Aim: to analyze the first neurological manifestation in patient with thrombotic thrombocytopenic purpura (TTP). Material and methods. The appeals from physicians who suspected TTP were included in the study. The neurological symptoms in 40 patients with TTP were also analyzed. Results. Among 208 physician appeals about the suspicion of TTP and the referral of plasma samples for testing of ADAMTS13 activity, there were 2% of neurologists. The diagnosis of TTP was confirmed in 40 patients. 9 (22.5%) had no neurological symptoms, and 31 (77.5%) had neurological disorders. Patients with neurological symptoms were older than patients without neurological symptoms (median 37 years and 31 years, p = 0.006). Neurological symptoms were less frequently observed in pregnancy-associated TTP (p = 0.008). Among neurological symptoms there were speech disorders (40%), headache (30%), cognitive deficits (32.5%), depression of consciousness (22.5%), paresis (25%), sensation disorders (20.5%), seizures (10%). Magnetic resonance imaging/computed tomography (MRI/CT) revealed ischemic stroke in 47.5%, hemorrhagic stroke in 15%. In 6 patients, despite neurological symptoms, no pathology was detected with MRI/CT. In 4 patients, in the absence of neurological symptoms, MRI/CT revealed ischemic foci in the brain. 8 patients died: 2 patients died from sepsis, 6 - from ischemic stroke. In surviving patients, neurological symptoms regressed after remission. Conclusion. Neurological symptoms are detected in most patients with TTP. Brain MRI/CT should be performed in all patients with TTP
Introduction. Pregnancy is one of the most frequent triggers of congenital and acquired forms of thrombotic thrombocytopenic purpura (TTP).Aim — to develop tactics for the treatment of pregnant women with TTP.Results. TTP was associated with pregnancy in 55.5 % of all cases of TTP in women. In 5 pregnancies in which the diagnosis of TTP was known before pregnancy, or established in the early stages, TTP was treated throughout the pregnancy. In the congenital form, plasma transfusions were performed once every two weeks until the 20th week of pregnancy, or weekly, if thrombocytopenia < 150 × 109 /L persisted. Plasma transfusions were performed weekly after the 20th week with the goal of achieving clinical remission. With acquired TTP, glucocorticosteroids and plasma exchanges were used as treatment, in 1 case — rituximab, with the aim of achieving a clinical remission and an ADAMTS13 activity > 20 %. In this group of pregnant women, 1 caesarean section was performed and there were 4 cases of vaginal deliveries, with a total of 5 children being born with an average Apgar score of 7.5. In 7 pregnancies in which TTP fi rst manifested late, leading to a delayed diagnosis, preventive and curative measures were not carried out before delivery. In this group there were 2 abortions of pregnancy, 5 surgical deliveries, 3 cases of preeclampsia, 3 acute cerebral circulatory disorders, 1 intraabdominal bleeding, 1 case of acute renal failure, with two women undergoing mechanical ventilation. There was 1 case of antenatal fetal death, with a total of 4 children being born, who were assessed on the Apgar scale with an average of 5 points.Conclusion. Timely diagnosis as well as ongoing therapeutic and preventive measures help to avoid complications during childbirth in pregnant women with TTP.
The article defines reference values for activated partial thromboplastin time, Quick’s value, INR, thrombin time, fibrinogen, antithrombin and II, V, VII, VIII, IX, X, XI and XII coagulation factors, according to existing standards on the automated Sysmex CS‑2000i analyzer.The aim of the study. To determine reference values for routine and specific parameters of the hemostasis, which may vary depending on the type of analyzer and utilized reagents.Materials and methods. After receiving informed consent from donors for medical survey and blood donation, blood samples were obtained from 100 healthy donors: 64 (64%) males и 36 (36%) females. We established reference values with the Sysmex CS‑2000i (Sysmex, Japan) hemostasis analyzer and reagents from Siemens (Siemens Healthcare, Germany).Results. The data obtained were compared with the literature data and the data presented in the instructions for the reagents used. The results obtained for activated partial thromboplastin time (23.59–35.69 sec), fibrinogen (1.67–3.59 g/l) and antithrombin (67.65–114.89%) are comparable to the available data. There are no data on other studied parameters of hemostasis for the Sysmex CS‑2000i analyzer and the reagents used in the work. The obtained reference intervals are consistent with the recommendations of the manufacturer.Conclusions. Reference values vary significantly depending on the analytical systems and reagent kits used, which confirms the need for local derivation or validation of reference intervals for each specific analytical system and in each laboratory.
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