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The lymphogranulomatosis represents primary tumoral disease of lymphatic system which is characterized by granulematozny growths with existence of cells of Berezovsky-Shternberg.
The standardized indicators of incidence of a lymphogranulomatosis fluctuate within 0,6 — 3,9 at men and 0,3 — 2,8 at women.
In our country according to selective researches indicators of incidence of a lymphogranulomatosis, HLL, lympho-and reticulosarcomas are almost identical. Among the diseased with a lymphogranulomatosis of the man make 60 — 70%, except for a nodulyarny sclerosis which is diagnosed generally for women. It meets at all age, including newborns, is 3 times more often in families where there were already such patients.
Etiology and pathogeny
The etiology of a lymphogranulomatosis is unknown. Tumoral qualities (an aneuploidy and clonality) inherent in Berezovsky's cell — Shternberg are established. Data on a possible role in development of some lymphoma of a virus of Epstein — Barrel collect. Increase of frequency of a disease among the persons which were exposed to long immune stimulation, receiving immunodepressive therapy is noted and also patients have AIDS.
Allocate 4 main histologic types of a lymphogranulomatosis: lymphoid dominance, nodulyarny sclerosis, smeshannokletochny option, lymphoid exhaustion. Dependence between them and a clinical current of a lymphogranulomatosis is established. Most often the nodulyarny sclerosis and smeshanno-cellular type meet, is more rare — lymphoid dominance and lymphoid exhaustion.
The type of a modular sclerosis is distinguished by tumoral growths of coarse-fibered connecting fabric which tyazh create nodulyarny structures. On structure of growth can be provided by all cellular types of a lymphogranulomatosis. Existence of the so-called "lacunary" cells which are characterized by "empty" cytoplasm wide as if is possible. Allocate a cellular presklerotichesky phase of a nodulyarny sclerosis at which fibrous tyazh are expressed poorly or are absent, lymphocytes and "lacunary" cells prevail. At a nodulyarny sclerosis rather slow progressing of a disease is noted.
The Smeshannokletochny type is characterized by cellular polymorphism: lymphocytes, histiocytes, neutrophils, zozinofila, plasmocytes, a large number of typical cells of Berezovsky — Shternberg meet. Often fields of fibrosis, the necrosis centers are found. This type of a lymphogranulomatosis proceeds less favorably, than previous.
At type with lymphoid dominance and especially lymphoid exhaustion in cytologic drugs of lymph nodes often there are no Berezovsky's cells — Shternberg that demands further histologic verification of the diagnosis. At smeshannokletochny type and a nodulyarny sclerosis in punctates lymphatic, nodes usually these cells find.
The type with lymphoid dominance is characterized by diffusion or focal proliferation of lymphocytes, is frequent in combination with histiocytes. The last are disseminated in the form of single cells, small accumulations, sometimes form small knots like epithelioid hillocks or are located big fields (lympho-histiocytic option with dominance of histiocytes). Berezovsky's cells — Shternberg contain in a small amount therefore serial sections are necessary for their identification. Often Hodzhkin's cells meet. Eosinophils and plasmocytes are usually not numerous. Fibrosis and necroses are absent. This type of a lymphogranulomatosis has an optimum current.
At lymphoid exhaustion sharp reduction of lymphocytes up to their total disappearance is observed. Allocate two options (subtype). At the first, designated as "a diffusion sclerosis" or "diffusion fibrosis, among tyazhy connecting fabric small accumulations of histiocytes, Hodzhkin's cells, typical cells of Berezovsky — Shternberg are visible. At "reticular option" growths consist generally from atypical huge one - and multinucleate cells, typical cells of Berezovsky — Shternberg meet. The type of lymphoid exhaustion is characterized by an adverse current and usually corresponds to the IV stage of distribution of an illness.
Approximate formulation of the diagnosis:
1. A lymphogranulomatosis, a nodulyarny sclerosis with defeat of a cervical lymph node on the one hand (IA a stage).
2. A lymphogranulomatosis, smeshannokletochny option with defeat of cervical, axillary, inguinal lymph nodes (on the one hand) and spleens, fever (II In a stage).
3. A lymphogranulomatosis, lymphoid exhaustion with defeat of peripheral lymph nodes, spleens, night sweats, fever, weight loss (III In a stage).
4. A lymphogranulomatosis, smeshannokletochny option with defeat of peripheral, mediastinal and mezenterialny lymph nodes, marrow, lungs, a liver, kidneys; fever, night sweats, generalized itch of skin (IVB stage).
