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Rissian journal listRissian journal listRissian journal listRissian journal listRissian journal listRissian journal list

Rissian journal listRissian journal listRissian journal listRissian journal listRissian journal listRissian journal list

Rissian journal listRissian journal listRissian journal listRissian journal listRissian journal list

Hematopoietic stem cells transplantation (HSCT) is a potentially curative method for patients suffering from different oncological, hemato-logical and inherited diseases. Two variants of these procedures are autologous (аuto-HSCT) and allogeneic (аllо-HSCT) transplantation. Indications, availability of donor (related, unrelated, haploidentical), conditioning regimen (myeloablative, reduce intensity conditioning regimens, non-myeloablative), methods of “graft versus host disease” prophylaxis are taken into account when performing HSCT. In allo-HSCT “graft versus leukemia” effect develops and it is the platform for providing of immunoadoptive therapy.

The pharmaceutical neuropathy is the most often complication from the part of the nervous system at children, receiving the therapy with chemical therapeutic agents. Among most unfavorable substance vincristine (VNC) is highlighted, which neurotoxicity often causes the dose reduction or its full withdrawal from the used treatment protocol. The clinical pattern of the VNC induced neuropathy in most cases is represented by the lesion of nerve fibers of upper and lower extremities with the development of symptoms of the peripheral polyneuropathy. In more rare cases VNC causes the lesion of axons of cranial and brain nerves. The given clinical case describes the monitoring of the cranial neuropathy, the lesion of the oculomotor nerve, presented by bilateral ptosis at a child with Ewing,s sarcoma, treated by СWS-2009 protocol.

In recent decades impressive results were achieved in the treatment of children»s acute leukemia. Anyway, there are some rare forms of such tumors, for which till the present time the tactic is unknown. To such diseases are referred acute biphenotypic leukemia (ABL), which is very rare at children, so it is difficult for clinicians to determine the treatment program. ABL is diagnosed, when the simultaneous expression of antigens of different hemopoietic is revealed on blast cells. The forecast for children is unknown and the adults, forecast is unfavorable. The optimal treatment approach is also unknown, but should comprise the high-dose chemotherapy, as well as the allogeneic stem cell transplantation. Nowadays this rare form of leukemia is being intensively studied by molecular, immunologic and cytogenic methods. The work presents the analysis of a clinical case of ABL at a child with Williams syndrome.

It is not possible to imagine the antitumor and supportive treatment in children oncology without permanent central venous access. The development and modernization of such systems caused the appearance of the central venous catheter (CVC) without external component. The external CVC has got several shortages during use, especially in children,s practice, while totally implanted port-systems not only have not got most of them, but have got a lot of advantages as well. All catheter associated complications can be conditionally divided into 2 groups: ones referred to the procedure of catheterization of central veins and ones developing in the process of CVC use. The base of the oncology and tumors, chemotherapy division has got the experience of use of infusion venous ports since February 2006. This article presents the clinical case of the mechanical complication development in the process of the port-system exploitation: detachment of the catheter distal end and its migration to the distal segment of the left distal pulmonary artery, as well as the tactics of the patient management.

