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.
Subject(s)
Lymphocytosis , Lymphoproliferative Disorders/pathology , Female , Humans , Immunophenotyping , Leukemia, Lymphocytic, Chronic, B-Cell/genetics , Leukemia, Lymphocytic, Chronic, B-Cell/immunology , Leukemia, Lymphocytic, Chronic, B-Cell/pathology , 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 , Male , Middle Aged , Syndrome , Terminology as TopicABSTRACT
Gangliosides of normal human blood serum and of blood serum of tumour hosts were studied. It has been shown the first time that ganglioside (NeuAc)2LacCer is present in the blood serum of different tumour hosts, but is absent in normal blood serum. The reasons for the appearance of this ganglioside in blood serum are discussed.
Subject(s)
Gangliosides/blood , Neoplasms/blood , Chromatography, Thin Layer , Gangliosides/analysis , Humans , Immunoenzyme Techniques , Neoplasms/analysisABSTRACT
Lipid-bound sialic acids were estimated in blood serum of 118 patients with tumors, of 13 donors and of 38 patients with non-tumoral diseases. Content of the sialic acids in blood serum of the patients with growing tumors constituted 23.1 +/- 1.1 mg/100 ml, which was 2-2.5-fold higher as compared with healthy donors (10.5 +/- 0.4 mg/100 ml) or with the patients with non-tumoral diseases (13.8 +/- 0.5 mg/100 ml). In the oncologic patients during the remission period the content of lipid-bound sialic acids in blood serum approached the normal level - 12.1 +/- 0.5 mg/100 ml. The highest level of the sialic acids was found in patients with lymphogranulomatosis II-IV step, with melanoma during the period of growth, with kidney tumor and with liver tissue primary tumor. Estimation of circulating lipid-bound sialic acids in the oncologic patients may serve as a test for evaluation of the tumor growth as well as of therapeutic treatment efficiency.
