The paper focuses on Epstein-Barr virus (EBV)-associated infl ammatory pseudotumor in the spleen, an extremely rare disease which so far remained undefi ned in the Russian literature. The morphology of it is not characterized by any specifi c features and is represented by a spindlecell component with pronounced infl ammatory infi ltration which complicates diff erential diagnosis. Previously this nosology was regarded within a group of the so-called infl ammatory pseudotumors due to the similarity of the clinical course and radiological presentation with tumor processes. Today the term "infl ammatory pseudotumor" is considered obsolete, since as pathogenesis was being studied within this disease group, the individual forms with similar morphology and diff erent histogenesis were identifi ed. Diff erential diagnosis in the context of additional diagnostic techniques is based in such cases on a wide range of reactive states, benign and malignant tumors.
In the past decade, a notable advance has been made in the understanding of the pathogenesis of NK/T-cell lymphomas; however, their diagnosis remains difficult because of their rarity and clinical and morphological variabilities. The paper generalizes the ten-year experience of the Hematology Research Center, Ministry of Health of Russia, in diagnosing and treating hepatosplenic T-cell lymphoma (HSTL), considers the problems of differential diagnosis with other hematological diseases occurring with similar clinical and laboratory symptoms, and lays down current approaches to the diagnosis and treatment of this condition. A clinician's view of the problem of diagnosis and treatment of this disease is given. HSTL is shown to be a heterogeneous group of diseases differing in a T-cell receptor chain gene rearrangement, the clinical course of the disease, and overall survival (OS). According to our data, 3-year OS was 12%; the median survival was 26 months. Two-year OS for γδ and αβ HSTL was equal to 25 and 70%, respectively. The difference in OS for the variants of HSTL failed to reach statistical significance (because the sample might be insufficient).
РЕФЕРАТАктуальность. В-клеточная лимфома селезенки неклас-сифицируемая -редкая и малоизученная нозологическая форма, впервые введенная в классификацию опухолей кро-ветворной и лимфоидной тканей ВОЗ в 2008 г. Эта форма лимфомы предполагает проведение дифференциальной диагностики между вариантным волосатоклеточным лей-козом (ВКЛ) и диффузной мелкоклеточной В-клеточной лимфомой красной пульпы селезенки (ДЛКПС). Цель. Разработать критерии диагностики ДЛКПС путем сопоставления трепанобиоптатов костного мозга и опера-ционного материала селезенки. Методы. В патологоанатомическом отделении ГНЦ МЗ РФ проведено сопоставление трепанобиоптатов костного моз-га и операционного материала селезенки (2013-2015 гг.) у 71 пациента (мужчины/женщины 1:2,6, возрастной диапазон 44-81 год, медиана 58 лет) с использованием морфологи-ческого и расширенного иммуногистохимического исследо-вания. В целях анализа мутационного статуса IgHV и выяв-ления мутаций MAP2K1, NOTCH, BRAF использован метод секвенирования по Сэнгеру, а также ПЦР-исследование. У 5 пациентов на образцах операционного материала селе-зенки проведено молекулярное исследование. Результаты. У 5 (7 %) из 71 пациента установлен диагноз ДЛКПС. В двух группах пациентов (с нормальным и высо-ким числом лейкоцитов) в ткани селезенки морфологиче-ская картина была сходной с опухолевым субстратом ВКЛ. При иммуногистохимическом исследовании во всех слу-чаях отмечалась мономорфная экспрессия опухолевыми клетками CD20, DBA.44 при вариабельности экспрессии CD11c, TRAP, CD103, CD123. Ни в одном из 5 наблюдений не выявлено экспрессии CD25, CD27, Cyclin D1, Annexin-1. В костном мозге в отличие от ВКЛ и его вариантной формы отмечалась преимущественно интерстициально-внутрисо-судистая скудная CD20+ лимфоидная инфильтрация (4 из 5 случаев) без различимых ядрышек в ядрах небольших лимфоидных клеток. В 1 наблюдении лимфоидная инфиль-трация носила смешанный характер -CD20+ мелкооча-гово-интерстициальная с внутрисосудистым компонентом. [2013][2014][2015] were compared in 71 patients (men/women ratio 1:2.6, age range 44-81, median age 58 years) using morphological and extended immunohistochemical studies. Sanger sequencing and PCR assay were carried out to analyze the mutational status of IgHV and to identify mutations in MAP2K1, NOTCH, BRAF. Results. SDRPL was diagnosed in 5 (7 %) of 71 patients. In 2 groups of patients (with normal and high WBC count), the morphological features of spleen tissue were similar to those of a neoplastic substrate of HCL-V. The immunohistochemical assay demonstrates monomorphic expression of CD20 and DBA.44 and heterogeneous expression of CD11c, TRAP, CD103, CD123 in all cases. In none of the 5 cases, expression of CD25, CD27, Cyclin D1, Annexin-1 was found. In bone marrow (unlike HCL and HCL-V), predominantly interstitial and intravascular scant CD20+ lymphoid infiltration (4 of 5 cases) was found without detectable nucleoli in nuclei of small lymphoid cells. In 1 case, there was a combined lymphoid infiltration: CD20+ microfocalinterstitial infiltration with an intra...
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