At 60 — 80% of patients the lymphogranulomatosis usually debuts increase cervical, at 6 — 20% — axillary, at 6 — 12% — inguinal and at 6 — 11% — mediastinal lymph nodes. In process of their growth there are a morbidity and symptoms connected with a prelum of surrounding fabrics and bodies. At 9% of patients process is initially localized in almonds, a spleen, lungs, skin, kidneys, a digestive tract etc. From primary center, mainly on lymphatic ways, metastasises extend to the adjacent and remote groups of lymph nodes.
In peripheral blood at the time of detection of a disease at 1/3 patients anemia is noted, at process generalization it is observed in most cases. In the period of an active phase also neutrophylic leukocytosis at a relative and absolute lymphocytopenia is observed and the accelerated SOE. At damage of marrow in trepanobioptata of an ileal bone typical cells of Berezovsky — Shternberg can sometimes come to light. Periodically arising fever which is replaced normal temperature, the expressed perspiration quite often with preferential certain localization are characteristic (a face and a neck, a front surface of a thorax, etc.).
For an assessment of prevalence of process allocate the following clinical stages:
Stage of I — defeat of one or two adjacent groups of the lymph nodes located on one side of a diaphragm or availability of one extranodal infiltrate (Ie).
Stage of II — defeat of two or more lymph nodes of the non-adjacent groups located on one side of a diaphragm or too in combination with extranodal infiltrate (IIE).
Stage of III — defeat of two or more groups of the lymph nodes located on both sides from a diaphragm is possible availability of extranodal infiltrates (IIIE) and damage of a spleen (IIIS) or existence and that, and other (IIIES).
Stage of IV — defeat of not lymphatic bodies (marrow, a pulmonary parenchyma, a pleura, a liver, kidneys, a digestive tract, etc.) which is combined or is not combined with damage of lymph nodes.
Along with staging of process depending on symptoms of the general intoxication distinguish also forms A and B; And — lack of clinical symptoms; B — existence of the following symptoms (one or several): rise in temperature to 38 °, profuse night sweats, weight loss more than for 10% of body weight for the last 6 months, a generalized itch of skin.
Establishment of a stage of a disease demands detailed inspection of the patient which includes: a biopsy of a lymph node or the extranodal center with the subsequent combined histologic and cytologic studying of prints; physical research, cytomorphological studying of peripheral blood and marrow (breast puncture, trepanobiopsiya of ileal bones); X-ray inspection of a thorax, skeleton, digestive tract; lower limfografiya or ultrasonic research, computer tomography; researches of function of a liver (transaminase, alkaline phosphate for, etc.); according to indications — scanning of a liver and spleen, gastroscopy, a laparoscopy and a puncture biopsy of a liver.
Approximate formulation of the diagnosis:
Lymphogranulomatosis, smeshannokletochiy type, LIPS a stage, with damage of cervical, intrathoracic and retroperitoneal lymph nodes and pulmonary fabric.
Verification of the diagnosis
The lymphogranulomatosis type with dominance of an adenoid tissue should be differentiated, first of all, from the nonspecific changes which are often arising in lymph nodes at different inflammations, intoxications, infectious diseases and also lymphadenites after vaccination at children. At considerable dominance of lymphocytes differentiation with a chronic lymphoid leukosis, a lymphocytic lymphosarcoma is carried out. At a large number of histiocytes it is necessary to exclude with an arch of doses of Beck, toxoplasmosis, allergic granulematozny lymphadenitis. Lymphoid exhaustion with the expressed sclerosis is differentiated from chronic inflammatory changes in fabric of a lymph node. Quite often the lymphogranulomatosis should be differentiated from metastasises of cancer, various options of lymphosarcomas, reactive and tubercular limfoadenopatiya.
At a splenectomy the diagnostic biopsy of a liver and lymph nodes of an abdominal cavity is important for specification of a clinical stage of an illness. In an early stage of damage of a spleen limfogranulematozny growths have the small sizes and are localized on the periphery of lymphoid follicles which often happen giperplazirovana. Damage of a liver meets less often, limfogranulematozny growths originally are located in the field of portal fields; lymphoid infiltrates, changes of hepatocytes, a cholestasia, a hemosiderosis are found. Marrow is surprised at generalization of process or upon its transition to bones from adjacent lymph nodes. At the same time Berezovsky's cells — Shternberg usually contain in a small amount and are not always found.
In the absence of specific damage of marrow increase in number of unripe granulocytes, especially eosinophilic myelocytes, plasmocytes, a delay of maturing of elements of an erythroidal row is observed.