Background: Epidemiological data in Haemophilia A (HA) report an improvement in haemophilia care over the last 50 years; the life expectancy of these patients now approaches that of the general population but evidence-based guidelines for the acute management and secondary prophylaxis of cardiovascular diseases in haemophilia patients are still lacking, Aims: We report the surgical and haematological strategies used in a mild haemophilia A patient who successfully underwent endovascular aortic aneurysm repair (EVAR) because very little is known about how to handle the increased risk of bleeding associated with invasive procedures and antithrombotic agents Methods: Case report: To our knowledge, about six other haemophilic patients treated with open surgery or EVAR were reported in the literature Results: A 64-year-old man with mild-moderate HA (F8:C 6.9%), without inhibitor was referred for treatment of a symptomatic infrarenal aortic aneurysm that had enlarged from 5.6 cm to 6 cm in diameter in 6 months. Among the personal antecedents: Acute Coronary Syndrome (1997), allergy to FVIII Concentrate of plasmatic origin, traumatic amputation of right lower limb and Arterial Hypertension on treatment.He are VIH positive on antiretroviral triple therapy. The patient was successfully treated with EVAR, Femoro-femoral bypass and right common iliac artery embolization with Coils (Endurant 32 * 14 mm) associated with perioperative administration of titrated doses of recombinant full length 3rd generation factor VIII concentrate (Octocog Alfa, Advateâ, Takeda Lab)(rCF8), administered on bolus The surgery was carried out under general anaesthesia and anticoagulation with bolus of unfractionated heparin(UFH)(Heparin Leoâ 5000 UI) administered inmediately before the surgery and after the perioperative administration of a bolus of aproximately 55 UI by kilogram of Advateâ to achieve a secure level of FVIII presurgery (105 %). 3 hours after the first bolus, an intraoperative control of FVIII was performed (using a chromogenic technique) and a new bolus of rCF8 was administered (aprox 20 UI by kilogram). At the end of surgery the UFH was reversed with protamine sulfate in a 1:1 ratio. To reduce the risk of hematoma, local dry weight and cold were applied after the withdrawal of the introducers. In the first 48 h post he received rCF8 to maintain minimum level of 80% and after, every 12 h/7 days (minimum level 50%) and another 7 days, every 24 h (minimum level 30%), performing antithrombotic prophylaxis (Enoxaparin sodium 40 mg / day) and concomitant antiaggregant therapy(Acetylsalicylic acid 100 mg / day, 2 months).The determination of the postoperative levels of FVIII was carried out by one stage coagulative assay (ACL Top). There were no perioperative complications and he was discharged at day 14, performing the determination of inhibitor at 15 days and 2 months after surgery, being negative (< 0.4 Bethesda units) Summary/Conclusion: This case report shows that EVAR is a relatively safe procedure in haemophilic patients by mean...

Мета. Узагальнити показання до спленектомії у хворих з В-лімфопроліферативною неоплазією і оцінити її ефективність. Матеріали і методи. Спленектомію виконано у 41 хворого з хронічною лімфоцитарною лейкемією (ХЛЛ), у 46 хворих з лімфомою маргінальної зони селезінки (ЛМЗС), у 27 хворих з волосистоклітинною лейкемією (ВКЛ). Результати. Спленектомія за наявності сумнівів дає змогу діагностувати варіант лімфоїдної неоплазії. Результатом операції є видалення значного об’єму пухлини, усунення абдомінальної компресії, припинення гемолізу, нормалізація числа тромбоцитів, зниження або зникнення потреби в цитостатичній терапії. Безпосереднього позитивного результату досягнуто у 92,7% хворих з ХЛЛ, у 95,7% хворих з ЛМЗС, у 81% хворих з ВКЛ. Висновки. Спленектомію при лімфоїдній неоплазії виконують за діагностичними і/або лікувальними показаннями. Спленектомія з лікувальною метою показана за наявності резистентної до цитостатичної терапії масивної спленомегалії з явищами абдомінальної компресії, гемоцитопенії імунного або гіперспленічного характеру, регіональної портальної гіпертензії.

