Abnormal Platelet Research Articles

Platelet abnormalities in autoimmune thyroid diseases: A systematic review and meta-analysis.

Some degree of platelet index abnormality has been found clinically in the autoimmune thyroid disease (AITD), but the findings are not uniform. The PubMed, Web of Science, Cochrane Library, and Embase databases were searched for relevant articles published up to August 16th, 2022, with no restrictions on the language of the articles. Reference lists of eligible articles were also searched. A random effect model was used to pool the standardized mean difference (SMD) and 95% confidence interval (95% CI) of platelet count (PLT), mean platelet volume (MPV), and platelet distribution width (PDW) between AITD patients and healthy controls, and subgroup analyses were performed. A total of 19 articles with 6173 people (3824 AITD patients and 2349 healthy people) were included in the meta-analysis. The results showed that PLT and MPV values were significantly increased in AITD patients when compared with healthy people (SMD: 0.164, 95% CI: 0.044 to 0.285; SMD: 0.256, 95% CI: 0.013 to 0.500), while no significant difference was found in PDW between the AITD group and the control group (SMD: 0.060, 95% CI: -0.164 to 0.284). Subgroup analysis according to disease type and thyroid function revealed that for PLT, this difference was only found in the Hashimoto's thyroiditis (HT) and hypothyroid groups, but not in the Graves' disease (GD) and hyperthyroid groups. For MPV, the results were the opposite of those for PLT: MPV was significantly higher in the GD, hyperthyroid, and euthyroid groups than in the control group, but not in the HT and hypothyroid groups. Sensitivity analysis showed that the stability of the pooled MPV was not good. No publication bias was found. PLT and MPV are significantly elevated in patients with AITD, with PLT being more significantly elevated in HT and hypothyroidism, and MPV being more significantly increased in GD and hyperthyroidism. Appropriate clinical attention can be paid to the thyroid function of patients when abnormal platelet indices are found, and conversely, the consequences of abnormal platelet parameters such as elevated MPV lead to an increased occurrence of cardiovascular events, which should also be addressed in the AITD population. https://www.crd.york.ac.uk/PROSPERO/, identifier CRD42022341823.

Efficacy and safety of phenobarbital for benign convulsions with mild gastroenteritis: A prospective randomized controlled study.

previous studies have shown that phenobarbital (PB) is a effective and safe drug in the treatment of benign convulsions with mild gastroenteritis (CwG), but there is a lack of large sample prospective randomized controlled study of different doses. This study was a prospective randomized controlled study on the efficacy and safety of different doses of phenobarbital for CwG. There has been no similar study. One hundred twenty CwG cases were included in this study. All of them were hospitalized in the Department of Neurology of Jiangxi Provincial Children's Hospital from January 2019 to August 2021. They were randomly divided into 10 mg/kg single dose group (Group A, n = 60) and 5 mg/kg single dose group (Group B, n = 60). The criteria for judging the efficacy of PB in our study were there was no convulsion in the course of acute gastroenteritis within 2 weeks after using PB. The effective rate was 93.33% in group A and 80.00% in group B. There was significant difference between the 2 groups (P < .05). Drowsiness was the most frequent adverse reaction. 14 cases in group A and 7 cases in group B had drowsiness. There was no significant difference between the 2 groups in the incidence of adverse events such as somnolence, ataxia, abnormal liver function, anemia, abnormal leukocyte, respiratory depression, cognitive impairment, rash, abnormal platelet and abnormal renal function (P > .05). All side reaction were transient. it is suggested that PB 10 mg/kg intravenously should be used as soon as possible for CwG, which has high effectiveness and safety.

Etiological Profiles of Patients With Thrombocytopenia in Central India: A Tertiary Centre Study.

