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The authors have examined 134 children with acute lymphoblast cell leukemia (ALCL) who were treated at the Research Institute of Pediatric Oncology and Hematology, Russian Cancer Research Center, in January 1990 to November 1999, and followed up till March 1, 1999. The mean duration of follow-ups was 57.46 +/- 2.87 months. The minimum follow-up was 4 months. The immunophenotypical features of stem cell immunological subvariant of ALCL identified from the presence of 10% or 15% of CD34+ lymphoblast cells were similar in the leukemia clone. There is evidence for the isolation of the least mature, stem-cell immunological subvariant of ALCL in children. The immunophenotypic features of stem-cell ALCL in children were as follows: the expression of myeloid antigens (linear and different marker leukemias) and the higher rates of involvement of B-cell leukemias. The clinical and hematological features of stem-cell versus CD34-negative ALCL in children were the low levels of leukocytes (p = 0.009) and blast cells (p = 0.018) in the peripheral blood at diagnosis. At the same time, stem-cell ALCL showed a poorer prognosis. Moreover, blast cell CD34 antigen expression deteriorated prognosis for pre-pre-B (common) immunological variant of ALCL (p = 0.035). Intensified ALCL treatment programmes improved relapseless survival of patients with stem-cell ALCL (p = 0.0042).

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We analyzed CD95(Fas/APO-1) antigen expression on bone marrow blasts in 38 children with acute lymphoblastic leukemia (ALL) receiving a treatment in the Department of Leukaemias at the Cancer Research Center in 1987-1989 years (n = 22) and in 1994-1997 years (n = 16). CD95 antigen expression was studied by monoclonal antibodies (MoAbs) IPO-4 in indirect immunofluorescence analysis. CD95 antigen was expressed on 35.8 +/- 7.5% bone marrow blasts, most frequently (63.6%) in the clinically favourable Pre-B ALL. Only in this group CD95 antigen expression was correlated with CD10 antigen expression that has a positive influence to the time of complete remission in ALL patients. Our data showed that CD95 expression on blast cells is a favourable prognostic sign, associated with increased relapse-free and total survival. On the contrary, the absence of CD95 antigen on blasts is an unfavourable sign for disease evolution.

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The effectiveness of modification of the BFM-87 schedule of treatment received by 32 children with different forms of acute non-lymphoblastic leukemia since April, 1991, has been studied. Complete remission was recorded in 90% and 5-year recurrence-free survival--in 47%. Although the complication rate was higher as compared with the "7 + 3" schedule, mortality rates were relatively lower due to application of adjuvant therapy.

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Lipid peroxidation (LP) occurring in pediatric cancer patients receiving polychemotherapy has been investigated. Plasma level of malonic dialdehyde in children with retinoblastoma (Rtb) was found to drop while it remained unchanged in patients with acute lymphoblastic leukemia (ALL). The treatment caused different changes in the red cell catalase levels in said groups: the enzyme concentration increased in the Rtb patients in the course of therapy and decreased in the ALL group. A slight decline in alpha-tocopherol and retinol levels the Rtb group was matched by a relevant rise in blood-plasma in the ALL group. To adjust LP regulation and improve resistance, antioxidants should be given to pediatric cancer patients suffering peroxidation-related stress.

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The tolerance of cardioxan (JCRF-187, dexrazoxan), an ethylenediamine tetra-acetic acid analog and a Chiron Company product, administered to pediatric oncohematological patients, is discussed. The drug was used in 37 cases of acute lymphoblastic and non-lymphoblastic leukemia, non-Hodgkin's lymphoma and Hodgkin's disease treated by polychemotherapy including anthracycline antibiotics administration. No untoward side-effects or inhibition of the therapeutic effect were observed.

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Antiviral humoral immunity was studied in patients with Hodgkin's disease, idiopathic thrombocytopenic purpura (ITP), and aplastic anemia (AA). Insufficiency of antiviral humoral immunity as regards antigenically unrelated viruses and Mycoplasma pneumoniae was characteristic of all these conditions. A remission of Hodgkin's disease and ITP was associated with a reliable increase of the studied parameters in comparison with the acute period of the disease. After splenectomy humoral immunity parameters were virtually unchanged in patients with ITP, whereas in patients with Hodgkin's disease and AA the deficiency of antibody production is 40% increased after the operation.

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Cooperative investigations were conducted in seven Pediatric Hematologic Clinics (in Moscow, Leningrad, Kiev, Minsk and Tbilisi) to study the nature of late (after five years of remission) relapses of acute leukemia that were diagnosed in 21.6% of cases (in 80 out of 371 children) with long-term remissions. Late relapses in most patients occurred on the 6-7th year of remission. In cases when the treatment was abolished, relapses took place 1-2 years after the abolition. Extramedullary foci of leukemic lesions (CNS, sexual glands, etc.) were detected more frequently (41 children). Bone marrow lesions were recorded in 21 combined relapses were observed in 8 patients. It has been stressed that initial risk factors should be taken into consideration, and current diagnostic tests should be applied to individualize therapy at all the stages of the treatment.

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Antilymphocytic monoclonal antibodies of HKO and OKT series were used to study surface antigens of peripheral blood and lymph node lymphocytes in 13 untreated patients with Hodgkin's disease. The purpose of the study was to identify the pathogenetic mechanism underlying the development of immunodeficiency in the early disease stages. The data obtained have demonstrated that the deficiency of T lymphocytes in the peripheral blood was connected with that of mature T cells in the lymph nodes revealed with the use of differential antigens complex. Deficiency of immunologically differentiated T lymphocytes in the lymph nodes combined with high counts of the cells whose membranes carry Ia-like antigen point to the immunologically unfavourable course of the disease.

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8 children with chronic idiopathic thrombocytopenic purpura received a high dose therapy with monomeric IgG. Before and after the treatment immunological investigations were carried out. All the children showed a positive clinical response, in 5 children there was an increase of thrombocytes to 111-340 X 10(9)/l. A clinico-haematological effect could be shown in those children with an increased percentage of marrow megakaryocytes with IgG fixed on the membrane before treatment. There was no haematological effect neither in cases with fixed IgG and IgA nor IgM and IgA combined, as well as in the case when there was no fixed IgG on the membrane. A steady clinical effect was provided if the number of bone marrow megakaryocytes with fixed IgG reached the norm. The suppression of the synthesis of antithrombocytic antibodies of the same immunoglobulin class can be regarded as a specific mechanism of high IgG doses.

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Phase II clinical trials of L-asparaginase of leunase manufactured by "Kyowa" (Japan) was performed in cooperation by 5 institutions of the USSR on 49 patients with various forms of hemoblastosis, including 15 patients aged 1 to 15 and 34 patients aged 16 to 75. The drug was used in a daily dose of 200 IU per 1 kg body weight administered as intravenous drips daily for 2--3 weeks. The daily dose was divided into 2 doses administered at an interval of 12 hours. The efficiency of the treatment did not depend on the patients' sex. Significant efficiency of leunase was observed in children with acute lymphoblastic leukemia (85.7 per cent). The use of the drug in treatment of adults with systemic malignant blood affections was less effective. Some effects recorded in patients with generalized forms of hematosarcoma were transient. The following side effects were noted: nausea, vomiting in 8 children and 13 adults, allergic reactions in the form of pruritus and rashes in 8 adults, impairment of liver and pancreatic functions in 2 children and 1 adult. Acute pancreatonecrosis was recorded in one child. The effect on the peripheral blood was insignificant. Leunase has probably no advantages as compared to other L-asparaginase preparations.

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