Giant-follicular lymphoma (synonym: Brill-Simmers disease, malignant giant-follicular adenopathy, gigantofollicular lymphoblastoma, lympho-follicular sarcoma, lymphoreticulosis, nodular lymphoma) is a chronic process characterized by local or primary clergy, with a pattern, atop of the department, with a pattern
The etiology and pathogenesis of the disease are not clear. The most common view of giant-lymphoma lymphoma as a tumor process. Giant-follicular lymphoma is observed in people of any age, mainly from 40 to 60 years, somewhat more often in men. There are three types of giant-follicular lymphoma: glandular, splenic and glandular-splenic. Lymph nodes and spleen are most often affected, but foci of giant-follicular lymphoma are also found in the tonsils, fork of the spectacle gland, gastrointestinal tract, skin, eye socket, lacrimal gland, and lungs. During the course of the disease, two phases are distinguished: benign (pre-arthritic) and malignant (sarcomatous). Changes usually begin in any one group of lymph nodes (cervical, axillary, or inguinal), and for many years the disease flows without any clinical manifestations; lymph nodes are mobile, soft, significantly enlarged in size, on a cut of whitish-pink color.Go
Microscopically (Fig.) Revealed a sharp follicular hyperplasia (the so-called pseudofollicles); sometimes they merge and have the appearance of light reticular cells, on the periphery of lymphoid cells surrounded by a shaft. The following types of cells that make up the follicles are distinguished: large hypochromic lymphoblastomaceous cells such as large lymphocytes and transitional cells, small, with a nucleus rich in chromatin. As the disease transitions into the second phase, cells appear morphologically immature, with figures of mitoses, and the lymph nodes on palpation become inactive.
The clinical picture depends on the localization process. In case of a systemic lesion of the lymphadenoid apparatus, nonspecific changes in the blood are observed in the form of moderate hyperchromic anemia, leukopenia, or slight leukocytosis with relative lymphocytosis, monocytosis or monocytopenia. In bone marrow punctate there are proliferating reticular cells. The ectopic localization of giant-follicular lymphoma can simulate a polyp of the rectum (when localized in this part of the intestine), appendicular syndrome (giant lymphoma in the cecum and its appendix), etc. In 1/3 of cases of giant-follicular lymphoma splenomegaly develops. With the defeat of the skin, the effects of acanthosis, intra- and extracellular edema are observed. The regional lymph nodes are greatly enlarged, and dark pigmentation of the skin appears. The duration of the disease is from a year to 5-10 years.
The diagnosis of giant lymphoma due to extremely poor symptomatology can only be made by histological examination of excised lymph nodes.
The prognosis is more favorable than with systemic processes such as reticulosarcoma or lymphogranulomatosis, which is primarily due to the extremely high sensitivity of the giant follicular lymphoma to radiation therapy.
Treatment. Effective chemotherapy: dopan, degranol, endoxan (cyclophosphamide), actinomycin C (sanamycin); in some cases, the use of corticosteroids (40-50 mg of prednisone per day) is beneficial - alone or in combination with other drugs and with radiation therapy.
Radiation therapy of the giant-follicular lymphoma usually brings success, because the tumor has a high radiosensitivity at any Localization. In the treatment of single peripheral nodes, external fractional irradiation from various sources (radiotherapy, gamma therapy, fast electrons) is used in doses from 400 to 5000 r. With such irradiation, the tumor is usually completely resorbed and the disease does not recur for several years. In case of generalized forms and the transition of the giant follicular lymphoma to a systemic malignant disease, P 32 (radioactive phosphorus) is used. Single doses - 1 mcuri, total - from 6 to 28 mcuri. The average survival of patients with this method does not exceed 7 years. In the primary lesion of the lymph nodes of the mediastinum, lungs, stomach, spleen, surgical or radiation treatment is used. See also Lymphosarcomatosis of the skin.