -cell lymphoma

From the group of low malignancy, about ten percent of cutaneous lymphomas, typical of old people. Tumor T- cells express the CD30 antigen. It usually takes individual tumors and tumor nodules that ulcerate often; no large-scale infestation of the skin. The prognosis is good, spontaneous remissions possible. Therapeutic tumor nodules are surgically removed, interferon, and immunosuppressants used. Irradiation with UV-A as well as X-rays also promises success.

Sezary Syndrome

From the group with high malignancy, disease of the elderly, preferably men. Histologically, the same degenerate T cells as in mycosis fungoides, these are also detectable in the blood ( " Sézary cells"). Typical are still heavily reddened skin ( erythroderma ), possibly also hyperpigmentation, severe itching and excessive keratinization of the palms and soles. Treatment similar to that of mycosis fungoides, but poor prognosis.

Large-cell CD30- cutaneous T -cell lymphoma

From the group with high malignancy. It quickly emerge plaques, nodules and tumors on the skin. Later infestation of the entire skin and organ involvement, poor prognosis. Is treated in individual herds with radiation, or chemotherapy.

Important B -cell lymphomas of the skin

Cutaneous follicle center lymphoma

From the group of low malignancy, about ¼ of all cutaneous lymphomas. Papular - nodular appearances, especially on the scalp, neck, or trunk. The prognosis after surgical removal and radiation very well, treatment with monoclonal antibodies ( rituximab) also promises success.

Immunocytoma

(also marginal zone lymphoma; margo, lat the edge - in the lymph nodes sit the B- cells usually in the peripheral areas ), from the group of low malignancy. A rather rare lymphoma of the adult human. It quickly emerge brownish -red plaques and nodules, preferably at the extremities, the patient does not complain of discomfort. Although the B cells produce immunoglobulins, but these are rare in plasma demonstrated. The prognosis is excellent, the lymphoma is very sensitive to the irradiation with fast electrons.