Macrofollicular lymphoma - Practical hematology of children's age

THE ILLNESS SHAVED — SIMMERSA (THE MACROFOLLICULAR LYMPHOMA)
In 1925 M. E. Brill described a disease of lymph nodes under the name "generalized hyperplasia of lymph nodes and spleen". The author carried a disease to high-quality process, considering that inflammatory reaction of lymph nodes is the cornerstone of it. In 1938 D. Symmers on the basis of comparative morphological research at this syndrome and malignant lymphoma suggested about malignant character of a disease. On modern representations, Brill's illness — Simmersa treats tumoral malignant defeats of lymphatic fabric. In the explanation to the international WHO classification (1976) it is specified that macrofollicular sarcoma of Brill — Simmersa corresponds to a nodulyarny lymphosarcoma and has the V-cellular origin. Nodulyarny character of a tumor defines a long high-quality current. At the same time tumoral process has two accurate phases which are usually characteristic of hemoblastoses: high-quality and malignant sarkomatozny transformation.
The etiopathogenesis of a macrofollicular lymphoma is up to the end not clear. Assume that reorganization of an adenoid tissue is caused by change of an immune responsiveness of an organism as a result of influence of infectious factors. On supervision of L. A. Durnov and coauthors, in the anamnesis sick children have frequent respiratory infections, long infectious diseases (measles, parotitis, whooping cough). The histologic picture of a lymph node is characterized by existence of large huge follicles with a clear boundary, they are located in cortical and a medulla of a node. The centers of follicles of a giperplazirovana are also executed by lymphoblastoid cells, there are mitosis figures. Lymphoid elements in the center of a follicle have the monoclonal nature allowing to regard them as tumor cells. On the periphery of a follicle there is a rim from the lymphocytes having the polyclonal nature, that is being not tumor cells. Sine of a lymph node narrow, cells in a sine not of a giperplazirovana. Around follicles the dense network of reticular fibers is noted. Upon transition to a malignant phase drawing of a lymph node is erased, a nodulyarny or diffusion infiltration by lymphoblasts, a large number of figures of a mitosis, a bigger increase in the huge follicles merging among themselves are noted. In places separate follicles break in sine. The similar picture at the first and second stage of a disease is observed in a spleen.
The clinical picture of an illness of Brill — Simmersa depends on localization of tumoral process, extent of generalization and dissimination. In clinical disease of Brill — Simmers, as well as at all malignant lymphoma, is allocated by 4 stages: The I stage (localized) — defeat of one or two adjacent groups of lymph nodes.

1. stage (regional) — defeat of two or more non-adjacent groups of the lymph nodes located on one side of a diaphragm.
2. stage (generalized) — defeat of various groups of the lymph nodes located on both sides of a diaphragm. The IV stage (disseminated) — involvement in process of visceral bodies.

Also allocate existence (B) or lack (A) of the general symptoms of intoxication.
The main clinical sign — a local hyperadenosis. Unlike reactive lymphadenitis of a polyadenia, as a rule, it is not observed. Peripheral lymph nodes dense, painless, not soldered among themselves and surrounding fabrics, mobile. Skin over them is not changed. L. A. Durnov and coauthors (1979) provide the following data on the frequency of primary defeat of various groups of lymph nodes at the children having Brill's illness — Simmersa — on the first place there is an area of a neck, on the second — axillary lymph nodes, follow further inguinal and submaxillary.
Primary localization seldom meets in mediastinal lymph nodes. In these cases thorax pain, cough, an asthma are noted. In isolated cases there is primary defeat of lymph nodes of an abdominal cavity which is followed by a non-constant abdominal pain. As a rule, the hyperadenosis of a mediastinum and an abdominal cavity demonstrates process generalization. Local increase in peripheral lymph nodes a long time proceeds is good-quality, remaining isolated and not extending to other groups. Primary defeat of mediastinal lymph nodes is also characterized by slow progressing of process. Children remain active, symptoms of intoxication are absent. They appear in III and IV stages of an illness. The condition of children quite often worsens, decreases appetite, pallor, blue under eyes, subfebrile condition appear. Many children even at process generalization can are absent or have poorly expressed symptoms of intoxication. At the same time the spleen often is surprised. The splenomegaly has moderate character, at a palpation a spleen dense, painless. At a number of patients increase in a liver is noted. At dissimination of process damage of lungs, a digestive tract is possible.
In peripheral blood there are no special changes. Are sometimes noted a leukocytosis with a neutrocytosis, increase in SOE. Miyelogramma without features.
The forecast in many respects depends on timely diagnosis and purpose of adequate therapy. The illness slowly progresses for 3 — 6 and at timely begun treatment in the I—II stage it is possible to achieve long permanent remission. The positive take of therapy is possible also at III And a stage. Much worse the forecast at patients with the III B and the IV stage of a disease though the combination therapy allows to stabilize their state.
Differential diagnosis of an illness of Brill — Simmersa, despite accurately outlined histologic picture, presents certain difficulties. Usually this process is differentiated with a lymphogranulomatosis and lymphadenitis. Crucial importance has research of a biopsirovanny lymph node. According to I. A. Kassirsky (1970), safety of drawing of a lymph node can serve as the reason of a diagnostic mistake when changes regard as inflammatory and reactive reaction. A distinguishing character of an illness of Brill — Simmersa is existence of huge follicles. After histologic verification of the diagnosis it is necessary to establish an illness stage. For this purpose use radiological methods of research of a thorax, bones, the lower limfografiya, radionuclide methods for an assessment of parenchymatous bodies, lymph nodes, kidneys. According to indications carry out an eksplorativny laparotomy with a splenectomy.
At the I stage of an illness of Brill — Simmers a tumor is deleted in the operational way and appoint radiation according to the radical program. High sensitivity to radiation therapy therefore the radical program is also effective at II and III And stages is noted. At the III B and the IV stages carry out polychemotherapy according to the TsOP, TsOPP, MOPP program.
At a serious condition of patients use symptomatic, disintoxication therapy, antibiotics for treatment of secondary complications, the guarding mode.