-
Internal Medicine (Tokyo, Japan) 2024Thrombocytopenia, anasarca, fever, renal dysfunction, and organomegaly (TAFRO) syndrome is an inflammatory disorder with an unclear pathogenesis. We herein report a case...
Thrombocytopenia, anasarca, fever, renal dysfunction, and organomegaly (TAFRO) syndrome is an inflammatory disorder with an unclear pathogenesis. We herein report a case of TAFRO syndrome in remission in a patient who experienced recurrent intracranial bleeding despite a normal platelet count and coagulation system. A further investigation suggested the presence of anti-glycoprotein VI (GPVI) autoantibodies in the plasma, which induced platelet dysfunction and bleeding tendency. No new bleeding or relapse of TAFRO syndrome occurred after immunosuppressive therapy was initiated. These findings may help elucidate the autoimmune pathogenesis of TAFRO syndrome.
Topics: Humans; Autoantibodies; Recurrence; Syndrome; Platelet Membrane Glycoproteins; Cerebral Hemorrhage; Thrombocytopenia; Fever; Female; Middle Aged; Male; Blood Platelet Disorders
PubMed: 38945933
DOI: 10.2169/internalmedicine.2799-23 -
Transfusion Medicine (Oxford, England) Jun 2024Regulatory requirement of fixed holding time (6 h) of whole blood (WB) at room temperature, that is, 22-24°C (RT) results in sub-optimal component separation. The aim...
BACKGROUND AND OBJECTIVES
Regulatory requirement of fixed holding time (6 h) of whole blood (WB) at room temperature, that is, 22-24°C (RT) results in sub-optimal component separation. The aim was to evaluate the platelet concentrates (PC) prepared by both platelet rich plasma (PRP) and buffy coat (BC) methods after overnight hold (18-24 h) at RT.
MATERIALS AND METHODS
A prospective experimental study was performed. A total of 48 WB units collected were divided into four groups (12 each) control-1 (C1) and test-1 (T1) for PRP and control-2 (C2) and test-2 (T2) for the BC method. Control groups were processed within 6 h, and in test groups, components were prepared after overnight hold, followed by evaluation of quality parameters.
RESULTS
Irrespective of the method used, all PCs had similar volume, platelet yield, swirling, no bacterial contamination, RBC contamination, PaO and PaCO levels. PCs in the T1 group had significant differences in glucose and MPV values on d1, which were resolved by d5 of storage. PCs in T2 has significant differences in pH, glucose, and MPV levels throughout storage. PRBC in test and control groups had similar quality parameters till d42 of storage. FFPs in all tests were noninferior to the concurrent control groups till 3 months of storage.
CONCLUSION
Overnight holding of WB had no lasting deleterious changes. Though a few biochemical parameters in the test groups were significantly different, they can be accepted to improve the logistics of component separation. Overall PRP method seemed to have a better result than the BC method after an overnight hold.
PubMed: 38945170
DOI: 10.1111/tme.13064 -
Thrombosis Research Jun 2024Dysregulated host response to infection causes life-threatening organ dysfunction. Excessive inflammation and abnormal blood coagulation can lead to disseminated...
INTRODUCTION
Dysregulated host response to infection causes life-threatening organ dysfunction. Excessive inflammation and abnormal blood coagulation can lead to disseminated intravascular coagulation (DIC) and multiple-organ failure in the late sepsis stages. Platelet function impairment in sepsis contributes to bleeding, secondary infection, and tissue injury. Platelet transfusion is considered in patients with sepsis with DIC and bleeding; however, its benefits are limited and of low quality. Fibrinogen plays a crucial role in platelet function, and establishing a fibrin network binds to activated integrin αIIbβ3 and promotes outside-in signaling that amplifies platelet functions. However, the role of fibrinogen in sepsis-induced platelet dysfunction remains unclear.
MATERIALS AND METHODS
We evaluated the effects of fibrinogen on platelet hyporeactivity during septic shock in adult male Wistar rats using lipopolysaccharide (LPS) injection and cecal ligation and puncture (CLP) surgery. Changes in the hemodynamic, biochemical, and coagulation parameters were examined. Platelet activation and aggregation were measured using whole-blood assay, 96-well plate-based aggregometry, and light-transmission aggregometry. Additionally, platelet adhesion, spreading, and fibrin clot retraction were evaluated.
