Репозитарій

Hemophagocytic lymphohistiocytosis (HLH) is polygenetic disease caused by mutations in genes associated with granule-dependent lymphocyte-mediated cytotoxicity (PRF1, UNC13D, STX11, STXBP2, LYST, AP3B1, AP3D1 RAB27A, XIAP, SH2D1A).

Our cohort of patients demonstrated a repeated mutation in the UNC13D genec.2346_2349delCTCC(p.R782fs), which may be associated with the “founder effect” in Slavic countries. Atypical manifestation causes difficulties in rapid diagnosis, fast sequencing of all PID genes are necessary for establishing correct diagnosis and start appropriate treatment.

The presence of coagulopathy as part of the systemic inflammatory response syndrome is a characteristic feature of severe coronavirus disease 2019 (COVID-19). Hematological changes (increased DD-dimer, prolonged activated partial thromboplastin clotting time [APTT] and prothrombin time [PT], high fibrinogen levels) have been observed in hospitalized patients with COVID-19, which characterize the risk of thrombotic events. Against the background of COVID-19 there is endothelial dysfunction, hypoxia and pulmonary congestion, mediated by thrombosis and microvascular occlusion. Up to 71.4% of patients who died from COVID-19 had disseminated intravascular coagulation syndrome, compared with only 0.6% of survivors. The main manifestation of COVID-19-associated coagulopathy is a significant increase in DD without a decrease in platelet count or prolongation of APTT and PT, indicating increased thrombin formation and the development of local fibrinolysis. An increase in DD levels of more than 3-4 times was associated with higher in-hospital mortality. Therefore, COVID-19 requires assessment of the severity of the disease for further tactics of thromboprophylaxis. The need for 1 continued thromboprophylaxis, or therapeutic anticoagulation, in patients after inpatient treatment for two weeks using imaging techniques to assess of thrombosis assessment.

УДК 616.379-006.443:616.988.23 + 61.921.5-08-053.2

Langerhans cell histiocytosis (LCH) is an abnormal clonal proliferation of Langerhans cells. The prognosis varies depending on the form of the disease and organ damage. Any organs and systems can be involved in the pathological process in various combinations. A poor response to standard therapy and an unfavorable prognosis are characteristic of patients with a multisystem form of LCH and involvement of organs at risk. Skin lesions are a classic sign of LCH. Purpose - to describe the complexity and duration of diagnosis of LCH with multisystem damage in a boy aged 2 years and 2 months, infected with poliomyelitis and coronavirus. Clinical case. The first clinical manifestations of LCH in the child debuted with an eczematous-seborrheic rash on the scalp with spread to the limbs and trunk. The child was treated for toxicoderma, hemorrhagic vasculitis at the place of residence for 6 months. The boy lost 1.5 kg of body weight in 1 month. At the time of hospitalization, seborrheic-eczematous rashes on the skin with a hemorrhagic component, trophic-inflammatory changes in the nails of the hands, signs of protein-energy deficiency, stomatitis, gingivitis, hepatosplenomegaly, polyserositis, diabetes insipidus, osteolytic foci of the frontal bones were found. Results of the tests: anemia, thrombocytopenia, hypoproteinemia and hypoalbuminemia, coagulation disorders. The patient had the onset of lower flaccid paraparesis, muscle hypotonia. The boy was diagnosed with a number of infectious complications, including poliomyelitis (a derivative of vaccine poliovirus type 2), COVID-19. The child received LCH-III cytostatic therapy with a positive effect. The research was carried out in accordance with the principles of the Helsinki Declaration. The informed consent of the patient was obtained for conducting the studies. No conflict of interests was declared by the authors.

The role of iodine as an anticarcinogenic agent is just beginning to be widely appreciated.
METHODS The aim of our study is to identify a link between iodine deficiency and the development of hematological malignancies in children. We screened iodine status in 36 children with oncohematological diseases and 32 healthy. Children were tested for iodine in the urine, ultrasound of thyroid glands were done.

As more data is collected, hematologists will be able to gain more insight into the impact of coronavirus disease 2019 (COVID-19) on pediatric patients with hematological malignancies. Material and methods: We analysed 21 cases of COVID-19 in pediatric patients with onco-hematological diseases treated in the Western Ukrainian Pediatric Medical Center from March 2020 through May 2021. The majority of patients (71.4%) were diagnosed with acute lymphoblastic leukemia. All patients from the analyzed cohort had an asymptomatic, mild or moderate course of coronavirus-19 infection. The most common symptoms of COVID-19 were fever, cough, gastrointestinal symptoms, and dermatitis. Severe severe acute respiratory syndrome coronavirus 2 increased the risk of liver toxicity and venous thrombosis. Results and conclusion: Our analysis showed that pediatric patients with hematological malignancies need the same treatment approach for COVID-19 as for other infective complications.