Lymphoma gigantopithicus

Lymphoma gigantopithicus (synonym: disease Brill-Simmers, malignant gigantopithicus adenopathy, gigantopithecus limfoblasta, infofortuna sarcoma, lymphoreticular, lymphoma nodular) is a chronic process, characterized by local or systemic increase lymphatic system with hyperplasia his reticular stroma and sharp increase of follicles.
The etiology and pathogenesis of the disease is not clear. The most common view of lymphoma gigantopithicus as the tumor process. There L., people of any age, mainly from 40 to 60 years, more often in men. There are three types of L. G.: ferrous, splenic and glandular splenic. The lymph nodes and spleen are affected more often, but pockets of L. was found in the tonsils, pitchfork spectacle gland, gastrointestinal tract, skin, eye socket, lacrimal gland, lungs. In the course of the disease there are two phases: benign (presentatelnuu) and malignant (sarcomatous). Usually change the start at any one group of lymph nodes (neck, underarm or groin), and for many years the disease is flowing without any clinical manifestations; lymph nodes are mobile, soft, much enlarged, on the cut whitish-pink color.
Microscopically (Fig) revealed sharp hyperplasia follicles (the so-called pseudopolymelia); sometimes they merge and have the form of light reticular cells, on the periphery surrounded by a wall of lymphoid cells. There are the following types of cells that make up the follicles: large hypochromic lymphoblastoid cell type large lymphocytes and transitional cells are small, with core, rich chromatin. On progression of the disease in the second phase appear cells, morphologically immature, with figures mitoses, and lymph nodes palpation become sedentary.
The clinical picture depends on the localization process. In systemic defeat lymphadrainage apparatus there are non-specific changes of blood in the form of moderate hyperchromic anemia, leukopenia Or mild leukocytosis with relative limfotsitoz, monocytosis or monocytopenia. In punctate bone marrow - breeding reticular cells. Ectopic localization lymphoma gigantopithicus can simulate the gut polypus (in the localization in the intestine), appendicular syndrome (lymphoma gigantopithicus in the caecum and in the process), etc., In 1/3 of cases, L. developing splenomegaly. With the defeat of the skin there are phenomena of acanthosis, intra - and extracellular edema. Regional lymph nodes are greatly enhanced, appears dark pigmentation of the skin. Duration of disease - from one year to the next 5-10 years.
Diagnosis L., due to the extremely sparse symptoms can be supplied only by histological examination istechenii lymph nodes.
The prognosis is better than when the system processes like retikulosarkoma or Hodgkin's disease, which is associated first of all with extremely high sensitivity L. g to radiation therapy.
Treatment. Effective chemotherapy: doban, migranal, Endoxan (cyclophosphamide), With actinomycin (kanamycin); in some cases, benefits corticosteroids (40-50 mg prednisolone per day) - alone or in combination with other drugs and radiation therapy.
Radiation therapy Lymphoma gigantopithicus usually brings success, because the tumor has high radiosensitivity in any location. In the treatment of single peripheral nodes use external fractional exposure from various sources (radiotherapy, gamma-therapy, fast electrons) in the dose of 400 to 5000 rub. In this irradiation of the tumor, usually fully rezorbiruetsa and the disease is not recurs within a few years. In generalized forms and transition L., malignant systemic disease apply R (radioactive phosphorus). Single dose - 1 mturi, total - from 6 to 28 mkori. The average survival of patients with this method does not exceed 7 years. At primary defeat of the mediastinal lymph nodes, lungs, stomach, spleen used surgical or radiation treatment. Cm. also Lymphosarcomatous skin.