variants underlying pediatric low-hypodiploidy B-cell acute lymphoblastic leukemia demonstrate diverse origins and may persist as a hematopoietic clone in remission.

Pediatric low-hypodiploidy B-cell acute lymphoblastic leukemia (LH-ALL) with variants has been proposed to be considered a manifestation of Li-Fraumeni syndrome (LFS). However, our study demonstrates that of the majority the pathogenic variants in the TP53 gene are somatic (70.5%), and only 12.

Blinatumomab as postremission therapy replaces consolidation and substantial parts of maintenance chemotherapy and results in stable MRD negativity in children with newly diagnosed B-lineage ALL.

The bispecific T cell-binding antibody blinatumomab (CD19/CD3) is widely and successfully used for the treatment of children with relapsed or refractory B-cell precursor acute lymphoblastic leukemia (BCP-ALL). Here, we report the efficacy of a single course of blinatumomab instead of consolidation chemotherapy to eliminate minimal residual disease (MRD) and maintain stable MRD-negativity in children with primary BCP-ALL.Between February 2020 and November 2022, 177 children with non-high-risk BCP-ALL were enrolled in the ALL-MB 2019 pilot study (NCT04723342).

Incidence and prognostic value of central nervous system involvement in infants with B-cell precursor acute lymphoblastic leukemia treated according to the MLL-Baby protocol.

Aim: The aim of the study was to evaluate the incidence and prognostic impact of central nervous system (CNS) involvement in infants with B-cell precursor acute lymphoblastic leukemia (BCP-ALL), as well as its relation with minimal residual disease (MRD) data.

Methods: A total of 139 consecutive infants with BCP-ALL from the MLL-Baby trial were studied. Cerebrospinal fluid (CSF) samples were investigated by microscopy of cytospin slides.

Efficacy of combined immunosuppression with or without eltrombopag in children with newly diagnosed aplastic anemia.

We compared the efficacy and safety of eltrombopag (ELTR) combined with immunosuppressive therapy (IST) and IST alone in treatment-naïve children with severe (SAA) and very severe (vSAA) aplastic anemia. Ninety-eight pediatric patients were randomized to receive horse antithymocyte globulin (hATG) and cyclosporin A (CsA) with (n = 49) or without (n = 49) ELTR. The primary endpoint was the overall response rate (ORR) at 4 months.

Comparison of minimal residual disease measurement by multicolour flow cytometry and PCR for fusion gene transcripts in infants with acute lymphoblastic leukaemia with KMT2A gene rearrangements.

This study aimed to evaluate the concordance between minimal residual disease (MRD) results obtained by multicolour flow cytometry (MFC) and polymerase chain reaction for fusion gene transcripts (FGTs) in infants with acute lymphoblastic leukaemia (ALL) associated with rearrangement of the KMT2A gene (KMT2A-r). A total of 942 bone marrow (BM) samples from 123 infants were studied for MFC-MRD and FGT-MRD. In total, 383 samples (40.

Prognostic value of minimal residual disease measured by fusion-gene transcript in infants with KMT2A-rearranged acute lymphoblastic leukaemia treated according to the MLL-Baby protocol.

The prognostic value of minimal residual disease (MRD) measured by fusion-gene transcript (FGT) detection was investigated in 76 infants (aged ≤1 year) with acute lymphoblastic leukaemia (ALL) with lysine methyltransferase 2A (KMT2A) rearrangements. Either at the end of induction or at later time-points, FGT-MRD-positivity was associated with poor outcome. FGT-MRD-positivity after first consolidation or first high-risk block detected 46·5% of infants with extremely poor outcome [disease-free survival (SE) 0·06 (0·06), cumulative incidence of relapse (SE) 0·91 (0·05)], which was also confirmed in multivariable analysis.

The role of nelarabine in the treatment of T-cell acute lymphoblastic leukemia: literature review and own experience.

Aim: The analysis of experience of nelarabine use in refractory/relapsed T-cell acute lymphoblastic leukemia (T-ALL) depending on the immunophenotype and the line of therapy.

Materials And Methods: All the patients with relapsed or refractory T-ALL aged from 0 to 18 years who received treatment with nelarabine as a part of the therapeutic element R6 were included in the study. For all patients a detailed immunological analysis of leukemia cells with discrimination of immunological variants TI, TII, TIII or TIV was performed.

[Adverse side effects of anticancer drugs in treatment of children with acute lymphoblastic leukemia].

The introduction of acute leukemia chemotherapy programs in clinical practice improved effectiveness of treatment and patients' survival rate. However there is a high incidence of anticancer drugs toxicity leading to a decrease in therapeutic effect of treatment. Acute lymphoblastic leukemia (ALL) is a leader among oncohematological pathologies in childhood and adolescence.

[Invasive mucormycosis in patients with hemoblastosis in St.-Petersburg].

Thirty four patients with mucormycosis in 10 hospitals of St. Petersburg were observed in 2004-2013. The most frequent underlying diseases of mucormycosis were acute leukoses (64%).

[Comparative evaluation of antiplatelet efectiveness drugs of original and reproduced enteric forms of acetylsalicylic acid (clinical study ICAR))].

