The paper describes a case of autoimmune hemolytic anemia (AIHA) in a 27-year-old woman whose examination revealed mesenteric teratoma. AIHA was characterized by a hypertensive crisis and a temporary response to corticosteroid therapy that was complicated by the development of somatogenic psychosis and discontinued. A relapse of hemolysis developed 6 months later. The patient underwent laparoscopic splenectomy and removal of mesenteric root teratoma. Immediately after surgery, a hematological response was obtained as relief of hemolysis and restoration of a normal hemoglobin level. There is a sustained remission of AIHA for the next 16 months.
Stenosis of the iliac veins is common in patients with chronic venous insufficiency. The article describes the methods for the diagnosis and treatment of iliac vein stenosis. The causes of iliac vein stenosis include extravasal compression or the consequences of ileofemoral thrombosis. Stenosis of the iliac veins exists in 1/4 of the entire adult population, but clinical manifestations do not occur in all patients. Stenosis of the iliac veins should be considered in case of an unknown edema, more often in the left lower extremity, since venous duplex ultrasound of lower extremities is not sensitive and specific enough when examining the veins above the inguinal ligament. The most accurate diagnostic method is intravascular ultrasound (IVUS) but the appeared computed tomography angiography (CTA) and magnetic resonance angiography (MRA) with high-quality images have become a good replacement for IVUS. The main method of treatment of iliac vein stenosis, besides stenting, isindispensable drug therapy consisting of antithrombotic and phlebotonic drugs.
Background: Background:The clinical course of myeloproliferative neoplasms (MPN) is often complicated by impaired microcirculation, arterial and venous thrombosis. According to the literature, common for MPNs JAK2 gene mutations may contribute to the development of venous thrombosis. Even in the absence of overt MPN, a significant proportion of patients with recurrent cerebral and/or portal vein thrombosis carry the JAK2 V617F mutation. It is known that hereditary thrombophilic conditions, such as congenital deficiency of anticoagulants (antithrombin, protein C, and protein S) and genetic mutations (factor V Leiden, prothrombin G20210A, etc.), play an important role in the pathogenesis of venous thrombosis. However, limited data concerning the potential contribution of these mutations to the thrombotic risk in MPNs have been provided so far.
Introduction. Immune thrombocytopenia (ITP) is an autoimmune disorder characterized by isolated thrombocytopenia. Splenectomy is one the most effective treatment methods for this disorder.Aim — to evaluate the effi cacy and safety of a splenectomy in patients with primary immune thrombocytopenia.Subjects and methods. 111 patients (31 males, 80 females) with primary immune thrombocytopenia who were hospitalized to perform laparoscopic splenectomy were included in a prospective study conducted at the National Research Center for Hematology from 2015 to 2019. Disease duration from onset to splenectomy was from 1 month to 51 years. Response to the splenectomy, complications, and correlation with immediate preoperative platelet count were analyzed.Results. Complete response was achieved in 79 (71.2 %) cases, a partial response was achieved in 11 (9.9 %) cases, and in 21 (18.9 %) cases there was no response. Immediate preoperative platelet count was signifi cantly higher in patients with complete response in comparison with the group with no response, median (95% CI): 47 (35–58) vs 16 (9–20), p < 0.001.Multivariate analysis (logistic regression) was performed. According to this regression, a risk factor for an unfavorable response was detected — males > 60 years of age, p = 0.05; RR (95% CI): 2.0 (0.9–7.1). A predictor of unfavorable response was identifi ed – immediate preoperative platelet count < 23 × 109/l (cutoff point determined in ROC-analysis); p = 0.001, RR (95% CI): 2.5 (1.1–8.6). The probability of complete response was lower with the number of treatment lines prior to splenectomy (weak inverse correlation: r S = −0.30; p = 0.01). The frequency of postoperative complications was 12.6 %. According to our follow-up data, a complete response was preserved in 66/79 (83.5 %) of patients, with a follow-up of 2.7 years.Conclusion. Splenectomy is an effective and safe treatment method for ITP. Factors of unfavorable response were identifi ed: males > 60 years of age and immediate preoperative platelet count < 23 × 109/l. It is safe to perform splenectomy regardless of effectiveness of preoperative splenectomy treatment and platelet count.
Antigens of the immunological synapse (IS), particularly immune checkpoint proteins, have been of great interest for decades. These antigens can be very important characteristics of B-cell lymphoproliferative diseases (B-CLD). 1-3 Identification and understanding of the underlying differences in antigens of IS in B-CLD play a significant role in understanding how tumor cells avoid T-cell immunity. 4,5 The prognosis for different B-CLDs is varied. It can be suggested that tumor B cells in various B-cell neoplasms use different mechanisms to evade the immune response. Here, we present a study of IS antigens in B-CLD. The study included 48 patients with B-CLD who previously received no specific therapy. There were 26 patients diagnosed with chronic lymphocytic leukemia (CLL), 12-with mantle cell lymphoma (MCL), and 10-with splenic marginal zone lymphoma (SMZL). The median age of CLL patients was 59.5 years, MCL-56.5 years, and SMZL-60 years. CLL patients were divided into stages according to the Binet classification: A-10 patients, B-11, and C-5. MCL patients were divided according to the cytogenetic risk: intermediate-risk group included only t(11;14)-7 patients; unfavorable risk-del17p13 or p53 mutations (5 patients). Peripheral blood was collected in ethylenediaminetetraacetate tubes. Tumor cells and T cells were analyzed by flow cytometry (BD FACSCanto II (BD Biosciences, USA)). Monoclonal antibody panel for B-cell analysis included CD19 PE, CD5 PerCP-Cy5.5, CD80 FITC, CD279 (PD-1) FITC, CD86 APC, CD274 (PD-L1) PE-Cy7, CD95 (FAS)
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