Introduction Platelet-related disease may result from an abnormal platelet count, namely thrombocytopenia or thrombocythemia, or altered platelet function, and thus is associated with bleeding or with thrombotic manifestation. Thrombocytopenia is defined as a subnormal number of platelets i.e. less than 1,50,000/µL in the peripheral blood. It can lead to inadequate clot formation and increased risk of bleeding and is a common indication for bone marrow aspiration and biopsy. Methodology The study was a hospital-based prospective observational study from January 2019 to June 2020. All cases of thrombocytopenia which were diagnosed first on haematology analyser (platelet counts <150,000/µL) and confirmed subsequently by peripheral smear with/without bleeding manifestations due to thrombocytopenia were taken up for the study. The aims and objectives of this study were to find out the epidemiological spectrum and prevalence of thrombocytopenia according to age and sex along with the correlation of haematological and bone marrow findings of such patients. Result This study comprised 100 cases of thrombocytopenia, with acute leukaemia accounting for the majority (28/100), followed by dimorphic anaemia (15/100), megaloblastic anaemia (11/100), hypocellular marrow, infection, and other conditions.All cases (100%) displayed the clinical symptom of widespread weakness and pallor, which was followed by fatiguability (72%) and dyspnoea (48%). Many thrombocytopenic individuals also had lymphadenopathy and hepatomegaly, the last two least common appearances. Lymphadenopathy and hepatomegaly which were the last two least common presentations were present in a significant number of thrombocytopenic patients. Conclusion The study of bone marrow is helpful in the diagnosis of thrombocytopenia cases. Bone marrow examination is a simple, safe outpatient procedure and yields an impressive amount of diagnostically valuable data in a wide variety of disorders of thrombocytopenia.An evaluation of the patient's bone marrow unquestionably aids in the early diagnosis and treatment of their ailment.

Associations of arsenic exposure with blood pressure and platelet indices in pregnant women: A cross-sectional study in Wuhan, China

BackgroundEnvironmental inorganic arsenic (iAs) exposure is potentially related to abnormal blood pressure (BP) changes and abnormal platelet activation. However, limited epidemiological studies have explored the impacts of iAs exposure on platelet change mediated by BP, especially for pregnant women. ObjectivesOur purpose was to investigate the associations of arsenic exposure with blood pressure and platelet indices among pregnant women. MethodsThe present study population included 765 pregnant women drawn from a prospective birth cohort study in Wuhan, China, recruited between October 2013 and April 2016. Urine sampled in the second trimester were used to assess arsenic species concentrations. The relative distribution of urinary arsenic species was used to measure human methylation capacity. BP parameters and platelet indices originated from the medical record. We applied multivariable linear regression models to explore the cross-sectional relationships between urinary arsenic metabolites, BP parameters, and platelet indices. We utilized mediation analysis to investigate the impacts of arsenic exposure on platelet indices through BP as mediator variables. ResultsWe observed significant positive correlations between iAs and systolic BP (SBP), diastolic BP (DBP), and mean arterial pressure (MAP). Pregnant women with higher methylation capacity to metabolize iAs characterized by higher secondary methylation index (SMI) and total methylation index (TMI) had a more significant reduction in SBP, DBP, and MAP. Pregnant women with higher DBP and MAP had higher platelet counts (PLC). A decreased PLC was found in subjects wither higher SMI. Additionally, SMI was negatively linked to PLC mediated through MAP. ConclusionsObtained results suggested that higher methylation capacity to metabolize iAs might contribute to decreased PLC among pregnant women, and MAP might mediate the influence of SMI on PLC.

Identification of Prognostic Factors for Recurrence and Mortality in Patients With Acute Pulmonary Embolism.

This study aimed to explore prognostic factors for 1-year recurrence and mortality in patients with acute pulmonary embolism (APE). APE patients who attended the Emergency Department of Fujian Provincial Hospital from January 2016 to June 2020 were recruited. Univariate and multivariate logistic regression analyses were carried out to determine the prognostic factors for 1-year recurrence and mortality. A total of 458 APE patients were included, of whom 81 (17.69%) had recurrence, and 97 (21.18%) died. Multivariate logistic regression analyses revealed that smoke (OR: 1.949; 95% CI: 1.094-3.470; P = 0.023), abnormal platelet distribution width (OR: 3.013; 95% CI: 1.574-5.767; P = 0.001), and interrupted maintenance therapy (OR: 18.280; 95% CI: 9.777-34.179; P < 0.001) were significantly associated with an increased risk of 1-year recurrence in APE patients. Age ≥65 years (OR: 3.492; 95% CI: 1.876-6.500; P < 0.001), history of malignancy (OR: 7.190; 95% CI: 3.804-13.587; P < 0.001), history of long-term immobilization (OR: 6.244; 95% CI: 3.472-11.228; P < 0.001), mechanical ventilation (OR: 5.971; 95% CI: 3.154-11.304; P < 0.001), and interrupted maintenance therapy (OR: 2.414; 95% CI: 1.315-4.432; P = 0.004) were independent prognostic factors for 1-year mortality. The AUC of 1-year mortality and recurrence prediction models were 0.852 (95% CI: 0.805-0.898) and 0.868 (95%CI: 0.832-0.905). In patients with APE, history of smoking, abnormal PDW, and interrupted maintenance therapy were significantly associated with the risk of 1-year recurrence, while age ≥65 years, history of malignancy, history of long-term immobilization, mechanical ventilation, and interrupted maintenance therapy were independent prognostic factors for 1-year mortality.