Background: Chronic lymphocytic leukemia (CLL) is a neoplasm composed of monomorphic small mature B cells that coexpress CD5 and CD23. The finding of ≥55% prolymphocytes defines B-cell prolymphocytic leukemia (B-PLL), and cases with 15-55% of the prolymphocytes called atypical CLL (aCLL, previously called CLL/PL). Aims: The aim of this study was evaluation of the clinical and prognostic significance of aCLL. Methods: We reviewed the medical records, peripheral blood, and bone marrow findings of 121 patients with untreated CLL (n = 101), aCLL (n = 9), and B-PLL (n = 11) between January 1995 and June 2018. CLL and aCLL patients were classified as Binet stage A (<3 areas of lymphadenopathy, hemoglobin >10 g/dL, platelets >100k/mL), B (≥3 areas of lymphadenopathy, hemoglobin >10 g/dL, platelets >100 k/mL), or C (hemoglobin <10 g/dL or platelets <100k/mL). All patients underwent immunohistochemistry and/or flow cytometric immunophenotyping, among them; 107 patients underwent karyotyping, and 15 patients underwent fluorescent in situ hybridization. Results:The median age at diagnosis was 63.5 (range 25-85) years, 68.0 (40-77) years, and 66.0 (29-78) years and the ratio of males to females was 2.0, 2.0, and 1.8 in CLL, aCLL, and B-PLL, respectively. Lymphadenopathy was more common in CLL (42%, 42/101) and aCLL (56%, 5/9) than in B-PLL (0%), whereas splenomegaly was more in B-PLL (100%) than CLL (25%, 25/101) and aCLL (33%, 3/9). (P = 0.683 and 0.010, respectively). aCLL showed more severe anemia, elevated lactate dehydrogenase, and b2-microglobulin than CLL and B-PLL (P = 0.001, 0.027, and 0.037, respectively). Binet stage A and B were more in CLL (51% and 26%) than in aCLL (30% and 0%), whereas Binet stage C was more in aCLL (70%) than CLL (23%). (P = 0.013). Patients with B-PLL had an atypical immunophenotype with high frequencies of CD5 or CD23 negativity, FMC7 positivity, and strong CD22 positivity (P = 0.672, 0.440, 0.004, and <0.001, respectively). Especially in this study, patients with aCLL showed higher frequencies of FMC7 positivity and strong CD22 positivity than CLL in Western study (P = 0.032 and <0.001, respectively). In B-PLL, normal karyotype was less common and complex karyotype was more common than CLL and aCLL (P = 0.028). In the CLL group, cytogenetic abnormalities were observed in 35% of patients (33/94). The descending order of frequency was trisomy 12 (11%, 10/94), 13q deletion (10%, 9/94), complex karyotype (7%, 7/94), 11q deletion (5%, 5/94), 14q deletion (2%, 2/94), and 17p deletion (1%, 1/94). In the aCLL group, cytogenetic abnormalities were present in 50% of patients (4/8), including 3 cases of trisomy 12, 2 cases of 14q deletion, 1 case of 13q deletion, 11q deletion, 17q deletion and complex karyotype. In the B-PLL group, cytogenetic abnormalities were observed in 80% of patients (8/10), of whom 4 had complex karyotypes. The overall survival rate at 10 years were 65.6%, 22.2%, and 46.3 % in patient with CLL, aCLL, and B-PLL, respectively (P = 0.155). However, only OS of CLL and aCLL showed ...

Background. Modern immunochemotherapy (ICT) and infusion therapy (IT) in the treatment of malignant lymphomas, further supportive and concomitant therapy require constant and reliable vascular access. Today, the hematology clinic uses both short-term and minimally invasive methods of vascular access (venipuncture, peripheral catheters) and long-term options (peripherally inserted central catheter, subclavian vein catheterization). The choice of the optimal method of access to vessels, its preservation and care, and the avoidance of complications associated with the functioning of such access require the joint efforts of many specialists. Objective. To outline the indications for the establishment, advantages and disadvantages of the use of implanted port systems (IPS) in hematological patients. Materials and methods. 8 patients with newly diagnosed malignant lymphomas and established IPS who received volumetric and long-term IT during antilymphoma treatment were observed. 7 patients were diagnosed with primary mediastinal large B-cell lymphoma (PMBCL), and 1 patient was diagnosed with Hodgkin’s lymphoma (HL). Gender distribution was the following: 7 women and 1 man. Age of patients ranged from 26 to 48 years (median – 29.8 years). Patients were implanted with one of the two IPS available on the Ukrainian market (U-port 6.6/8.4 Fr or B-Braun Celsite® ST301 6.5/8.5 Fr). Catheterization of the right or left subclavian vein was performed in the operating room using local anesthesia and under X-ray control. IPS began to be used immediately after implantation. Results and discussion. Patients who were scheduled for long-term and volumetric infusions for the treatment of malignant lymphomas, or who had problems with short-term vascular access, were suggested to have IPS. As a result 7 patients with PMBCL received ICT, which involved a continuous 96-hour infusion of antilymphoma drugs and concomitant IT, the total volume of which was 5.0-6.5 