[Use of flow cytometry in the diagnosis of acute leukemias in childhood]. / Vyuzití prutokové cytometrie v diagnostice akutních leukémií detského veku.

The authors examined, using the method of flow cytometry, 56 children with acute lymphoblastic leukaemia. Leukaemic cells of the bone marrow aspirate and peripheral blood were examined on a FACS 440 apparatus for establishment of the diagnosis before treatment was initiated. Individual immunological subtypes were differentiated by means of a panel of monoclonal antibodies. 80.5% of acute lymphoblastic leukaemias originated from different developmental stages of B cells, 12.5% were formed by leukaemias from T cells and 7% were non-differentiated leukaemias. The mean follow-up period in the group was 33 months. According to the therapeutic results children with leukaemia ensuing from precursors of B cells had a more favourable prognosis than children with T leukaemia and children with non-differentiated leukaemia. Quantitative examination of nuclear DNA of leukaemic cells revealed in 55% of the patients of the group aneuploidy with clear predominance of hyperdiploidy, 45% of the patients suffered from diploidy. The least number of relapses was recorded in the investigation period in children with hyperploid acute lymphoblastic leukaemia. The proliferating activity of leukaemic blasts was expressed by the number of cells in the S + G2M stage of the cellular cycle and was higher in the bone marrow than in peripheral blood but did not differ in individual immunological subtypes or in diploid leukaemias. The authors were not able to prove its prognostic importance. Flow cytometry is a rapid and sensitive diagnostic method which makes it possible to characterize more satisfactorily the heterogeneous group of acute lymphoblastic leukaemias.

Syndrome of secondary polymyositis with leukemia.

A boy with neurofibromatosis suffered mumps at the age of 5. A full-blown juvenile polymyositis developed shortly afterwards. First hematological consultation was done for monocytosis in peripheral blood at the age of 7. He suffered varicella at the age of 8. Diagnosis of acute nonlymphocytic leukemia with monosomy 7 was done before the age of 9. The boy died at the age of 10.

[Histiocytic syndromes in childhood]. / Histiocytární syndromy v detském veku.

The authors submit an account of the new classification of histiocytic diseases in childhood, they mention contemporary views on their aetiology, pathogenesis, advances in diagnosis and therapy. The paper is supplemented by case-histories from the authors' own practice.

[Familial cerebral degeneration with myocardial involvement and dyserythropoiesis]. / Familiární cerebrální degenerace s postizením myokardu a dyserytropoesou.

A combination of dyserythropoietic anaemia, encephalopathy and cardiomyopathy was found in two siblings of different sex. They shared the same clinical history and pathomorphology what made authors suppose that it was a new not yet described syndrome with presumed autosomal recessive heredity.

[Advances in the treatment of acute lymphoblastic leukemia in childhood: experience with intensive protocol treatments]. / Pokrok v lécbe detské akutní lymfoblastické leukémie: zkusenosti s lécbou pomocí intenzívních protokolu.

The authors analyzed a group of 64 children with acute lymphoblastic leukaemia (ALL) treated according to three protocols of different intensity. The best therapeutic results were obtained in children treated according to the most intensive protocol of the West German Multicentre Investigation BFM 83 which is graded as to its intensity with regard to the degree of risk of an adverse course. Successful remission in the entire group of patients was 93%, one third of the children developed during the investigation period a relapse of the basic disease. 12% of the children died during remission from complications of treatment. The surprising agreement of therapeutic results of different protocols after three years' complete remission is apparent from the fact that early relapses during the first two years of treatment, implying resistance to administered therapy, are at present the greatest problem of effective treatment which is not resolved even by the ever increasing intensity of treatment. In the conclusion the authors define the group of patients with a high risk of early relapse for whom new therapeutic procedures must be sought.

[Familial hemophagocytic lymphohistiocytosis]. / Familiární hemofagocytující lymfohistiocytosa.

