Gray-zone lymphoma. Examples of rare clinical manifestation.

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.

Constrictive pericarditis in a patient with inherited factor VII deficiency.

Constrictive pericarditis (CP) is the final stage of a chronic inflammatory process characterized by fibrous thickening and calcification of the pericardium that impairs diastolic filling, reduces cardiac output, and ultimately leads to heart failure. We present a clinical case of CP in a patient with rare inherited bleeding disorder - factor VII deficiency. Heart failure due to CP was suspected based on clinical symptoms, results of ultrasonic and radiological investigations. The diagnosis was verified by the results of cardiac magnetic resonance imaging. Pericardectomy was performed resulting in significant improvement in the patient's condition.

[Progressive pulmonary hypertension in a patient with type 1 Gaucher disease]. / Progressiruiushchaia legochnaia gipertenziia u patsienta s bolezn'iu Goshe I tipa.

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 ß-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.

[Thrombotic events in patients with hemophilia]. / Tromboticheskie oslozhneniia u bol'nykh gemofiliei.

The paper describes 4 clinical cases of thrombotic events (pulmonary embolism, deep vein thrombophlebitis, acute myocardial infarction, ischemic stroke) that have occurred in patients with hemophilia. It discusses the possible causes of their development and methods for their prevention and treatment. Controlled natural hypocoagulation, in which the dose of an administered deficient factor decreases to such an extent that in order to maintain the safe level of hypocoagulation (plasma factor activity is 15-20%; activated partial thromboplastin time is 1.5-2 times normal values), is proposed as one of the treatment options.

[Diagnosis of early manifestations of myocardial dysfunction at early stages of antitumor treatment in patients with lymphogranulomatosis and lymphosarcomas].

AIM: To comparatively assess the capabilities of currently available instrumental studies in the diagnosis of early cardiac performance changes in patients with lymph tumors at different stages of treatment and to study the myocardial histomorphological pattern in relation to the intensity of the therapy performed (as evidenced by sectional studies). MATERIALS AND METHODS: 44 patients, including 26 with various types of lymphogranulomatosis (LGM) and 18 with lymphosarcomas were examined at different stages of antitumor treatment. Radionuclide equilibrium ventriculography (REVG), echocardiography (EchoCG), and electrocardiography (ECG) were used. Postmortem studies of the myocardial histological pattern were conducted in 20 patients (archive data). RESULTS: No significant pathological REVG, EchoCG, and ECG changes were found in 10 patients examined prior to treatment. In a group of 17 patients receiving a total dose of doxorubicine of 240 +/- 30 mg/m2, there was a significant decrease in diastolic duration, a reduction in diastolic volume, end systolic volume, stroke volume, stroke index, filling fraction over 1/3 diastole. In a group of 17 patients receiving a total dose of doxorubicine of 250 +/- 30 mg/m2 and radiotherapy applied to the mediastinum, the above changes were more marked. There were myocardial histomorphological changes whose magnitude progressed as therapy became more intensive. CONCLUSION. The findings have indicated that by using relatively small cumulative dose of anthracyclines, cardiovascular dysfunction can occur at the early stages of programmed treatment for LGM and lymphosarcomas. REVG has the greatest advantage in their detection.

[Plasma replacement in the treatment of ischemic heart disease]. / Plazmozameshchenie pri lechenii ishemicheskoi bolezni serdtsa.

AIM: To investigate whether plasma replacement (PR) can raise efficiency of drug therapy of ischemic heart disease (IHD). MATERIAL AND METHODS: 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. RESULTS: 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. CONCLUSION: Because PR is beneficial by many parameters, it lessens the requirement in pharmacological support of patients with complicated IHD.

[Atypical electrolytic disorders in patients with diseases of the blood system]. / Netipicheskie élektrolitnye narusheniia u bol'nykh s zabolevaniiami sistemy krovi.

AIM: To describe and analyze rare iatrogenic disturbances of water-electrolyte and acid-base balances in systemic blood diseases. MATERIAL AND METHODS: Five cases of life-threatening electrolytic imbalance resultant from therapy in patients with hemoblastoses and a patient with acute intermittent porphyria (AIP). RESULTS: Hyperkalemia arose in 2 patients treated with cyclosporin A and trimethoprim-sulfamethoxasole. One patient developed severe arrhythmia because of hypomagnesemia following long-term therapy with aminoglycosides and amphotericin B. In one AIP patient hyponatremia ending in coma was attributed to the disease activity and infusion therapy. This was the cause of coma. In a patient with acute leukemia and pneumonia amphotericin B therapy entailed distal renal tubular acidosis and marked dyspnea which was primarily mistaken for acute respiratory failure. Pathogenetic, diagnostic and therapeutic aspects of the above water-electrolyte defects are provided. CONCLUSION: The appearance of water-electrolyte imbalance in hemoblastosis patients calls up a detailed analysis of the going-on therapy whether the imbalance is iatrogenic.

[Effect of plasmapheresis with substitution by dextran solutions on hemostatic parameters in patients with ischemic heart disease]. / Vliianie plazmafereza s zameshcheniem dekctranovymi rastvorami na pokazatelei gemostaza u bol'nykh ishimicheskoi bolezn'iu serdtsa.

Plasmapheresis on a continuous blood flow fractionator to remove 1,200-2,000 ml plasma and substitute for the same values of dextran solutions was employed in the multi-modality treatment of 25 patients with Functional Classes II-IV exertional angina and myocardial infarction. Plasma and thrombocyte hemostatic parameters were examined just before and 24 and 48 hours after plasmapheresis. The analysis of the baseline data revealed that hemostatic changes were directly related to the severity of a disease in the patients and there was a correlation between the increased platelet aggregability and the elevated levels of fibrin-monomeric complexes. Plasmapheresis along with substitution of removed plasma for dextran solutions was accompanied by a profound hypocoagulative effect, as evidenced by plasma and thrombocyte hemostatic parameters. In addition, in patients with baseline high values of platelet aggregability plasmapheresis resulted in its decrease, whereas in those with baseline low values, it led to its increase.

[Exercise tolerance in patients with exertion angina pectoris after therapeutic plasmapheresis]. / Tolerantnost' k fizicheskoi nagruzke u bol'nykh so stenokardiei napriazheniia posle lechebnogo plazmafereza.

The bicycle ergometric test was used to examine exercise tolerance in 73 patients with coronary heart disease following a single remedial plasmapheresis session, which removed an average of 1562.2 ml plasma. A rapid increase in exercise tolerance was significantly revealed in patients with Functional Class IV exertional angina.