liters per day. In 2 patients before the initiation of ICT there were significant obstacles to the establishment of short-term vascular access (phlebitis, postphlebotic changes in peripheral veins), another 1 patient had similar problems after the second course of ICT. The patient with HL at the time of chemotherapy had a poor condition of peripheral veins, which did not allow their frequent and prolonged catheterization, and the mode of application of antilymphoma drugs (every 2 weeks, 12 injections) made it inexpedient to catheterize the subclavian vein. Eventually, the presence of IPS has greatly simplified the permanent vascular access and care for the port system itself. In all of our patients, IPS ensured the continuity of the infusion and the planned volume. In the intercourse period, IPS did not require special care: patients followed their normal lifestyle, including taking a shower. Subsequently, in the long term (up to 24 months) IPS appeared to be functionally complete and with regular care (every 2-4 weeks) allowed for maintenance and concomitant therapy. One patient had a hematoma in the pocket area of the reservoir of the port system, which resolved spontaneously. No infection around the port area and no cases of catheter induced sepsis were observed. To date, IPS has been removed without complications in 5 patients, one continues ICT, and in another one IPS was decided not to be removed. Conclusions. The use of IPS in hematological patients has shown its benefits in long-term and large-scale ICT and IT regimens to ensure persistent, multiple and safe access to blood vessels. The use of IPS is also indicated for patients with vascular lesions, venous diseases or their unsatisfactory condition. The medical staff avoids the constant search for “working vessels” for the placement of peripheral catheters or special care for the central catheter. IPS provides the opportunity to conduct initial, supportive and concomitant therapy for months with minimal care for vascular access. Complications during the installation and operation of IPS are extremely rare and minor. IPS placement can be considered an integral part of ICT and IT in the hematological practice.

Objective. To present the immediate results of the splenectomy and preferable variants of transfusion therapy performance in patients with malignant non-Hodgkin’s lymphoma (MNHL). Materials and methods. 109 splenectomies were performed in patients with MNHL at the Department of General and Hematological Surgery of the institute from 1987 to 2020. The surgery was conducted by upper middle laparotomy under general anesthesia with intubation and, in particular cases, under spinal anesthesia. Results and discussion. The indications for splenectomy in patients with MNHL were as follows: massive splenomegaly, abdominal syndrome, associated hemocytopenia, inefficacy of cytostatic therapy, absence of diagnosis. All patients underwent vaccination against capsular bacteria for prevention of post-splenectomy infection in 10-14 days prior to the surgery. In case of anemia, which has been observed in 55 % of patients, the RBC concentrate was applied. All the patients, who received corticosteroid hormones prior to splenectomy, were administered prednisolone and hydrocortisone in the amount of 3 mg/kg of body mass at similar doses in an hour before the surgery for prevention of adrenal insufficiency during the surgery. The patients, who did not receive those medications, were also intramuscularly administered prednisolone at a dose of 0.5 mg/kg of body mass in an hour before the surgery for the same aim. The patients with PLT value <150.0×109/L were administered 1-2 doses of PLT concentrate immediately before the laparotomy. M-gradient was found in blood serum of 3 patients prior to the surgery. They underwent courses of therapeutic plasmapheresis due to the risk of intraoperative hemorrhage. 2 patients with hyperleukocytosis (WBC >80.0×109/L) underwent two courses of leukapheresis. The patients with concomitant regional portal hypertension and in case of manipulations close to the pancreatic tail were administered somatostatin drugs in the course of the splenectomy. The splenectomy proved to be effective in 100 (92 %) of patients with MNHL: the great tumor mass was removed, the abdominal syndrome and concomitant hemocytopenia were neutralized, the signs of hypersplenism ceased, the hemolysis ceased, the cytostatic therapy became less necessary or unnecessary, the final diagnosis was established. The most serious postsurgical complications were acute adrenal failure (n=3), postsurgical intra-abdominal hemorrhage (n=2), pancreonecrosis (n=6). The postsurgical lethality was 2.7 %. Conclusions. The splenectomy proved to be effective in 92 % of patients with MNHL. The infusion therapy is individual for each patient and may include transfusion surgeries if indicated. The main objective of the infusion therapy in patients with MNHL is prevention and elimination of intra- and postsurgical complications.