Macrothrombocytopenia: Role of Automated Platelet Data in Diagnosis.

Inherited macrothrombocytopenia is an underdiagnosed condition and may result in misdiagnosis and inappropriate management. This research was done to study this condition in a hospital setting. This study was conducted over 6 months in a teaching hospital. Patients whose complete blood count (CBC) samples were sent to the hematology laboratory were included. Patients were suspected to have inherited macrothrombocytopenia according to pre-defined criteria. Demographic information, automated CBC and peripheral smear examination was carried out. Seventy five healthy individuals and 50 patients with secondary thrombocytopenia were also analyzed. Likely inherited macrothrombocytopenia was identified in 75 patients. Automated platelet count in these patients ranged from 26 × 10^9/L to 106 × 10^9/L while MPV ranged from 11.0 to 13.6 fL. There was significant difference (p < .001) in mean platelet volume (MPV) and platelet large cell ratio (P-LCR) amongst patients with likely inherited macrothrombocytopenia, those with secondary thrombocytopenia and the control group. Mean platelet diameter was significantly higher (3.5 ± 1.1μm) in patients with likely inherited macrothromboctopenia compared to those with secondary thrombocytopenia (2.4 ± 0.7μm) and control group (1.9 ± 0.7μm). All patients with suspected inherited macrothrombocytopenia showed abnormal platelet histograms with descending limb in the high volume and red cell zone. Four distinct histogram patterns were identified. Inherited macrothrombocytopenia is an underdiagnosed condition. The patient's history, clinical examination, judicious use of automated CBC data including platelet histograms and careful review of the peripheral blood smear are useful tools to suspect this condition. The online version contains supplementary material available at 10.1007/s12288-022-01590-6.

A STUDY ON HAEMATOLOGICALAND HEMOSTATIC ABNORMALITIES IN PATIENTS WITH CHRONIC LIVER DISEASE.

The Liver is the largest organ of the body. It plays a crucial role in maintaining hemostasis and keeping the hematological parameters within normal limits. Hematological abnormalities in patients with cirrhosis of the liver increase morbidity and mortality to the underlying illness. Consequently, it is vital to look into those abnormalities in a patient with liver disease. The purpose of the study was to determine the hematological and hemopoietic abnormalities in decompensated chronic liver disease patients to guide treatment decisions toward reducing mortality and morbidity. Objectives: To assess RBC; WBC; platelet abnormalities and the derangement of clotting factor secretion and function in patients of chronic liver disease; To know the type of anemia in patients with chronic liver disease. Materials And Methods: ACross-Sectional Observational Study was carried out at Narayana Medical College and Hospital for a duration of 18 months from February 2021 to October 2022, about 70 patients were randomly selected among inpatients Inclusion Criteria: All the patients with signs and symptoms of liver disease for more than 6 months of any etiology. Exclusion Criteria: Patients with Acute hepatic failure; Known case of any malignancy and with primary abnormalities in hemostatic functions; Patients suffering from chronic kidney disease, and coronary artery disease. Patients with a preexisting cause of anemia other than liver disease. Results: Most common type of anemia is Normocytic normochromic anemia. Leucopenia is less common than leukocytosis. Most of the patients had Thrombocytopenia, Prolonged PT, and APTT. Conclusion: From this study, we conclude that hematological alterations are very common in a patient with liver disease which needs to be identied and corrected early for a better clinical outcome

A STUDY ON HAEMOTOLOGICAL ABNORMALITIES IN CHRONIC LIVER DISEASE

Background:- Chronic liver disease ia an end stage disease and hematological abnormalities are common nding in it, adding morbidity to primary pathology and increase the mortality. The study was conducted to assess the hematological abnormalities and haemostatic derangements in CLD patients. Broadly , the hematological abnormalities are viewed under abnormalities in RBCs, WBCs, platelets and coagualation prole total 100 patients who fullled our Methods:- criteria were enrolled in our hospital , king George hospital, Visakhapatnam . patients were investigated after their oral and written consents wherever required. various Results :- hematological abnormalities encountered were normocytic normochromic anemia followed by microcytic anemia. Macrocytic anemia mostly in alcoholics. Leukocytosis was observed more than leucopenia and thrombocytopenia. prothrombin time and APTT were increased. Conclusion:- every chronic liver disease patients must be evaluated for hematological abnormalities and should be treated accordingly as early as possible to decrease mortality.

