Search

Mediastinal gray-zone lymphoma (MGZL, lymphoma with features intermediate between classical Hodgkin lymphoma and diffuse large B-cell lymphoma) was declared as a separate entity in WHO classification of Tumors of Haematopoetic and Lymphoid Tissues in 2008 and 2017 years. Despite of similar pathomorphological characteristics between primary mediastinal B-cell lymphoma and Hodgkin lymphoma, clinical features and optimal therapeutic approach to MGZL are not clearly defined. Usually MGZL manifests with mediastinal lymphadenopathy, although extranodal lesions often occur (grey-zone lymphoma, GZL). Patients with MGZL have unfavorable prognosis, taking into account high rate of relapse. This article describes two cases of MGZL. First case manifested by arrhythmias due to primary heart involvement. In spite of cardiac failure antracycline-containing chemotherapy (6 courses of R-DA-EPOCH) it allowed to achieve a complete remission and resolving of arrhythmias. Second case was represented by metachronous tumors: primary mediastinal B-cell lymphoma at the time of disease onset and classical Hodgkin lymphoma, NS II, diagnosed after disease progression. Thus, we demonstrated two examples of MGZL that differ by clinical manifestation, response to chemotherapy, which emphasizes an importance of pathogenesis studying, and using of new therapeutic approaches.

Gaucher disease is the most common form of hereditary enzymopathies combined into a group of lysosomal storage diseases. The basis for the disease is a hereditary deficiency of the activity of acid β-glucosidase, a lysosomal enzyme involved in the catabolism of lipids, which results in the accumulation of nonutilized cellular metabolism products in the macrophage lysosomes. The main clinical manifestations of type 1 Gaucher disease are cytopenia, hepatomegaly, and splenomegaly, and bone lesion. One of the atypical clinical manifestations of Gaucher disease is damage to the lungs with the development of pulmonary hypertension, which is usually considered within the underlying disease - the development of pneumosclerosis due to macrophage dysfunction. The paper describes a case of progressive pulmonary hypertension in a patient with type 1 Gaucher disease.Болезнь Гоше - наиболее частая форма наследственных ферментопатий, объединенных в группу лизосомных болезней накопления. В основе заболевания лежит наследственный дефицит активности кислой β-глюкозидазы - лизосомного фермента, участвующего в катаболизме липидов, что приводит к накоплению неутилизированных продуктов клеточного метаболизма в лизосомах макрофагов. Основные клинические проявления болезни Гоше I типа: цитопения, гепато- и спленомегалия, поражение костей. Одним из нетипичных клинических проявлений болезни Гоше служит поражение легких с развитием легочной гипертензии, обычно рассматриваемое в рамках основного заболевания - развития пневмосклероза вследствие нарушения функции макрофагов. Приведено описание случая прогрессирующей легочной гипертензии у пациента с болезнью Гоше I типа.

To investigate whether plasma replacement (PR) can raise efficiency of drug therapy of ischemic heart disease (IHD).A 30-60% replacement of circulating plasma for salt or dextran using PF-05 unit has been performed in 324 patients 35-79 years of age with recurrent myocardial infarction and angina pectoris. A total of 520 PR procedures were performed. Biochemical, acid-base, coagulative, viscosity, microcirculatory blood parameters were taken, ECG and stress tests were made.PR resulted in a significant reduction in packed cell volume, total protein, fibrinogen, low density lipoproteins, total cholesterol and led to diminution of blood viscosity, acceleration of capillary blood flow, improvement of O2/CO2. Lowering of fibrinogen levels, number of platelets and their aggregation, enhancement of fibrinolytic blood activity created conditions for moderate controlled hypocoagulation. As shown by stress tests, two weeks after PR 60% of anginal patients of functional class IV can be transferred to class III.Because PR is beneficial by many parameters, it lessens the requirement in pharmacological support of patients with complicated IHD.