ТРАНСПЛАНТАЦИЯ ПЕЧЕНИ ПРИ ЭПИТЕЛИОИДНОЙ ГЕМАНГИОЭНДОТЕЛИОМЕ С КАНЦЕРОМАТОЗОМ БРЮШНОЙ ПОЛОСТИ: КЛИНИЧЕСКОЕ НАБЛЮДЕНИЕ

We report the case of a young woman who underwent liver transplantation for epithelioid hemangioendothe- lioma with diffuse intraperitoneal metastases. We observe the 1-year survival without tumor progression on everolimus-based immunosuppression. The different therapies and the results of surgery for this disease are discussed in short literature review. В статье представлен клинической случай трансплантации печени у молодой пациентки со злокачественной эпителиоидной гемангиоэндотелиомой печени и канцероматозом брюшной полости. Мы наблюдаем 1-летнюю выживаемость без признаков опухолевой прогрессии на фоне иммуносупрессии эверолимусом. В кратком литературном обзоре рассмотрены лечебные опции и результаты операций при данном заболевании.

LIVER TRANSPLANTATION FOR EPITHELIOID HEMANGIOENDOTHELIOMA WITH DIFFUSE INTRAPERITONEAL METASTASES: A CASE REPORT

We report the case of a young woman who underwent liver transplantation for epithelioid hemangioendothe- lioma with diffuse intraperitoneal metastases. We observe the 1-year survival without tumor progression on everolimus-based immunosuppression. The different therapies and the results of surgery for this disease are discussed in short literature review

Rare cases of laboratory tests discrepancies in diagnostics of pediatric Burkitt lymphoma/leukemia

Introduction. The main features of bone marrow blasts cells in Burkitt lymphoma/leukemia (BL) are L3 morphology, mature immunophenotype of blasts with surface IgM expression, and presence of typical MYC gene rearrangements.The aim of the study was to show discrepancy examples in laboratory signs of BL.Patients and methods. 10 patients (8 boys and 2 girls) aged 1 to 18 years were included in the present study. The inclusion criterion was the identification of discrepancies between flow cytometric, morphological and cytogenetic data.Results. In 2 cases there were no rearrangements of the MYC gene. In 2 patients, the L2 morphological variant went against the presence of typical MYC gene rearrangements. In one case, undifferentiated blasts cells were described by morphology together with presence of surface IgM, and atypical genetics. In 8 patients, there was no expression of surface IgM. Of these, patients with absence of cytomorphological data cytometric and genetic data were controversial.Сonclusion. The cases presented in this study and the cases described in the literature demonstrate the importance of an attentive and comprehensive approach in evaluating the results of laboratory tests in the diagnosis of BL

Results of external quality control study in flow cytometric acute lymphoblastic leukemia diagnostics

Comparison of interpretation of acute lymphoblastic leukemia (ALL) flow cytometric diagnostics data was the aim of the study. Immunophenotyping data obtained from 10 patients with ALL were analysed separately in 26 laboratories from Russian Federation and Kazahstan. Results comparison showed four main type of discordance: B-lineage ALL diagnostics during heavy bone marrow regeneration, great variability of T-ALL interpretation, complexity of ambiguous lineage acute leukemia and, finally, very different report types, unique for each laboratory. All these problems are the serious obstacles for standardization of flow cytometric ALL diagnostics in multicenter setting. Continuation of similar QC rounds following by consecutive discussions with further development of consensus diagnostic algorithm could be the first step for standardization of ALL immunophenotyping in Russian Federation and CIS countries