Comprehensive analysis of clinicalf eatures, treatment options, overall survival, and prognostic factors in lymphoma cell leukemia patients: A retrospective study.

Lymphoma cell leukemia (LCL) is regarded as patients presenting a high extensive lymphoma cell ratio in bone marrow (BM), which is recognized as lymphoma of stage IV by invading into BM. This study aimed to investigate the clinical characteristics, treatment options, survival profiles, and prognostic factors in patients with LCL. Clinical data of 42 patients with LCL were retrospectively reviewed, and baseline characteristics and treatment records were extracted. In addition, overall survival (OS) was calculated, and the causes of death were analyzed. Out of the 42 patients with LCL, 9 (21.4%) had primary BMLCL, 20 (47.6%) had Non-Hodgkin lymphoma (NHL) complicated with LCL, and 13 (31.0%) had NHL evolving into LCL. Common clinical characteristics included B syndromes (n = 21, 50.0%), abnormal white blood count (n = 28, 66.5%), decreased hemoglobin (n = 28, 66.7%), and platelet (n = 30, 71.4%). Additionally, elevated Eastern Cooperative Oncology Group (ECOG) with a score greater than one occurred in 26 patients (61.9%), and elevated lactate dehydrogenase (LDH) occurred in 25 patients (59.5%). For treatments, chemotherapy was the most common therapy (n = 35, 83.2%), followed by symptomatic treatment and radiotherapy plus chemotherapy. Additionally, the mean OS of the patients was 16.9 (95% CI: 12.8-20.9) months, among which primary patients with BMLCL showed shorter OS than those with NHL complicated with LCL and NHL evolving into patients with LCL. A total of 9 (21.4%) patients with LCL died during follow-up, among which the central nervous system (CNS) invasion was the most common cause of death. Furthermore, primary BMLCL, higher ECOG, and higher LDH were potential predictive factors for worse OS in patients with LCL. This study gives an overview of the treatment and prognosis of LCL, which provides additional information for the management of LCL.

Type 2 Diabetes Mellitus, Platelet Activation and Alzheimer's Disease: A Possible Connection.

Type 2 diabetes mellitus DM (T2DM) is associated with a 70% increased risk for dementia, including Alzheimer's disease (AD). Insulin resistance has been proposed to play a pivotal role in both T2DM and AD and the concept of "brain insulin resistance" has been suggested as an interpretation to the growing literature regarding cognitive impairment and T2DM. Subjects with T2DM present an abnormal platelet reactivity that together with insulin resistance, hyperglycaemia and dyslipidaemia effect the vascular wall by a series of events including endothelial dysfunction, oxidative stress and low-grade inflammation. Activated platelets directly contribute to cerebral amyloid angiopathy (CAA) by promoting the formation of β-amyloid (Aβ) aggregates and that Aβ, in turn, activates platelets, creating a feed-forward loop suggesting the involvement of platelets in the AD pathogenesis. Moreover, islet amyloid polypeptide deposition, co-localized with Aβ deposits, is a common finding in the brain of patients with T2DM. These observations raise the intriguing prospect that traditional or novel antiplatelet therapeutic strategies may alleviate fibril formation and could be used in the prevention or treatment of AD subjects with diabetes.

Dengue Fever with Corona Virus Disease 2019: Is it a “Double Trouble” with Concurrent Both Diseases or Single Disease with “Polyhedron” Nature and Antigenic Cross Reactivity?

