Combined morphoimmunological studies of tumor elements with a broad panel of monoclonal antibodies including activation antibody Ki-1(CD-30) enabled us to single out large-cell anaplastic lymphomas (LCAL) from a group of non-Hodgkin's lymphomas. Morphological features of the tumor elements and their similarity with large-cell sarcomas of different genesis (sarcoma of soft tissues, primitive neuroectodermal tumors, histiocytic tumors, etc.) dictate the necessity to conduct cytoimmunological differential diagnosis. 23 children were examined. four types of LCAL were identified: 1. LCAL-Ki1+(CD30+), 2.LCAL-Ki1+B-cell (CD30+CD19+CD20+), 3. LCAL-B-cell (CD19+CD20+CD22+), 4. LCAL-T-cell (CD7+CD4+CD5+). These LCAL types were diagnosed in 8, 5, 6 and 4 children, respectively. The types 1-3 have similar clinical manifestations: severe aggressiveness of the process, rapid dissemination, involvement of different lymph nodes, soft tissues, bones with destruction. Specific alterations in the bone marrow and CNS were not registered. T-cell phenotype is less aggressive, more frequent involvement of mediastinal lymphatic tissue and bone marrow. Bones are not involved. There are no definite and persistent morphological differences in well anaplastic tumor elements in different LCAL. This makes almost impossible to verify the tumor type. Monoclonal antibodies indicate immunological trend in differentiation of the tumor substrate elements which facilitates choice of adequate treatment schemes the latter being markedly different from standard schemes of treating other non-Hodgkin's lymphomas.
Differential diagnosis was established by complex morphological examination in 290 pediatric patients with Langerhans cell, sinus or malignant histiocytosis. An immunologic study was performed in one-third of patients. Clinical symptoms and results of therapy are presented.
Современная эндоскопия представлена разнообразными методами, которые могут применяться как изолированно один от другого, так и в виде комплексного эндоскопического исследования. Эндоскопическая диагностика лимфосаркомы у детей должна быть комплексной: при подозрении на лимфосаркому абдоминальной локализации должны выполняться фиброгастроскопия, колоноскопия и лапароскопия (по показаниям). Комплексная эндоскопическая диагностика позволяет как идентифицировать локализацию первичного поражения, так и объективно оценивать распространенность лимфосаркомы у детей, эффективность специального лечения. Окончательная оценка характера поражения, расцененного по результатам визуальной эндоскопической диагностики как лимфосаркома, должна основываться на данных морфологического исследования биопсийного материала.
Thirty eight children with large-cell anaplastic lymphomas have been recently treated at the Pediatric Department of Malignant Lymphomas, Russian Cancer Research Center, Russian Academy of Medical Sciences. Cytoimmunological studies have revealed that 24 patients had 4 types of cellular elements of large-cell anaplastic lymphoma: Kil(+), Kil(+)B, B-cell, and T-cell lymphomas in 11, 2, 8, and 3 children, respectively. Intensive chemotherapy was performed in relation to the immunophenotype.
Treatment for mediastinal lymphosarcoma was given to 71 patients, aged 3-14 years, at the Institute's Clinics during 1982-1991. In that group, there were more males than females (3.4:1), mean duration of the condition of 3 months, T-cell immunity pattern (89.9%), enlarged anterior mediastinal lymph nodes and thymus, pleural lesions including pleuritis, mean mediastinal-thoracic index of 0.5, compression syndrome, elevated concentration of lactate dehydrogenase and presence of tumor cells in the bone marrow and peripheral blood. Among major negative factors of prognosis were inadequate therapy, pleural lesions and stage IV tumor.
The paper presents a retrospective analysis of clinical evidence elucidating correlations between the disease cytomorphology, proliferative activity, clinical stages, hemopoiesis and survival of the patients. A combined approach oriented on individual measurement of the disease proliferative parameters is recommended to increase lymphosarcoma prognosis reliability and administration of adequate therapy.
The paper presents a retrospective analysis of clinical evidence elucidating correlations between the disease cytomorphology, proliferative activity, clinical stages, hemopoiesis and survival of the patients. A combined approach oriented on individual measurement of the disease proliferative parameters is recommended to increase lymphosarcoma prognosis reliability and administration of adequate therapy.
The course of histioproliferative diseases was analyzed in 308 children treated at the All-Union Cancer Research Center, USSR AMS. Of these, there were 8 children with benign proliferation (Rosai-Dorfman disease). Histiocytosis-X was revealed in 232 children. The authors describe the regularities of histiocytic proliferation manifestations depending on the patients' age. Provide morphological (cytological) criteria for different types of histioproliferative diseases and characterize the treatment data.
Emphasis is laid on the role and high information content of cytological research methods in early diagnosis of oncohematological diseases in children. The Soviet cytoimmunological classification scheme of lymphosarcomas is presented. The use of the scheme will be helpful in unifying the estimation of the tumor substrate in different clinics of this country and will make it possible to carry out cooperative studies. A detailed cytological analysis of differential diagnostic differences in histoproliferative diseases of tumorous and reactive nature is provided. The difficulties of differential diagnosis in cases of bone marrow lesion in neurogenic tumors and acute lymphoblastic leukemia are related. The necessity is stressed of the training of skilled personnel and employing cytologists on the staff of the laboratories of large children's hospitals.
Emphasis is laid on the role and high information content of cytological research methods in early diagnosis of oncohematological diseases in children. The Soviet cytoimmunological classification scheme of lymphosarcomas is presented. The use of the scheme will be helpful in unifying the estimation of the tumor substrate in different clinics of this country and will make it possible to carry out cooperative studies. A detailed cytological analysis of differential diagnostic differences in histoproliferative diseases of tumorous and reactive nature is provided. The difficulties of differential diagnosis in cases of bone marrow lesion in neurogenic tumors and acute lymphoblastic leukemia are related. The necessity is stressed of the training of skilled personnel and employing cytologists on the staff of the laboratories of large children's hospitals.
Comparative analysis of hemopoiesis and study of the morphofunctional characteristics of blast cells in 89 children with hematosarcomas and in 152 children afflicted with acute leukemia revealed significant differences both in the nature of blast metaplasia of bone marrow and in the degree of inhibiting erythro- and thrombocytopoiesis in acute leukemias and hematosarcomas in the stage of leukemization. Study of the function of blast cells allowed one to distinguish specific features of tumor elements similar in their cytological parameters. The differences in the character of hemopoiesis derangement in different forms of hemoblastoses can be used under clinical conditions for differential diagnosis of the leukemic stage of hematosarcomas and leukemias.
Analysis was made of the results of the treatment of 18 children afflicted with lymphoblastic lymphosarcoma, with estimation of predictors influencing the patients' survival (from 1980 to 1986). The most important predictors influencing the 2-year relapse-free survival were found to be the stage, localization of the process, the presence of the symptoms of intoxication and biological activity. The data obtained were used later in the treatment of 160 children during 1986 to 1989. The results of the 2-year survival of this patients' group appreciably differ from those derived before.