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

• Published in: Terapevtic̆eskii arhiv Vol. 71; no. 7; p. 47
• Main Authors: 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
• Format: Journal Article
• Language: Russian
• Published: Russia (Federation) 1999
• Subjects: Female; Humans; Immunophenotyping; Leukemia, Lymphocytic, Chronic, B-Cell - genetics; Leukemia, Lymphocytic, Chronic, B-Cell - immunology; Leukemia, Lymphocytic, Chronic, B-Cell - pathology; Lymphocytosis; Lymphoma, B-Cell - genetics; Lymphoma, B-Cell - immunology; Lymphoma, B-Cell - pathology; Lymphoma, Large B-Cell, Diffuse - genetics; Lymphoma, Large B-Cell, Diffuse - immunology; Lymphoma, Large B-Cell, Diffuse - pathology; Lymphoma, Non-Hodgkin - genetics; Lymphoma, Non-Hodgkin - immunology; Lymphoma, Non-Hodgkin - pathology; Lymphoproliferative Disorders - genetics; Lymphoproliferative Disorders - immunology; Lymphoproliferative Disorders - pathology; Male; Middle Aged; Syndrome; Terminology as Topic
• ISSN: 0040-3660

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.