It has been suggested that platelet function in chronic immune thrombocytopenic purpura (ITP) may be abnormal. Thrombopoietin mimetics used for treatment can affect it, but the data remain limited. We investigated platelet function of 20 children diagnosed with severe ITP (aged 1-16 years, 12 females and eight males). Platelet functional activity in whole blood was characterized by flow cytometry before and after stimulation with SFLLRN plus collagen-related peptide. Levels of CD42b, PAC1, and CD62P, but not CD61 or annexin V, were significantly increased (P < 0.05) in resting platelets of patients before treatment compared with healthy donors. On average, PAC1 and CD62P in patients after activation were also significantly elevated, although some patients failed to activate integrins. Romiplostim (1-15 μg/kg/week s.c.) was prescribed to seven patients, with clinical improvement in six. Interestingly, one patient had clinical improvement without platelet count increase. Eltrombopag (25-75 mg/day p.o.) was given to four patients, with positive response in one. Others switched to romiplostim, with one stable positive response, one unstable positive response, and one non-responding. Platelet quality improved with romiplostim treatment, and their parameters approached the normal values. Our results suggest that platelets in children with severe ITP are pre-activated and abnormal, but improve with treatment.

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Pigmented epithelioid melanocytoma (PEM) is an uncommon, recently described melanocytic lesion. There is a high rate of regional metastases, but limited evidence of distant metastases, an indolent clinical course and favorable outcome. The article presents a clinical case of PEM with aggressive behavior and distant metastases in patient with Fanconi Anemia after hematopoietic stem cell transplantation. Parents gave their consent to use information about the child, including fotos, in the article.

Введение. Тромбозы правого предсердия (ТПП) являются редкой патологией, которая чаще встречается среди новорожденных и детей, перенесших кардиохирургическое вмешательство. Истинная распространенность, а также подходы к лечению ТПП у детей, получающих терапию по поводу злокачественного заболевания, неизвестны. Цель работы: оценить распространенность ТПП, а также эффективность и безопасность применения низкомолекулярного гепарина (НМГ) у детей с ТПП. Материалы и методы. Проведен анализ электронной базы данных пациентов в возрасте 0-17 лет. Для оценки динамики размеров тромбов в анализ были включены только пациенты с объективно подтвержденным ТПП, у которых имелось не менее двух эхокардиографических исследований, и которым проводилась терапия НМГ. Оценка случаев кровотечений и тромбоэмболии легочной артерии/артерий (ТЭЛА) проводилась по записям в историях болезни пациентов. Результаты. Изолированный ТПП или ТПП в комбинации с тромбозами других локализаций был диагностирован у 13,7%, изолированный ТПП – лишь у 5% детей. При проведении антикоагулянтной терапии НМГ отсутствие динамики со стороны размеров тромба наблюдалось в 1 случае, нарастание размеров тромба – в 1 случае, уменьшение размеров тромба или полный лизис – в 13 случаях. За период наблюдения не было выявлено ни одного случая крупных кровотечений и ТЭЛА. Заключение. ТПП встречаются в 13,7% случаев среди пациентов с тромбозом. Мы считаем применение НМГ эффективным и безопасным методом вторичной антитромботической профилактики у детей с ТПП. Introduction. Right atrium thromboses (RAT) are rare pathology which is more specific for neonates and children undergoing cardiac surgery. True RAT prevalence as well as approaches to its treatment in children receiving therapy for malignant disease are unknown. Aim: to assess RAT prevalence as well as effectiveness and safeness of low molecular weight heparin (LMWH) application in children with RAT. Materials and methods. Electronic hospital database analysis was performed to find patients with objectively confirmed RAT. Children aged 0-17 years old with two or more echocardiographic examinations who received LMWH were included in final analysis. Thrombus size dynamics, the rate of pulmonary embolism (PE) and major bleeding served as effectiveness and safeness endpoints. Results. Isolated RAT or RAT in combination with thromboses of other localizations was found in 13,7%, isolated RAT – only in 5% of children. Under anticoagulant therapy with LMWH we found no dynamics in clot size – in 1 case, clot size increasing – in 1 case, reduction of clot size or its complete lysis – in 13 cases. There were no cases of major bleeding and PE during LMWH treatment. Conclusion. RAT occurs in 13,7% of cases among patients with thrombosis. We consider LMWH treatment to be effective and safe method of secondary antithrombotic prophylaxis in children with RAT.