Introduction: Corona virus disease 2019 (COVID-2019) disease caused by a novel coronavirus, severe acute respiratory syndrome coronavirus 2, is having propensity of pulmonary and extrapulmonary involvement. Although dengue virus has a predominant extrapulmonary effect, pulmonary involvement is less common. COVID-19–dengue overlap is a mixture of both diseases sharing few similarities. Methods: A multicentric observational study conducted from May 2021 to October 2021, in MIMSR Medical College and Venkatesh Hospital, Latur, India, included 300 COVID-19 cases with dengue NS1 or dengue immunoglobulin M (IgM) positive, with lung involvement documented and categorized on high-resolution computerized tomography (CT) thorax at the entry point. We have recorded demographic parameters as age, gender, comorbidity, and laboratory parameters such as total white blood cell count, platelet count, lactate dehydrogenase (LDH), C-reactive protein (CRP), interleukin-6 (IL-6), dengue serology, and use of BIPAP/NIV in COVID-19 cases in critical care settings in all study cases. CT severity scoring was done as per universally accepted standard mild if score < 7, moderated if score 8–15, and severe if score > 15. The final radiological outcome as presence or absence of fibrosis with clinical recovery was documented. All cases were subjected to dengue immunoglobulin G (IgG) antibody titers and dengue IgM/IgG antibody titer analysis after 12 weeks of discharge from the hospital after clinical recovery. Statistical analysis is done by using Chi-square test. Results: In a study of 300 COVID-19 pneumonia cases, COVID-19–dengue overlap was documented in 16.3% (49/300) of cases. Predominant age groups between 18–95 years and age ≥ 50 years were 60% (180/300), age < 50 were 40% (120/300). In the gender distribution in the study group, male population was 70.3% (211/300) and females were 29.7% (89/300). The main symptoms in the study group were shortness of breath in 79% of cases, fever in 71%, cough especially dry in 48%, and fatigability in 79%, tachycardia in 72%, tachypnea in 24%, and oxygen desaturation on 6 minutes walk in 29%. Positive dengue serology was documented as per CT severity scoring in 26/42 mild CT severity cases, 16/92 in moderate CT severity cases, and 7/166 in severe CT severity cases (P < 0.00001). Hematological parameters were having a significant association in COVID-19 cases with and without dengue overlap such as abnormal white blood cell count (P < 0.0076) and abnormal platelet count (P < 0.00001). Clinical parameters like hypoxia have a significant association in COVID-19 cases with and without dengue overlap (P < 0.00001). “Inflammatory markers” analysis such as interleukin 6 (IL-6) (P < 0.00001), CRP (P < 0.00001), and LDH (P < 0.00001) has documented significant association in COVID-19 cases with and without dengue overlap. In a study of 49 cases of COVID-19–dengue overlap, post-COVID lung fibrosis was documented in 1 case while 251 COVID-19 patients with negative dengue serology documented post-COVID lung fibrosis in 45 cases (P < 0.004). In a study of 49 cases of COVID-19–dengue overlap, actual serological assessment in dengue IgM/IgG and COVID-19 antibody titers was documented in significant association (P < 0.00001). Conclusion: COVID-19–dengue overlap is very frequently documented in tropical settings and disease of concern in critical care settings as the natural trend of this entity is different and has an impact on clinical outcome if diagnosis is delayed. COVID-19 pneumonia with dengue fever behaves like “two sides of the same coin” or the “polyhedron” nature of COVID-19 due to antigenic cross reactivity. Rationality for coexistent pathology is still undetermined.

Abnormal platelet immunophenotypes and percentage of giant platelets in myelodysplastic syndrome: A pilot study.

Myelodysplastic syndrome (MDS) is a heterogeneous hematopoietic stem cell disorder with thrombocytopenia. Flow cytometric immunophenotyping of blood cells has been instrumental in diagnosis as co-criteria, but the data regarding platelets remains lacking. This study aims to determine if there is a difference in surface antigen levels on platelets by comparing surface antigen levels in MDS patients and healthy control subjects. Concurrently, as flow cytometric gating can reveal the diameter of cells, this study will investigate differences in giant platelet percentage by comparing these percentages in high- and low-risk MDS patients. Twenty newly diagnosed MDS patients were enrolled in this study. Platelet surface antigen levels were determined by measuring the binding capacity of antibodies with flow cytometry. Platelets of MDS patients were shown to have a lower level of CD61 and higher levels of CD31 and CD36 than healthy controls. Judged by forward scatter (FSC), MDS patients' platelets appeared to be larger than those of healthy control subjects, whereas the MFI adjusted by diameter (MFI/FSC ratio) of CD31, CD41a, CD42a, CD42b and CD61 on platelets were lower in MDS patients than in healthy control subjects. There was a significant quantity of giant platelets found in MDS patients, and the high-risk MDS patients tended to have a higher percentage of giant platelets than low-risk patients. Conclusions: All the results indicate that MDS patients exhibit a lower antigen presentation (MFI) adjusted by diameter on platelets than healthy controls and the giant platelets detected by flow cytometry might correlate with the condition of MDS.

