Lymphogranulomatosis

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lymphogranulomatosis

[‚lim·fə‚gran·yə‚lō·mə′tō·səs]
(medicine)
McGraw-Hill Dictionary of Scientific & Technical Terms, 6E, Copyright © 2003 by The McGraw-Hill Companies, Inc.
The following article is from The Great Soviet Encyclopedia (1979). It might be outdated or ideologically biased.

Lymphogranulomatosis

 

Hodgkin’s disease, a condition characterized by granulomas of the lymph nodes, first described in 1832 by the English physician T. Hodgkin.

The cause of lymphogranulomatosis has not been established. It has been speculated that its origin is viral; the influence of genetic, blastomogenic, or auto-aggressive factors is also possible. The disease may affect persons of any age, but it is chiefly found in the young (under 40). Usually, one group of lymph nodes (axillary, cervical, mediastinal, retroperitoneal, or mesenteric) is affected initially. Gradually spreading, the lymphogranulomatosis then begins to attack all of the lymph nodes, as well as organs, including the lungs, bones, and skin.

In accordance with the generally accepted International Clinical Classification of Lymphogranulomatosis (1965), four stages are distinguished in the course of the disease: the first, or localized, stage involves one or two adjacent regions of nodes; the second, or regional, stage involves two or three nonadjacent regions of nodes on one side of the diaphragm (that is, either in the thoracic cavity or in the abdominal cavity); the third, or generalized, stage involves only the lymphatic system, on both sides of the diaphragm (lymph nodes, spleen, tonsils, Waldeyer’s ring); the fourth, or disseminated, stage involves the entire lymphatic system, in combination with affection of the viscera (lungs, pleura, liver, kidneys, bone marrow, bones), soft tissues, and skin. The affected lymph nodes characteristically contain giant cells, or Berezovskii-Sternberg cells (first described by the Russian physician S. Ia. Berezovskii in 1890).

Lymphogranulomatosis is characterized by periodic fever (sometimes wavelike), general weakness, abundant sweating, itching, phenomena associated with pressure by the enlarged mediastinal lymph nodes on the neighboring organs, cough, dyspnea, dilatation of the veins, and edema of the face and neck. When the retroperitoneal nodes are affected there is also distention of the abdomen, diarrhea, and pain. Anemia and moderate leukocytosis develop, and the hemogram changes. The erythrocyte sedimentation rate (ESR) rises sharply (to 60–80 mm per hour). The course of the disease and its prognosis depend greatly on the form of the disease, the time of diagnosis, and the treatment used.

Treatment is conducted in specialized dispensaries and on-cohematological clinics. Radiation therapy (distance gamma-therapy) entails high-dose irradiation of the foci of the condition and prophylactic irradiation of the lymphatic zones to which the process may spread. Surgical treatment involves removal of the affected focus in combination with irradiation. Antitumoral cytostatic agents, antitumoral antibiotics, and hormonal therapy are also used.

REFERENCES

Kurdybailo, F. V. Limfogranulomatoz, 2nd ed. Leningrad, 1971. (Bibliography, pp. 128–142.)
Kraevskii, N. A., N. M. Nemenova, and M. P. Khokhlova. “Limfo-granulomatoz.” In the collection Patologicheskaia anatomiia i voprosy patogeneza leikozov. Moscow, 1965. Pages 228–236.
Larionov, L. F. Khimioterapiia zlokachestvennykh opukholei. Moscow, 1962.
G. A. ALEKSEEV
The Great Soviet Encyclopedia, 3rd Edition (1970-1979). © 2010 The Gale Group, Inc. All rights reserved.
References in periodicals archive ?
Nodular lymphocyte-predominance Hodgkin disease is an uncommon form of Hodgkin disease that most often presents as asymptomatic lymphadenopathy and follows a relatively indolent course.
While the association of Hodgkin disease with monoclonal gammopathy is rare,[2-6] the association with GHCD is extremely rare.
Cozzolino et al[15] described a 50-year-old woman with a history of nodular sclerosis Hodgkin disease who presented with GHCD, stage 2B.
Within the nodules were numerous Reed-Sternberg cells and their variants in a mixed inflammatory background (Figure, B), characteristic of Hodgkin disease. By immunohistochemical staining, the neoplastic cells were positive for CD15 (Leu-M1) (Becton Dickinson, San Jose, Calif), CD30 (Ki-1), and fascin (Dako Corporation, Carpinteria, Calif) (Figure, C through E).
All these clinical and laboratory findings supported the diagnosis of stage IE primary Hodgkin disease of the terminal ileum with Crohn disease.
He had no evidence of disease at last follow-up, 8 months after the diagnosis of Hodgkin disease.
The syncytial variant of nodular sclerosing Hodgkin disease is an unusual form of Hodgkin disease characterized microscopically by the more typical features of nodular sclerosis, as well as the presence of cohesive aggregates of atypical mononuclear Reed-Sternberg cell variants.
Hodgkin disease is a lymphoma with a bimodal peak incidence occurring in the ages between 15 and 34 years, and 55 years and older.[1] It is one of the most common malignancies to appear in young adults.
Interestingly, immunologic studies have demonstrated that the nodular lymphocyte predominant type of Hodgkin lymphoma is characterized by the presence of polyclonal B lymphocytes, in contrast to the T-cell-rich infiltrates of other types of HD.[5] The RS cells in nodular lymphocyte predominance Hodgkin disease (NLPHD) variant stain positive with antibodies to leukocyte common antigen, L26, and pan-B-cell markers in contrast to the RS cells of other variants, which are Leu-M1 positive and nonreactive with pan-B-cell markers.
Hodgkin disease has been described to occur with NHL at the same site or simultaneously at different sites in a single patient or sequentially following NHL.
The involvement of the spleen by Hodgkin disease, monocyte-macrophage disorders, and plasma cell dyscrasias is well presented and discussed.
Differentiation of TCRBCL from T-cell NHL and Hodgkin disease is critical in this aggressive lymphoma,[2-5] and it should be treated in the same manner as an aggressive NHL.[6]