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Background: Transplant-associated thrombotic microangiopathy (TAM) is a life-threatening complication of hematopoietic stem cell transplantation (HSCT). There is some evidence of endothelial injury playing a significant role in TAM development. The efficacy of defibrotide was demonstrated for prophylaxis and treatment of another HSCT-associated endothelial damage syndrome-liver veno-occlusive disease. The data for defibrotide usage in TAM are limited. Case Description: A 9-year old boy underwent HSCT from a matched unrelated donor for monosomy seven-associated myelodysplastic syndrome treatment. A myeloablative preparative regimen and post-transplant immunosuppression with cyclophosphamide on days +3 and +4 and a combination of tacrolimus with mycophenolate mofetil from day +5 were used. From day +61, sustained fever with progressive neurologic impairment and no evidence of infection was observed. On day +68, the patient developed severe TAM with acute kidney injury requiring renal replacement therapy (RRT). Defibrotide therapy 25 mg/kg/day was administered for 7 days with resolution of TAM symptoms. It was followed by multiple hemorrhagic episodes-epistaxis, hemorrhagic cystitis, and renal hemorrhage, which are presumed to be the complications of defibrotide therapy. Conclusion:Defibrotide could be an effective therapy for TAM, but adequate doses, duration of therapy, and drug safety profile both for pediatric and adult patients need to be evaluated by randomized prospective studies.

BACKGROUND: The mortality rate of children with hematological cancer and acute respiratory distress syndrome is still high, which is often associated with the ineffectiveness of traditional mechanical ventilation methods in the treatment of critical hypoxemia in these patients. Currently, the search continues for alternative methods of respiratory support, one of which is the combined high-frequency jet artificial ventilation of the lungs. AIM: This study aimed to evaluate the efficacy and safety of combined high-frequency jet artificial ventilation in the treatment of children with hematological malignancies and severe acute respiratory distress syndrome. MATERIALS AND METHODS: The study was conducted in the Department of Resuscitation and Intensive Care of the Dmitry Rogachev National Medical Research Center for Pediatric Hematology, Oncology and Immunology, from 2016 to 2020. Combined high-frequency jet artificial ventilation was used as an alternative method of respiratory support. RESULTS: In case of severe hypoxemia caused by secondary severe acute respiratory distress syndrome, the use of combined high-frequency jet ventilation after 12 h leads to a significant improvement in arterial blood oxygenation, improves the biomechanical characteristics of the respiratory system, and reduces the likelihood of developing ventilator-associated lung damage. An increase in oxygenation and absence of an effect on the indicators of central hemodynamics provide a greater delivery of oxygen to the tissues, thereby improving the general condition of the patients. CONCLUSIONS: In severe parenchymal respiratory failure accompanied by critical hypoxemia, combined high-frequency jet artificial ventilation of the lungs can be considered an alternative method of respiratory support.

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