Abstract
Introduction: Histiocytosis is a heterogeneous group of rare diseases of unknown etiology. LCH is characterized by an abnormal proliferation of histiocytes (activated dendric cells and macrophages). Langerhans cell histiocytosis (LCH) is the most common form of histiocytosis, it is a potentially fatal diseases. Early detection of LCH plays an important role in its prognosis and outcome. However, the role of advanced methods of nuclear medicine in diagnosis of LCH is still to be researched. We have long-term experience in observing pediatric patients with LCH. Taking into the account the difficulty of diagnostic task for bone scintigraphy in identifying lytic bone destruction, we stated the following objective of the study.Purpose: Retrospective and prospective analysis of bone scintigraphy examinations of treatment-naive pediatric patients with LCH and calculate the diagnostic efficacy of bone scintigraphy. Material and methods: We analyzed 60 examinations of treatment-naive pediatric patients with proven case of LCH (2014-2019). The scanning was performed using whole body mode, 3 hours after intravenous injection of bone-seeking radiopharmaceutical 99mTc-MDP on Symbia E, T2 (Siemens, Germany). The median age was 5.6 years.Results: During examination we visualized 88 lesions with pathological level of accumulation of radiopharmaceutical in 60 patients. 84 with high level of accumulation (>120 %); 3 with slightly increased level of accumulation (100–120 %) and 1 with lower than normal level of accumulation (<100 %). The median level of accumulation of radiopharmaceutical was 268 %. Max level of accumulation was 1422 % (patient with subtotal involvement of femoral bone). Min — 60 % (patient with lytic destruction in orbital bone). During X-Ray we found out 97 pathological focuses of bone lytic destruction. In 3 patients with polyostotic form we found extra focuses on scintigraphy, which were confirmed with following examination and X-Ray. 8 lesions were not found on scintigraphy in 8 patients.Сonclusions: We evaluated diagnostic accuracy of bone scintigraphy with 99mTc-MDP in treatment-naïve pediatric patients with proven case of LCH. Sensitivity, specificity, NPV and PPV — 91.6, 50.0, 11.1, 98.6 %, respectively.
Highlights
Histiocytosis is a heterogeneous group of rare diseases of unknown etiology
Bar-Sever Z описывает успешную визуализацию костных очагов поражения у пациента с Лангергансоклеточный гистиоцитоз (ЛКГ) при помощи сцинтиграфии с 201Tl-хлоридом [20]
Information about the authors: Krylov A.S., http://orcid.org/0000-0002-8476-7879 Ryzhkov A.D., http://orcid.org/0000-0002-9571-801X Kaspshik S.M., http://orcid.org/0000-0002-1384-9551 Krylova M.A., https://orcid.org/0000-0002-4147-0389 Stanyakina E.E., https://orcid.org/0000-0002-0949-0320 Dolguyshin M.B., http://orcid.org/0000-0003-3930-5998
Summary
Результаты: У 60 пациентов при остеосцинтиграфии было визуализировано 88 очагов в костях с патологическим уровнем накопления РФП. Для цитирования: Крылов А.С., Рыжков А.Д., Каспшик С.М., Крылова М.А., Станякина Е.Е., Долгушин М.Б. Роль остеосцинтиграфии в диагностике первичных пациентов детского возраста с лангергансоклеточным гистиоцитозом. Выделяют более распространенную группу гистиоцитозов из клеток Лангер ганса, а также существенно реже встречающиеся гистиоцитозы нелангергансового типа и злокачественные гистиоцитарные заболевания [1]. Роль остеосцинтиграфии в диагностике первичных пациентов детского возраста. При развитии заболевания наблюдается избыточное накопление клеток Лангерганса в различных частях тела, с образованием опухолевых узлов и поражением органов. В различных исследованиях собрана информация, указывающая, что поражение костей происходит примерно у 78 % пациентов с ЛКГ и часто с вовлечением черепа (49 %), бедренных и костей таза (23 %), костей голени (17 %) и ребер (8 %).
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