Introduction. Intravascular large B-cell lymphoma is a rare variant of large B-cell, highly invasive extranodal tumors of the lymphatic system. The pathogenesis of the disease lies in the ability of tumor cells to penetrate into small vessels and capillaries of various organs. The clinical presentation is atypical for diffuse large B-cell lymphoma. In the relevant literature, information on the diagnosis and treatment of this pathology is extremely rare, therefore each publication makes a significant contribution to expanding the horizons of hematologists and morphologists.Aim – to present a case of diagnosing intravascular B-cell lymphoma.Main findings. A clinical case of a 78-year-old patient who fell ill acutely is presented. At the onset of the disease, febrile fever was noted. In the general blood test: hemoglobin – 104 g/L; erythrocytes – 3.0 × 1012/L; ESR – 24 mm/h; platelets – 112 × 109/L, leukocytes – 4.9 × 109/L, 4 % of cells with lymphoblast morphology were found in the leukocyte formula. Blood serum tests revealed: an increase in uric acid concentrations – up to 639 μmol/L, LDH – up to 1885 U/L, beta-2-microglobulin – up to 8.9 mmol/L, C-reactive protein – up to 0.6 g/L, a decrease in the concentration of total protein – up to 45 g/L, an increase in the concentration of aspartate aminotransferase – up to 48 units/L at normal concentrations of bilirubin and alanine aminotransferase.The histological and immunohistochemical picture, according to the study of bone biopsy, most corresponded to bone marrow damage by intravascular large B-cell lymphoma. Immunophenotyping was carried out – 15.7 % of blast cells with immunophenotype CD19+HLA/DR+CD24+CD37+CD20+CD10+IgM+ were detected. Cytogenetic studies revealed no karyotype abnormalities. The result of fluorescence in situ hybridization of the IGH locus (14q32) was normal. Based on the data obtained, the final clinical diagnosis was established: diffuse large B-cell lymphoma, stage IVB, intravascular variant with bone marrow involvement, aggressive course. The patient was prescribed the first line of therapy according to the R-CHOP scheme (rituximab, cyclophosphamide, vincristine, prednisolone). In the control study of the bone marrow, after the first course of therapy, the number of lymphoid elements was 3.6 %, laboratory parameters returned to normal.
The pronounced progress in the achievement of full remission (in acute myeloid leukemia 58,8%, in acute lymphoblastic leukemia 66%) is revealed in studies of the results of randomizational use of intensive programs of polychemotherapy of 23 patients with acute leukemia. The intensification of the programmed treatment is connected with a high risk of complications. For their decrease the adequate maintaining therapy and the treatment in aseptic wards become necessary.
Адрес для переписки:Исаева Наталья Васильевна ФГБУН «Кировский научно-исследовательский институт гематологии и переливания крови» Федерального медико-биологического агентства 610027, Россия, Киров, Красноармейская ул., 72. Тел.: 8 (8332) 54-51-83. Факс: 8 (8332) 54-97-31.