RESULTS
Rats with LPS- and CLP-induced sepsis displayed considerable decreases in plasma fibrinogen levels and platelet aggregation, adhesion, spreading, and clot retraction. The aggregation of platelets obtained from rats with sepsis was markedly augmented by fibrinogen supplementation. Additionally, fibrinogen administration improved platelet adhesion, spreading, and clot retraction in rats with sepsis.
CONCLUSIONS
Fibrinogen supplementation could serve as a potential therapeutic intervention for alleviating platelet hyporeactivity in patients with sepsis and bleeding.
PubMed: 38945093
DOI: 10.1016/j.thromres.2024.109072 -
Mymensingh Medical Journal : MMJ Jul 2024Chronic non-healing ulcers present significant challenges in diabetic, dermatological and surgical patients. Platelet-Rich Plasma (PRP), enriched with bioactive factors,... (Observational Study)
Observational Study
Chronic non-healing ulcers present significant challenges in diabetic, dermatological and surgical patients. Platelet-Rich Plasma (PRP), enriched with bioactive factors, offers promise for wound healing enhancement. The prospective observational study was done in the Department of Transfusion Medicine at AIIMS Bhopal for a period of two years, from April 2020 to 2022. This study evaluates PRP's efficacy, prepared via the single spin method, in non healing chronic ulcers. Aseptically, 100 ml of blood was drawn into CPDA (citrate-phosphate-dextrose-adenine) bags. PRP was prepared by centrifuging blood at 3400 rpm for 10 minutes, yielding PRP and buffy coat. Patient serum and 10.0% calcium gluconate were added to fibrin gel. PRP was injected around the ulcer and then dressed. Dressings were changed on the 5th, 15th and 20th days with PRP. The evaluation was done on day 30 using surface area and volume assessments. Thirteen patients aged 18-65 participated. The patients treated with single spin PRP (four females, nine males) had mean hemoglobin of 10.79±2.22gm/dL. Initial lesions (14.72cm²) significantly reduced to (8.78cm²) after application of single Spin PRP sessions (p=0.005). The PRP showed a mean platelet count of 1,527.00±192.26 × 10⁹/L. Ulcer surface area decreased from 9.87cm² to 7.56cm²; lesion volume reduced from 14.72cm³ to 8.78cm³. Platelet count differences between whole blood and PRP were significant (p<0.05). The single-spin PRP method exhibited considerable improvements in healing parameters, showcasing its potential for chronic ulcer management.
Topics: Humans; Platelet-Rich Plasma; Female; Male; Middle Aged; Prospective Studies; Adult; Wound Healing; Aged; Chronic Disease; Adolescent; Young Adult; Skin Ulcer
PubMed: 38944737
DOI: No ID Found -
Blood Reviews Jun 2024Immune thrombocytopenia (ITP) is an autoimmune bleeding disease caused by immune-mediated platelet destruction and decreased platelet production. ITP is characterized by... (Review)
Review
Immune thrombocytopenia (ITP) is an autoimmune bleeding disease caused by immune-mediated platelet destruction and decreased platelet production. ITP is characterized by an isolated thrombocytopenia (<100 × 10/L) and increased risk of bleeding. The disease has a complex pathophysiology wherein immune tolerance breakdown leads to platelet and megakaryocyte destruction. Therapeutics such as corticosteroids, intravenous immunoglobulins (IVIg), rituximab, and thrombopoietin receptor agonists (TPO-RAs) aim to increase platelet counts to prevent hemorrhage and increase quality of life. TPO-RAs act via stimulation of TPO receptors on megakaryocytes to directly stimulate platelet production. Romiplostim is a TPO-RA that has become a mainstay in the treatment of ITP. Treatment significantly increases megakaryocyte maturation and growth leading to improved platelet production and it has recently been shown to have additional immunomodulatory effects in treated patients. This review will highlight the complex pathophysiology of ITP and discuss the usage of Romiplostim in ITP and its ability to potentially immunomodulate autoimmunity.