A familial case of MYH9 gene mutation associated with multiple functional and structural platelet abnormalities

Mutations in the MYH9 gene result in macrothrombocytopenia often associated with hemorrhages. Here, we studied the function and structure of platelets in three family members with a heterozygous mutation R1933X in the MYH9 gene, characteristic of closely related disorders known as the May-Hegglin anomaly and Sebastian syndrome. The examination included complete blood count, blood smear microscopy, platelet flow cytometry (expression of P-selectin and active integrin αIIbβ3 before and after activation), the kinetics of platelet-driven contraction (retraction) of blood clots, as well as scanning/transmission electron microscopy of platelets. Despite severe thrombocytopenia ranging (36–86) × 109/l, none of the patients had hemorrhages at the time of examination, although they had a history of heavy menstruation, spontaneous ecchymosis, and postpartum hemorrhage. Flow cytometry showed background platelet activation, revealed by overexpression of P-selectin and active αIIbβ3 integrin above normal levels. After TRAP-induced stimulation, the fractions of platelets expressing P-selectin in the proband and her sister were below normal response, indicating partial platelet refractoriness. The initiation of clot contraction was delayed. Electron microscopy revealed giant platelets with multiple filopodia and fusion of α-granules with dilated open canalicular system, containing filamentous and vesicular inclusions. The novel concept implies that the R1933X mutation in the MYH9 gene is associated not only with thrombocytopenia, but also with qualitative structural and functional defects in platelets. Platelet dysfunction includes impaired contractility, which can disrupt the compaction of hemostatic clots, making the clots weak and permeable, therefore predisposing patients with MYH9 gene mutations to the hemorrhagic phenotype.

Prevalence of Thrombocytopenia and Platelet Dysfunction in Women Delivering at a Tertiary Care Hospital and Prevalence of Platelet Abnormalities in their Neonates with Perinatal Outcomes: A Retrospective Study

Objective: To study the etiology and severity of platelet disorders, clinical manifestations, mode of delivery and neonatal outcome in pregnant women with thrombocytopenia and platelet dysfunction delivering in a tertiary care center in the Middle east. Methods: A retrospective cohort study was done on pregnant women admitted to Sultan Qaboos University Hospital (SQUH) from January 2011 to December 2020 with platelet abnormalities as per the registry were included. Thrombocytopenia associated with drugs, viral infection, pre-eclampsia, and HELLP syndrome were excluded. Results: Total number of deliveries were 39,522 during the study period. Of these 220 patients had thrombocytopenia and platelet abnormalities according to our inclusion criteria. The incidence of platelet abnormalities was 0.56% The mean gestational age at delivery was age 37.53 weeks. The mean platelet count was 90.41cells/dl. The highest two etiologies in this study were gestational thrombocytopenia (GT) (24.6%) and immune thrombocytopenic purpura (ITP) (22.6%). Out of 220, 9.5% were treated with prednisolone. There was a significant correlation between maternal platelet counts and medical treatment was received. No significant correlation between maternal platelet counts and mode of delivery was obtained. Nine neonates had platelet abnormalities, of which 44.4% were born to mothers with immune thrombocytopenic purpura (ITP). Mild to moderate neonatal thrombocytopenia was present in 66.6% of the neonates. 33.3% of the thrombocytopenic neonates had experienced hemorrhagic manifestations with good clinical outcome. Conclusion: The incidence of platelet abnormalities was 0.56% according to our study. ITP was more associated with severe thrombocytopenia and premature delivery.

Treatment of ursodeoxycholic acid with glucocorticoids and immunosuppressants may improve the long-term survival rate in primary biliary cholangitis patients.

