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Ter Arkh. 1999;71(7):47-58.

[Richter's syndrome: analysis of literature data and original observations].

[Article in Russian]

Abstract

AIM:

Review of literature data and original experience with Richter's syndrome.

MATERIALS AND METHODS:

250 patients suffering from malignant lymphoproliferative diseases with blood and bone marrow lymphocytosis were observed. 8 (3.2%) of them developed diffuse large-cell lymphoma (criteria and classification of REAL).

RESULTS:

5 of the above 8 patients demonstrated spontaneous regression of lymphocytosis. These cases may illustrate transformation (clonal progression) of one morphological variant of malignant non-Hodgkin's lymphoma into another one, more aggressive. For this rare variant of Richter's syndrome running with regression of lymphocytosis the term Richter-Lortolary syndrome is proposed. Lortolary was the first who revealed a decrease of lymphocytosis in Richter's syndrome. The studies of the genome structure, first of all, of immunoglobulin genes show that in Richter-Lortolary syndrome it is easier, to confirm monoclonality of the two tumors (lymphocytic and large-cell) than to reject it. However, the idea of transformation has not been confirmed morphologically yet.

CONCLUSION:

Development of diffuse large-cell lymphoma in the course of chronic lymphatic tumor does not always indicate terminal state, later stage of tumor progression and poor prognosis.

PMID:
10481868
[Indexed for MEDLINE]
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