PubMed: 38942688
DOI: 10.1016/j.blre.2024.101222 -
Medicine Jun 2024To analyze maternal and neonatal effects of placental abruption (PA) through a novel classification in the presence of hypertension. Initial hemoglobin parameters were... (Comparative Study)
Comparative Study Observational Study
To analyze maternal and neonatal effects of placental abruption (PA) through a novel classification in the presence of hypertension. Initial hemoglobin parameters were also compared to predict pregnancy outcomes in addition to hypertension. This retrospective cohort designed study was conducted on 115 pregnant women with PA. The main parameters scanned and recorded from the hospital database and patient medical files. Two groups were classified regarding of presence or absence of hypertension (53 hypertensive, 62 normotensive). Maternal demographical and clinical characteristics (abdominal pain, vaginal bleeding) were recorded. APGAR scores below 5 at 1st and 5th minute, fetal or neonatal death, admission and length of stay in Neonatal Intensive Care Unit were also investigated and compared between the groups. Stillborn to live-born ratio and lower APGAR scores < 5 at 5th minute were significantly higher in hypertensive group than normotensive group (P = .006 and 0.047, respectively). Poor maternal outcomes were detected in the hypertensive group than normotensive group regarding rate of blood transfusion (27/53, 50.9%; 18/62, 29%, respectively, P = .017). More abdominal pain and less vaginal bleeding were seen in PA with HT. Higher lymphocyte count, mean platelet volume, and platelet distribution width were reported in hypertensive group. Poorer maternal and neonatal outcomes of hypertensive patients with PA were detected. These patients should deserve greater attention to assess not only the possible risks associated with abruption but also the accompanying complications.
Topics: Humans; Female; Pregnancy; Retrospective Studies; Adult; Abruptio Placentae; Pregnancy Outcome; Infant, Newborn; Apgar Score; Hypertension, Pregnancy-Induced; Hypertension
PubMed: 38941372
DOI: 10.1097/MD.0000000000038633 -
Frontiers in Oncology 2024Infections represent one of the most frequent causes of death of higher-risk MDS patients, as reported previously also by our group. Azacitidine Infection Risk Model...
INTRODUCTION
Infections represent one of the most frequent causes of death of higher-risk MDS patients, as reported previously also by our group. Azacitidine Infection Risk Model (AIR), based on red blood cell (RBC) transfusion dependency, neutropenia <0.8 × 10/L, platelet count <50 × 10/L, albumin <35g/L, and ECOG performance status ≥2 has been proposed based on the retrospective data to estimate the risk of infection in azacitidine treated patients.
METHODS
The prospective non-intervention study aimed to identify factors predisposing to infection, validate the AIR score, and assess the impact of antimicrobial prophylaxis on the outcome of azacitidine-treated MDS/AML and CMML patients.
RESULTS
We collected data on 307 patients, 57.6 % males, treated with azacitidine: AML (37.8%), MDS (55.0%), and CMML (7.1%). The median age at azacitidine treatment commencement was 71 (range, 18-95) years. 200 (65%) patients were assigned to higher risk AIR group. Antibacterial, antifungal, and antiviral prophylaxis was used in 66.0%, 29.3%, and 25.7% of patients, respectively. In total, 169 infectious episodes (IE) were recorded in 118 (38.4%) patients within the first three azacitidine cycles. In a multivariate analysis ECOG status, RBC transfusion dependency, IPSS-R score, and CRP concentration were statistically significant for infection development ( < 0.05). The occurrence of infection within the first three azacitidine cycles was significantly higher in the higher risk AIR group - 47.0% than in lower risk 22.4% (odds ratio (OR) 3.06; 95% CI 1.82-5.30, < 0.05). Administration of antimicrobial prophylaxis did not have a significant impact on all-infection occurrence in multivariate analysis: antibacterial prophylaxis (OR 0.93; 0.41-2.05, = 0.87), antifungal OR 1.24 (0.54-2.85) ( = 0.59), antiviral OR 1.24 (0.53-2.82) ( = 0.60).
DISCUSSION
The AIR Model effectively discriminates infection-risk patients during azacitidine treatment. Antimicrobial prophylaxis does not decrease the infection rate.
PubMed: 38939343
DOI: 10.3389/fonc.2024.1404322 -
HLA Jun 2024HLA-B*40:86 differs from B*40:06:01:03 by a single nucleotide exchange in exon 3.
HLA-B*40:86 differs from B*40:06:01:03 by a single nucleotide exchange in exon 3.