The purpose of the study was a comparative study of antiplatelet activity of acetylsalicylic acid drugs, produced in gastro-resistant form trombopol 75 mg and aspirin cardio 100 mg in patients with high risk of cardiovascular events. Effect of trombopol 75 mg versus 100 mg aspirin cardio on platelet aggregation in 30 patients with high risk of cardiovascular events during 3 week treatment period was studied. Design method: blind, randomized, crossover method.

[Efficiency of the ALL-MB-2002 protocol in children with acute lymphoblastic leukemia].

Aim: To evaluate the efficiency of the original ALL-MB-2002 protocol within the multicenter study of treatment of acute lymphoblastic leukemia (ALL) in children.

Subjects And Methods: A total of 1873 primary patients with ALL aged 1 to 18 years, of whom 1544 patients were enrolled in this study, were notified at 36 clinics of Russia and Belarus from April 15, 2002, to January 1, 2008.

Results: With the median observation of 4.

[Radiotherapy in multiple modality treatment of children with nephroblastoma].

The role of radiotherapy in multiple modality treatment of Wilms' tumor is evaluated in 225 children aged 3 months to 11.5 years (mean age 3.5 years) with stage III-IV.

[Modern prospects for polychemotherapy of solid neoplasms in children].

To improve life quality and to increase survival in children with solid tumors, emphasis is placed on chemotherapeutical treatments along with other existing ones (surgery, radiation). By taking into account the fact that most malignant tumors in children are highly responsive to chemotherapy, programme treatment in children with neoplasms included the new drugs iphosphamide, vepeside, cisplatin, carboplatin, melfalan, bleomycin. The results of therapy with these drugs alone or in combination with well-known effective anthracycline antibiotics and plant preparations are presented.

[Early extubation of children in the the first year of life with inborn heart failure after surgery, performed with artificial blood circulation].

The possibility of early extubation of the first year of life children after surgical correction of congenital heart diseases while artificial blood circulation conduction was studied. For the 1996-1999 period surgical treatment was conducted in 381 patients aged at average (5.71 +/- 3.

[Metastatic lung tumors in children (clinical course, diagnosis, prognosis)].

According to data available at the Institute (1985-1999, approx. 1,200 patients), the lung is the most frequent site of cancer dissemination (up to 75%). Such most frequent pediatric children tumors as Wilms' tumor, neuroblastoma, rhabdosarcoma, osteogenic sarcoma and that of Ewing disseminate to lung in 85%, particularly, at advanced stages.

[Radiologic diagnosis of malignant tumors of the pelvic bones in children].

An analysis of the data on radiodiagnosis of malignancies of pelvic bones in 50 patients, aged 10-15, is presented. Diagnosis was morphologically confirmed in all the cases: Ewing's sarcoma (41)--82%, primitive neuroectodermal tumor (6)--12% and chondrosarcoma (3)--6%. The leading clinical syndrome was pain in the loin.

[The experience of application of peritoneal dialysis in cardiosurgery of an early age children].

The experience of the peritoneal dialysis (PD) successful application in 16 children of an early age was summarized. In all the patients the terminal state with an acute cardiac and renal insufficiency was noted after cardiosurgical operation conduction using artificial blood circulation. The improvement of hemodynamical indices, renal and pulmonary function as well, were promoted by an early application of PD.

[Some indices of gastrointestinal tract status in children cured fro malignant neoplasms].

A total of 2255 individuals who cured from oncological diseases in childhood in Moscow in 1978 to 1997 have been studied. Most (60%) children undergone special treatment have serious visceral abnormalities. The authors examined the negative impact of a special treatment on the gastrointestinal mucosa and changes in its function after termination of special therapy in 79 cured patients.

[Ultrasound tomography in diagnosis of malignant soft tissue tumors in children].

Ultrasound findings of 50 children with soft tissue sarcomas of the trunk and limbs were analyzed. There was a high proportion of diagnostic errors (62%), which resulted in the choice of adequate therapeutical measures at early stages of therapy and in advanced disease. The reasons of the errors were as follows: nonspecificity of the first clinical manifestations of soft tissue sarcomas; ignorance of instrumental studies that establishes a diagnosis in time; outpatient physicians' unconcern of cancer, misunderstanding of the nontumor nature of disease; no scientifically grounded algorithm of studies.

[Clinical manifestations and diagnosis of childhood mediastinal tumors].

Mediastinal tumors make up as high as 7% of the malignant neoplasms in children and are encountered in all age groups, slightly more commonly in preschool children. Three main groups of common symptoms that are typical of mediastinal tumors are identified. These include 1) symptoms associated with tumor compression of thoracic organs; 2) neurological disorders associated with compression or destruction of ganglionary nodes; 3) general symptoms.

[Cerebral pathology in inactive rheumatism].

Aim: Characterization of cerebral pathology in inactive phase of rheumatism.

Materials And Methods: Clinical, experimental psychological examinations were performed in 110 rheumatic patients with inactive disease. Computer tomography of the head was carried out in 45 patients.