Lymphogranulomatosis

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lymphogranulomatosis

[‚lim·fə‚gran·yə‚lō·mə′tō·səs]
(medicine)

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
References in periodicals archive ?
To our knowledge, an association of GHCD with the nodular lymphocyte-predominant form of Hodgkin disease has not been reported previously.
Perhaps more detailed examination of immunoglobulin rearrangements in cases such as ours and the cases reported by Di Benedetto et al[4] and Westin et al[17] may lead to a better understanding of the possible clonal relationship between Hodgkin disease and composite or sequential "non-Hodgkin" B-cell lymphoproliferative processes such as GHCD.
Hodgkin and Reed-Sternberg cells in lymphocyte predominant Hodgkin disease represent clonal populations of germinal center-derived tumor B cells.
Because primary gastrointestinal Hodgkin disease cases are so rare, and those arising in Crohn disease are even rarer, the diagnosis should only be made following strict histologic and other criteria proposed by Dawson et al[10]: (a) no superficial lymphadenopathy should be present at the time of diagnosis; (b) chest radiological studies should indicate no involvement of mediastinal lymph nodes; (c) the complete blood count and white cell differential should be within normal limits; (d) gastrointestinal lesion should predominate with or without positive adjacent lymph nodes; and (e) liver and spleen should be free of disease at the time of diagnosis.
4-6] In 1977, Codling et al[11] reported a case of Hodgkin disease complicating Crohn colitis.
Our case fulfills the criteria of Dawson et al[10] and the immunohistochemical criteria for the diagnosis of primary gastrointestinal Hodgkin disease.
Pathologic Diagnosis: Syncytial Variant of Nodular Sclerosing 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.