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We report the case of a seven-yr-old Caucasian girl who presented with progressive deterioration of renal function 13 months after HSCT for myelodysplastic syndrome. BK virus nephropathy was suspected and confirmed. After reduction of immunosuppression and treatment with IVIG, leflunomide, ciprofloxacin, and cidofovir, clearance of BK virus from blood was achieved, and further progression or renal failure was prevented. We believe that BK virus nephropathy should be considered in cases of renal function deterioration in all immunocompromised patients.

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истиоцитоз из клеток Лангерганса (ГКЛ)редкое новообразование, возникающее вследствие клональной пролиферации и диссеминации клеток миелоидного происхождения, фенотипически похожих на клетки Лангерганса, в различные органы и ткани, избыточного их разрастания, аномальной локальной экспрессии цитокинов, повреждения структуры и функции вовлеченных органов. Заболеваемость ГКЛ у детей составляет около 3 на 1 000 000 в год, а у детей до 1 года-около 9 на 1 000 000 в год. Поражение легких при ГКЛ может носить моносистемный характер или быть частью мультисистемного поражения. У детей гистиоцитарное поражение легких, как правило, развивается в рамках мультисистемного заболевания и сопряжено с поражением других органов и систем. Изолированное поражение легких у детей встречается крайне редко и составляет менее 1% всех гистиоцитарных поражений. В структуре мультисистемных гистиоцитозов поражение легких наблюдается приблизительно у 25% пациентов.

Background Langerhans cell histiocytosis (LCH) involves abnormal proliferation of Langerhans cells (LC), which is typically driven by the BRAF V600E mutation. High-risk LCH has a poor prognosis. Procedure Fifteen children (5 girls, 10 boys) with BRAF V600E+ LCH received vemurafenib (initial dose median 40 mg/kg/day, range: 11–51.6 mg/kg/day) between March 2016 and February 2020. All patients had previous received LCH-directed chemotherapy. The median age at LCH onset was 2 months (range: 1–28 months) and the median age at the start of vemurafenib treatment was 22 months (range: 13–62 months). The median disease activity score (DAS) at the start of vemurafenib treatment was 12 points (range: 2–22 points). Results The median duration of vemurafenib therapy was 29 months (range: 2.4–45 months). All patients responded to treatment, with median DAS values of 4 points (range: 0–14 points) at week 4 and 1 point (range: 0–3 points) at week 26. Toxicities included skin/hair changes (93%) and non-significant QT prolongation (73%). Two patients died, including 1 patient who experienced hepatic failure after NSAID overdose and 1 patient who developed neutropenic sepsis. Electively stopping vemurafenib treatment resulted in relapse in 5 patients, and complete cessation was only possible for 1 patient. Digital droplet PCR for BRAF V600E using cell-free circulating DNA revealed that 7 patients had mutation statuses that fluctuated over time. Conclusion Our study confirms that vemurafenib treatment is safe and effective for young children with BRAF V600E+ multisystem LCH. However, treatment using vemurafenib does not completely eliminate the disease.