This rare fatal disease of infants and early childhood occurred in seven children from four families. Six children died during 2-4 weeks from the beginning of disease, the last one has survived two years with cytostatic treatment. The disease was characterized by intermittent fever, hepatosplenomegaly and progressive pancytopenia as well as hyperbilirubinemia, hyperlipemia and hypofibrinogenemia. In addition to substantial enlargement of the liver and spleen prevailing autoptic findings were infectious complications due to severe immune defect or signs of hemorrhagic diathesis. Lymphocytes and histiocytes phagocytizing blood cells were increased in bone marrow, liver, spleen, often brain and further organs. Problems of differential diagnosis and pathogenesis were discussed.

[Factors affecting the prognosis of acute lymphoblastic leukemia in childhood]. / Faktory ovlivnující prognózu akutní lymfoblastické leukémie v detském veku.

The authors tried to test the value of some clinical and laboratory characteristics for the prognosis of acute lymphoblastic leukaemia (ALL) in a group of 69 children treated according to three different protocols. The results were evaluated by methods of one-dimensional and multidimensional analysis. The absolute number of blasts in the peripheral blood stream and initial leucocytosis during establishment of the diagnosis proved to be the most important risk factors influencing the prognosis of the patients. Other adverse signs for the prognosis of ALL in the group were a mediastinal tumour, L2 type of leukaemic blasts according to the morphological FAB classification and age above 10 years when the diagnosis was established. The patient's sex, immunophenotype of the leukaemic blasts, chromosomal abnormality of the karyotype in the leukaemic cells, marked hepatosplenomegaly, thrombocytopenia, haemoglobin values and PAS reaction in the blasts, did not affect the therapeutic results in the author's group of patients.

The importance of blast cell DNA content for prognosis of childhood acute lymphoblastic leukemia.

The prognostic value of cellular DNA content measured by static cytophotometry was evaluated in 69 children with acute lymphoblastic leukemia (ALL) using the pretreatment distribution of the DNA content in blast cells of bone marrow and peripheral blood. The median follow-up of the whole group of patients was 45 months. Aneuploidy was detected in 71% of children, most of them showing a hyperdiploid content (DNA index greater than 1.05). The duration of complete remission was significantly longer in patients with distinct hyperdiploid DNA content (DNA index greater than 1.16) than in those with less hyperdiploid and diploid DNA content (DNA index less than 1.16). The results achieved by static cytophotometry were compared with flow cytometry analysis and with cytogenetic investigations of chromosomal abnormalities in leukemic cells. Higher correlation was found between flow cytometry and cytogenetics. Flow cytometry proved to be a more convenient method for detection of the DNA content in leukemic cells than static cytophotometry.

Correlations of clinical and laboratory data for prognosis in childhood acute lymphoblastic leukemia.

Correlation of 24 different clinical and laboratory measures was performed for a calculation of the prognosis in 65 children with newly diagnosed acute lymphoblastic leukemia (ALL). The hematologic as well as genetic parameters were studied at the time of diagnosis. It was shown that the initial blast count in peripheral blood is the most important risk factor for the prognosis of ALL. Cytogenetic, DNA cytometric, and pedigree data correlate with blast count and also seem to be very important determinants for ALL prognosis. The statistically significant increase in the incidence of neoplasms in ALL families is reported.

Immunologic characterization of T-lymphoproliferative disorders by monoclonal antibodies.

A series of 11 mouse monoclonal antibodies reactive with human T lymphoid cells at different stages of differentiation was used for immunological classification of leukaemic cells of 16 patients with T cell lymphoproliferative disorders by using a fluorescence assay. The majority of T-ALL cells had an immature or early thymic phenotype and T lymphoblastic lymphoma had phenotypes corresponding to different levels of more mature stages of T cell differentiation, Two cases of T-CLL and one adult patient with mycosis fungoides had mature T cell phenotypes being T-3+, T-4-, T-8+, cytotoxic/suppressor cell types and one case of T-CLL had T-3+, T-4+, T-8-, "helper/inducer" cell type, too. These results suggested that surface marker analysis in T cell lymphoproliferative disorders may be used as a highly reproducible immunological classification system that will provide additional information about phenotypes of leukaemic cells in connection with morphological analysis and clinical diagnosis.