The research comprised 78 patients: girls – 36 (46 %), boys – 42 (54 %). At the moment of the allogeneic hematopoietic stem cell transplantation (аllо-HSCT) their age was 1–18 years (median – 9 years); the age at the moment of examination was 2.5–21 years (median – 11 years); the monitoring after allo-HSCT was 1–11 years (median – 3.5 years); the monitoring period of less than 5 years was at 50 (64 %), more than 5 years – at 28 (36 %) of patients.Patients, height, weight, waist, blood pressure (BP) were measured. Laboratory examination: determination of glucose, magnesium, thyreothropin, Т3, Т4, cortisone, follicle stimulating hormone, lutenizing hormone levels were measured, the lipidogram was made, at girls over 14 estrogens and progesterone levels were determined, at boys over 14 testosterone levels were determined. At 22 (28 %) patients was evaluated the activity of renine-angiotensine-aldosterone system (determination of renine and aldosterone concentration).Endocrinologic complications after allo-HSCT were observed at 68 (87 %) of patients. The deterioration of the thyroid gland function was diagnosed at 34 (44 %) of the children and teenagers, the secondary adrenal insufficiency was observed at 7 (9 %) of patients, the hypergondotropic hypogonadism was observed at – у 12 (57 %) patients, the arterial hypertension (BP > 130/85 mm Hg) – at 9 (12 %), hyperglycemia (glucose level > 5.6 mmol/l) – at 10 (13 %) of patients. The dislipidemia and double dislipidemia – at 44 (56 %) и 24 (31 %) of patients accordingly, hypertriglyceridemia – at 30 (68 %), hypercholesterinemia – at 22 (50 %), the decrease of high density lipoproteins – at 20 (45 %), the increase of the very low density lipoproteins, level – at 4 (9 %) of patients. Obesity – at 11 (14 %) of patients; among them I degree – at 10 (91 %), II degree – at 1 (9 %). The average age of patients with obesity at the moment of examination – 10.5 years. The metabolic syndrome was diagnosed at 3 (4 %) patients.Was revealed the authentic link between the dislipidemia and the chronic reaction of “graft versus host disease” (р = 0.001), hypomagniemia (р = 0.003), deterioration of the thyroid gland function (р = 0.007). The influence of the age at the moment of allo-HSCT (1–10 vs older10 years), sex, diagnosis (oncologic vs non-oncologic), conditioning mode (myeloablative vs conditioning mode with increased intensiveness), monitoring period after allo-HSCT (1–5 vs older 5 years) on the risk of dislipidemia development is not established.

The article contains the schedule of monitoring of late complications in children after hematopoietic stem cell transplantation (HSCT), аs well as peculiarities of the prophylactic examination of such patients both in specialized and general health facilities at the place of residence. There is a short description of late complications, its diagnostics and treatment. This publication is designed for physicians, carrying out several kinds of HSCT, children oncohematologists, hematologists, immunologists and pediatricians, performing the local follow-up of children, suffering with oncohematologic and immunologic diseases, as well as for medical specialists (endocrinologists, neuropathologist, oculists, gynecologists, cardiologists, gastroenterologists, nephrologists etc.).

Not like most malignant tumors, with Hodgkin,s lymphoma (HL) the possibility of curing remains at patients, which tumors did not respond to the 1st line therapy (primary resistance or disease recurrence). Nowadays the standard method of treatment of these patients is the high-dose chemotherapy with transplantation of autologous cells of hemopoiesis precursors (ТCHP). Anyway, in the RF this treatment method is used much less often than in developed world countries, what is stipulated by the deficit of available transplantation facilities. The impossibility of TCHP administration to all patients, who need it, has a negative impact on the total effectiveness of the HL treatment, requiring to look for factors, allowing to optimize the selection of patients for TCHP. Anyway, the analysis of results of foreign and national research shows, that the only factor, allowing to adequately select patients for TCHP, is the tumor response for the induction therapy (patients off remission should not receive TCHP). Other factors (treatment option, 2nd line therapy in the anamnesis) are not reliable enough to make decision to refuse to perform TCHP. So, all patients, obtaining remission due to the induction therapy, should be considered as candidates for TCHP, and the only method to prevent the peril of patients with recurrences and primary resistant HL course in the RF is the increase of the transplantation activity (number of transplantation centers).

We have evaluated the safety and efficiency of the comprehensive examination of the bronchoalveolar lavage (BAL) (n = 222) for the diagnosis of the invasive aspergillosis of lungs (IAL) at 150 patients after the hematopoietic stem cell transplantation (HSCT) in childhood. There were no major adverse effects during the bronchoscopy. IAL was revealed at 30 % of HSCT recipients with changes in lungs at the computer tomogram. The multivariant analysis the galactomannan (GM) test in IAL showed higher sensitivity indices in comparison with the direct microscopy and inoculation (83.3 and 46.3 % accordingly). With the optic density index equal to 0.925, the diagnostic precision, showed by the area under curve at ROC-аnalaysis, was 0.794 (95 % confidence interval 0.665–0.923). The most diagnostic efficiency is achieved by the multivariant analysis, when using all 3 mentioned methods – microscopy, inoculation and GM test in IAL.

While continuing the series of publications on the application of intravenous immunoglobulins (IVIG), as one of basic substances in the therapy of immunologic, hematologic and oncologic diseases we have focused on the safety of modern IVIG substances. The article comprises main requirements to IVIG solutions, as well as measures for the improvement of such substances in order to prevent adverse effects. The material comprises the updated statistics on the application of 10 % IVIG in the federal center.