title: [Richter's syndrome: analysis of literature data and original observations].
abstract: OBJECTIVE
  Review of literature data and original experience with Richter's syndrome.
  
  
  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.
  
  
  CONCLUSIONS
  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.
authors.forename: D Sh
authors.initials: DSh
authors.lastname: Osmanov
authors.forename: G V
authors.initials: GV
authors.lastname: Kruglova
authors.forename: N A
authors.initials: NA
authors.lastname: Probatova
authors.forename: T T
authors.initials: TT
authors.lastname: Kondrat'eva
authors.forename: M A
authors.initials: MA
authors.lastname: Frenkel'
authors.forename: E N
authors.initials: EN
authors.lastname: Sholokhova
authors.forename: E N
authors.initials: EN
authors.lastname: Sorokin
authors.forename: V V
authors.initials: VV
authors.lastname: Dvoretskiĭ
authors.forename: N N
authors.initials: NN
authors.lastname: Tupitsyn
mesh_terms: D005260:Female; D006801:Humans; D016130:Immunophenotyping; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000235:genetics / Q000276:immunology / Q000473:pathology; D008218:Lymphocytosis; D016393:Lymphoma, B-Cell / Q000235:genetics / Q000276:immunology / Q000473:pathology; D016403:Lymphoma, Large B-Cell, Diffuse / Q000235:genetics / Q000276:immunology / Q000473:pathology; D008228:Lymphoma, Non-Hodgkin / Q000235:genetics / Q000276:immunology / Q000473:pathology; D008232:Lymphoproliferative Disorders / Q000235:genetics / Q000276:immunology / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D013577:Syndrome; D009626:Terminology as Topic