Richter's syndrome: analysis of literature data and original observations
Osmanov, D.Sh.; Kruglova, G.V.; Probatova, N.A.; Kondrat'eva, T.T.; Frenkel', M.A.; Sholokhova, E.N.; Sorokin, E.N.; Dvoretskiĭ, V.V.; Tupitsyn, N.N.
Terapevticheskii Arkhiv 71(7): 47-58
1999
ISSN/ISBN: 0040-3660 PMID: 10481868 Document Number: 506899
Review of literature data and original experience with Richter's syndrome. 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). 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. 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.