Primary biliary cholangitis (PBC) is an autoimmune cholestatic liver disease. The clinical effectiveness of ursodeoxycholic acid (UDCA) plus glucocorticoids and/or immunosuppressants remains controversial in PBC patients. The study aimed to compare the efficacy of monotherapy and combination therapy in patients with PBC and to assess the factors affecting the efficacy. In this retrospective study, 266 patients diagnosed with PBC were divided into monotherapy group (UDCA), double therapy group (UDCA plus glucocorticoids or immunosuppressants), and triple therapy group (UDCA plus glucocorticoids and immunosuppressants) according to different treatments. Demographic characteristics, immune parameters, biochemistry profiles, and other indicators were evaluated at baseline, 6 months, and 1 year following treatment. The prognosis was evaluated using the Paris II standard. The liver transplant-free survival at 3, 5, 10, and 15 years was predicted by GLOBE score. All statistical analyses were conducted using SPSS (version 24) software (SPSS Inc, Chicago, IL). The long-term survival rate of the triple therapy group was significantly improved compared with the monotherapy group (P = .005). In addition, multivariate analysis showed that abnormal platelet count, alkaline phosphatase, and albumin levels were risk factors for poor response. When IgG levels were elevated but below twice the upper limit of normal, the clinical benefit was not significant compared with monotherapy (P > .05). Compared with monotherapy and double therapy, triple therapy may improve the long-term survival rate of PBC patients. Abnormal platelet count, alkaline phosphatase, and albumin levels were associated with a poor prognosis.

Brain-Derived Neurotrophic Factor (BDNF) Is Associated with Platelet Activity and Bleeding Tendency in Patients with Gaucher Disease.

Bleeding tendency, a prominent feature of patients with Gaucher disease (GD), is associated with abnormal platelet function. Brain-derived neurotrophic factor (BDNF) is a protein with neuroprotective potential stored in alpha granules of circulating platelets. Here we studied BDNF levels in 50 patients with type I GD (GD1) and their correlation with platelet activity and bleeding tendency. Flow cytometry was used to test unstimulated and stimulated measurement of platelet surface-activated expression of αIIbβ3 integrin, P-selectin and lysosomal-associated membrane protein (LAMP3/CD63). Serum and plasma BDNF levels were quantified using ELISA. The bleeding history was recorded by a bleeding questionnaire. Serum BDNF levels were positively correlated with platelet count and moderately correlated with unstimulated and stimulated platelet P-selectin expression. Patients with more than one bleeding manifestation were shown to have lower serum BDNF levels, albeit similar platelet count. Plasma BDNF levels were significantly elevated in splenectomized patients and showed a moderate positive correlation with stimulated platelet CD63 expression. These observations demonstrate the first association between BDNF levels in the peripheral blood with platelet dysfunction and increased bleeding manifestation. The role of measuring serum BDNF for assessing platelet alpha degranulation defects and bleeding risk in patients with GD and the general population needs further study.

Adsorptive cytapheresis for ulcerative colitis with focus on removing platelets and platelet-aggregates.

Platelet abnormalities and secondary thrombocytosis are clinical features of ulcerative colitis (UC) and seem to play a relevant role in pathogenesis. This work analyzed the adsorption characteristics of the adsorber Immunopure. A prospective study was performed to investigate the module in vitro with blood from healthy donors in a down-scaled recirculation model and in vivo in six patients suffering from UC. Furthermore, adsorber beads were investigated by immunofluorescence analyses. Apheresis was performed over 5 weeks at weekly intervals. In vitro as well as in vivo, the module showed a strong adsorption of platelets, monocytes, CD14+ CD16+ monocytes, neutrophils, and platelet leukocyte aggregates (PLAs). Five of the six patients benefited from the treatment (83%), and four (67%) went into remission. On average, the CAI was reduced by 6.4 points. Immunopure treatments improved the course of the disease and were well tolerated. The module strongly adsorbs platelets and platelet-aggregates.

Clinical features and prognostic factors of children with profound sudden sensorineural hearing loss.