Topics: Humans; Alleles; Base Sequence; East Asian People; Exons; Histocompatibility Testing; HLA-B40 Antigen; Polymorphism, Single Nucleotide; Sequence Analysis, DNA
PubMed: 38936820
DOI: 10.1111/tan.15491 -
Cytokine Jun 2024As a versatile element for maintaining homeostasis, the chemokine system has been reported to be implicated in the pathogenesis of immune thrombocytopenia (ITP)....
As a versatile element for maintaining homeostasis, the chemokine system has been reported to be implicated in the pathogenesis of immune thrombocytopenia (ITP). However, research pertaining to chemokine receptors and related ligands in adult ITP is still limited. The states of several typical chemokine receptors and cognate ligands in the circulation were comparatively assessed through various methodologies. Multiple variable analyses of correlation matrixes were conducted to characterize the correlation signatures of various chemokine receptors or candidate ligands with platelet counts. Our data illustrated a significant decrease in relative CXCR3 expression and elevated plasma levels of CXCL4, 9-11, 13, and CCL3 chemokines in ITP patients with varied platelet counts. Flow cytometry assays revealed eminently diminished CXCR3 levels on T and B lymphocytes and increased CXCR5 on cytotoxic T cell (Tc) subsets in ITP patients with certain platelet counts. Meanwhile, circulating CX3CR1 levels were markedly higher on T cells with a concomitant increase in plasma CX3CL1 level in ITP patients, highlighting the importance of aberrant alterations of the CX3CR1-CX3CL1 axis in ITP pathogenesis. Spearman's correlation analyses revealed a strong positive association of peripheral CXCL4 mRNA level, and negative correlations of plasma CXCL4 concentration and certain chemokine receptors with platelet counts, which might serve as a potential biomarker of platelet destruction in ITP development. Overall, these results indicate that the differential expression patterns and distinct activation states of peripheral chemokine network, and the subsequent expansion of circulating CXCR5 Tc cells and CX3CR1 T cells, may be a hallmark during ITP progression, which ultimately contributes to thrombocytopenia in ITP patients.
PubMed: 38936205
DOI: 10.1016/j.cyto.2024.156684 -
Zhongguo Shi Yan Xue Ye Xue Za Zhi Jun 2024To explore the efficacy and safety of haploidentical hematopoietic stem cell transplantation combined with umbilical cord blood infusion for the treatment of aplastic...
OBJECTIVE
To explore the efficacy and safety of haploidentical hematopoietic stem cell transplantation combined with umbilical cord blood infusion for the treatment of aplastic anaemia in children.
METHODS
Nine cases of children with aplastic anaemia treated with umbilical cord blood combined with haploidentical hematopoietic stem cell transplantation at the People's Hospital of Henan University of Chinese Medicine from January 1, 2021 to September 15, 2023 with a median age of 11(2-13) years and a median follow up of 18(7.5-21) months were included, and the clinical data were retrospectively analyzed. Hematopoiesis reconstitution, the incidence of graft-versus-host disease(GVHD), infections and survival of the patients were analyzed.
RESULTS
All 9 children were successfully implanted. The median time to neutrophil and platelet implantation was 11.11±1.27 d and 12.44±3.36 d, respectively. One case developed acute gastrointestinal GVHD of degree I, which was improved after treatment, and the patient developed superficial gastritis and chronic gastrointestinal GVHD at a later stage, which is currently under clinical follow-up. Acute GVHD of II-IV degree was 0%. Hemorrhagic cystitis in 3 cases, CMV infection in 5 cases and bacterial and fungal infections in 5 cases improved with symptomatic treatment.All 9 children demonstrated complete donor chimerism within 1 month after transplantation, at two years of follow-up, all nine children survived without recurrence or development of grade II-IV GVHD, and there were no children with transplant-related deaths.
CONCLUSION
Haploidentical hematopoietic stem cell transplantation combined with umbilical cord blood transfusion for aplastic anaemia in children has a low incidence and mild degree of GVHD, with significant efficacy, and can be used as a therapeutic option for children without an HLA full donor chimeric match.
Topics: Humans; Anemia, Aplastic; Child; Hematopoietic Stem Cell Transplantation; Child, Preschool; Graft vs Host Disease; Retrospective Studies; Adolescent; Cord Blood Stem Cell Transplantation; Fetal Blood; Transplantation, Haploidentical; Male; Female
PubMed: 38926985
DOI: 10.19746/j.cnki.issn.1009-2137.2024.03.037