Введение. Тромбоцитопения является частым осложнением онкогематологических заболеваний, которое провоцирует развитие геморрагического синдрома. Уровень тромбоцитов в периферической крови, являющийся пороговым для переливания концентрата тромбоцитов (КТ), окончательно не установлен. Применение тромбоэластографии (ТЭГ) при тромбоцитопениях у онкогематологических больных может повысить прогностические возможности определения риска кровотечений. Цель исследования: оценить вязко-эластические свой ства сгустка крови при тромбоцитопении менее 50×109/л и их взаимосвязь с проявлениями геморрагического синдрома у онкогематологических больных. Материалы и методы. В исследование включено 84 пациента с гемобластозами и тромбоцитопенией менее 50×109/л, 54 (64%) мужчины и 30 (36%) женщин, медиана возраста — 54 года. В зависимости от уровня тромбоцитов в периферической крови пациентов разделили на 2 группы: группа 1 — 44 пациента с числом тромбоцитов от 25 до 50×109/л, группа 2 — 40 больных с содержанием тромбоцитов менее 25×109/л. Исследовали показатели периферической крови, коагулограммы и ТЭГ. Степень тяжести кровотечений оценивали по шкале проявлений геморрагического синдрома (ВОЗ) и по шкале оценки побочных явлений Национального института рака (США). Результаты. Гипокоагуляция по ТЭГ в группе 1 выявлена в 46% случаев, в группе 2 — в 75%. Значимые межгрупповые различия показателей ТЭГ получены только в отношении максимальной амплитуды. Геморрагический синдром в группе 1 диагностирован у 6 (13%) пациентов, в группе 2 — у 14 (35%). Однако предиктивная роль влияния количества тромбоцитов на частоту развития геморрагического синдрома не установлена (β = 0,016; p = 0,92). Сочетание тромбоцитопении и гипокоагуляции по ТЭГ проявлялось в виде геморрагического синдрома в 3,3 раза чаще, чем без гипокоагуляции (RR = 3,3; 95% ДИ = 1,20–9,05). В ряде случаев основанная на результатах ТЭГ трансфузионная тактика позволила избежать переливания КТ при инвазивных манипуляциях у больных с тромбоцитопенией менее 25 × 109 / л. Заключение. Глубина тромбоцитопении не является надежным предиктором геморрагического синдрома. Применение ТЭГ у пациентов с тромбоцитопенией позволяет оптимизировать трансфузионную тактику, избежав ненужных переливаний КТ. Сочетание тяжелой тромбоцитопении с гипокоагуляцией по данным ТЭГ служит показанием для профилактических трансфузий КТ. Background. Thrombocytopenia is a frequent complication of hemoblastosis that causes hemorrhagic syndrome. The threshold of peripheral blood platelet count for transfusion of platelet concentrate is discussed. Thromboelastography (TEG) can be considered as additional prognostic method for bleeding risk assessment in thrombocytopenic patients with leukemia. Objectives: to study changes in the viscoelastic properties of blood clot in hemoblastosis patients with thrombocytopenia less than 50×109/L and their relationship with the manifestations of hemorrhagic syndrome. Patients/ Methods. The study included 84 hemoblastosis patients — 54 (64%) men и 30 (36%) women with thrombocytopenia less than 50 × 109/L, median age — 54 years. All patients are divided into 2 groups according to the platelet count: Group 1 — 25–50×109/L, Group 2 — less than 25×109/L. Peri pheral blood counts, TEG and coagulogram parameters were evaluated in both groups. Manifestations of hemorrhagic syndrome were assessed according to the WHO bleeding scale and Common terminology criteria for adverse events of the National Cancer Institute (USA). Results. Hypocoagulation according TEG in Group 1 was found in 46% of cases, in Group 2 — in 75%. Significant intergroup differences in TEG parameters were obtained only in maximum amplitude. Hemorrhagic syndrome in Group 1 diagnosed in 6 (14%) patients, in Group 2 — in 14 (35%). There was no dependence of hemorrhagic syndrome frequency on the severity of thrombocytopenia (β = 0.016; p = 0.92). The combination of thrombocytopenia and hypocoagulation according TEG manifested as a hemorrhagic syndrome 3.3 times more often than without hypocoagulation (RR = 3.3; 95% CI = 1.20–9.05). In some cases, TEG-based transfusion tactics allowed to avoid unnecessary platelet concentrate transfusion during invasive procedures in patients with thrombocytopenia less than 25 × 109/L. Conclusions. Severity of platelet depletion is not accuracy predictor for hemorrhagic events. Use of TEG in patients with thrombocytopenia allows to optimize the transfusion tactics and to avoid unnecessary platelet concentrate transfusions. The combination of severe thrombocytopenia with hypocoagulation according TEG serves as indication for prophylactic platelet concentrate transfusion.
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