To investigate the clinical features and factors affecting the prognosis of children with profound sudden sensorineural hearing loss (SSNHL). We retrospectively analyzed the clinical data of 147 children with profound SSNHL who received inpatient treatment at our department from January 2016 to January 2021. All children were administered with systemic steroid therapy and/or intratympanic steroid (ITS) treatment for 2 weeks. Statistical analyses were performed for the clinical features, treatment effectiveness, and factors affecting the prognosis using SPSS 23.0. The median age of the study population was 8 (6-10) years. The median treatment onset time was 8 (4-20) days. The most common concomitant symptom was tinnitus (45.58%). Laboratory findings showed that the percentages of children with abnormal leukocytes was 25.85%, abnormal platelet counts was 17.01%, abnormal cytomegalovirus IgG antibodies was 36.73% and abnormal Epstein-Barr (EB) virus IgG antibodies was 41.50%. The overall recovery rate of the treatment was 20.04%. The univariate analysis showed that age, treatment onset time, tinnitus, and ITS treatment were associated with the prognosis (p < 0.05). Regarding laboratory findings, the neutrophil count, lymphocyte count, and neutrophil-to-lymphocyte ratio differed significantly between the effective and invalid treatment effect groups (p < 0.05). The multivariable logistic regression analysis showed that treatment onset time [odds ratio (OR) = 0.936, 95% confidence interval (CI): 0.881-0.994] and ITS treatment (OR = 0.174, 95% CI: 0.044-0.0687) correlated with hearing recovery (p < 0.05). In this study, the earlier the treatment start time of children with profound SSNHL, the better was the prognosis. Further, ITS could be an effective treatment option.

Abnormal preoperative platelet count may predict postoperative complications following shoulder arthroplasty.

The purpose of this study is to investigate the association between preoperative platelet counts and postoperative complication rates within 30 days of total shoulder arthroplasty (TSA). The American College of Surgeons National Surgical Quality Improvement database was queried for all patients who underwent TSA between 2015 and 2019. The study population was then divided into 5 groups based on their preoperative platelet count: <100k (moderate-to-severe thrombocytopenia), 100-150k (mild thrombocytopenia), 150-200k (low-normal preoperative platelet count), 200-450k (normal, reference cohort), and >450k (thrombocytosis). Postoperative complications within 30 days of the TSA were collected. Multivariate logistic regression analysis was conducted to investigate the relationship between preoperative platelet counts and postoperative complications. A total of 19,721 patients undergoing TSA between 2015 and 2019 were included in this study. One hundred fifty-five patients (0.8%) had moderate-to-severe thrombocytopenia, 982 (5.0%) had mild thrombocytopenia, 3945 (20.0%) had a low-normal preoperative platelet count, 14,386 (72.9%) had a normal preoperative platelet count, and 253 (1.3%) had thrombocytosis. An increasing rate of overall complications was observed as the severity of thrombocytopenia progressed from low-normal (6.4%) to mild thrombocytopenia (10.2%) and to moderate-to-severe thrombocytopenia (18.7%). The overall complication rate of the thrombocytosis cohort was 14.6%. In comparison to normal platelet count, low-normal thrombocytopenia, mild thrombocytopenia, moderate-to-severe thrombocytopenia, and thrombocytosis were identified by multivariate analysis as significant predictors of overall complications (odds ratios [ORs] of 1.12, 2.15, 2.43, 2.71, respectively), postoperative anemia requiring transfusion (ORs of 1.63, 3.45, 5.97, 4.21, respectively), and minor complications (ORs of 1.39, 2.64, 3.40, 3.34, respectively). Minor complications include progressive renal insufficiency, urinary tract infection, transfusions within 72 hours after surgery, pneumonia, and superficial incisional surgical site infection. Increasing severity of thrombocytopenia correlated with higher overall postoperative complication rates following TSA. Interestingly, patients with thrombocytosis had the highest overall postoperative complication rates among all cohorts included in this study. Platelet counts are often the reflection of other comorbidities and a good indicator of patient's general health status. Long-term optimization of abnormal platelet counts may potentially reduce surgical complications.

GANGGUAN HEMOSTASIS PADA PENYAKIT GINJAL KRONIK DERAJAT 5

Patients with chronic kidney disease (CKD) develop hemostatic disorders mainly in the form of bleeding diatheses. Platelet dysfunction is the main factor responsible for hemorrhagic tendencies in advanced kidney disease. Anemia, dialysis, the accumulation of medications due to poor clearance, and anticoagulation used during dialysis have some role in causing impaired hemostasis in CKD patients. Platelet dysfunction occurs both as a result of intrinsic platelet abnormalities and impaired platelet-vessel wall interaction. The normal platelet response to vessel wall injury with platelet activation, recruitment, adhesion, and aggregation is defective in advanced renal failure. Dialysis may partially correct these defects, but cannot totally eliminate them. The hemodialysis process itself may in fact contribute to bleeding. Hemodialysis is also associated with thrombosis as a result of chronic platelet activation due to contact with artificial surfaces during dialysis.