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1.
J Inflamm Res ; 17: 4895-4904, 2024.
Article in English | MEDLINE | ID: mdl-39070134

ABSTRACT

Background: Severe fever with thrombocytopenia syndrome (SFTS) is an emerging epidemic infectious disease with high mortality rate. This study aimed to investigate the association of red blood cell distribution width (RDW) and mortality risk in hospitalized SFTS patients. Methods: Clinical data of SFTS patients was retrospectively collected from three hospitals between October 2010 and August 2022. Cox proportional hazards model was used to identity the risk factors for fatal outcome. The predictive value of RDW for fatal outcome was evaluated by the receiver operating characteristic (ROC) analysis and Kaplan-Meier methods. Results: Of 292 patients, the median age was 61.5 years. Non-survivors showed higher RDW value than survivors (13.6% vs.13.0%, P < 0.001). The mortality rate was 44.8% in patients with elevated RDW compared to 18.4% of patients with normal RDW, with a relative risk (RR) of 2.439. Elevated RDW was an independent risk factor of mortality (hazards ratio: 1.167, P = 0.019). Patients with elevated RDW had a higher cumulative mortality than patients with normal RDW. The area under the ROC curve (AUC) of RDW for the prediction of mortality was 0.690 (P < 0.001). Conclusion: Elevated RDW was associated with higher mortality risk for patients hospitalized for SFTS. RDW may be helpful for risk stratification in SFTS patients.

2.
Cureus ; 16(6): e63203, 2024 Jun.
Article in English | MEDLINE | ID: mdl-39070366

ABSTRACT

Idiopathic thrombocytopenic purpura (ITP) presents challenges in management, particularly in cases refractory to corticosteroids. Thrombopoietin receptor (TPO-R) agonists, such as eltrombopag, offer alternative therapeutic options. We report a case of a 72-year-old South Asian male with steroid-refractory chronic ITP who achieved a favorable response with biweekly eltrombopag dosing. Platelet response was comparable to daily dosing, suggesting the efficacy of less frequent administration schedules. This approach may enhance patient adherence and reduce treatment-related financial burdens. Biweekly eltrombopag dosing presents a promising alternative for chronic ITP management, warranting further investigation.

3.
Cureus ; 16(6): e63255, 2024 Jun.
Article in English | MEDLINE | ID: mdl-39070501

ABSTRACT

Background The most prevalent arbovirus infection in the world, dengue, has become a serious public health issue. This study aims to examine the clinical characteristics of individuals who present with dengue fever and use platelet count prediction to estimate the severity of dengue. Materials and methods This observational, cross-sectional study was conducted at Dr. D. Y. Patil Medical Hospital, Pune, Maharashtra, India, from February 2022 to May 2024. A total of 100 patients older than 12 years old who had dengue fever (presenting within three days of the first symptom) and were dengue NS1 antigen-positive in the laboratory were included. Patients under 12 years of age and pregnant women were excluded. Also excluded were patients with a history of prior dengue infection and patients on medications causing thrombocytopenia, such as antiplatelets (aspirin). Written informed consent was obtained from each patient. For adolescent boys and girls aged 13-18, consent was obtained from a parent or legal guardian along with the adolescent's assent. Data were collected through physical examinations and laboratory investigations. Statistical analysis was performed using IBM SPSS Statistics for Windows, Version 20 (Released 2011; IBM Corp., Armonk, New York), with descriptive statistics and tests for nonparametric data, setting the significance at p<0.05. Results The average age of the study participants was 29.48 ± 10.62 years, with 24% in the 0-20 year age group, 36% in the 21-30 age group, 24% in the 31-40 age group, 12% in the 41-50 age group, 3% in the 51-60 age group, and 1% in the 61-70 age group. Men comprised 65% of the population, with 35% being women. Weakness was the most prevalent symptom, followed by nausea and fever with chills. Patients with dengue fever without warning indications had a median platelet count of 114,000/µL upon admission; those with dengue fever with warning signs had a median count of 35,500/µL; and those with severe dengue had a median count of 25,000/µL. These distinctions attained statistical significance, underscored by p-values of <0.001. The predictive model for severe dengue using platelet count on presentation demonstrated a robust capacity to anticipate severe dengue with a noteworthy association (p<0.04), indicating an increased risk of severe dengue with a lower platelet count (<25,000/µL, odds ratio (OR) 7.5). Conclusion Dengue was more common in the young population, with a predominance of male patients. Weakness was the most common symptom. Patients with a platelet count less than 25,000/µL had 7.5 times more odds of developing severe dengue.

4.
Cureus ; 16(7): e65488, 2024 Jul.
Article in English | MEDLINE | ID: mdl-39071072

ABSTRACT

Drug-induced thrombocytopenia is a rare but significant adverse effect of certain medications, with the potential for severe bleeding, thrombosis, and death. This report discusses a rare case of severe thrombocytopenia induced by ceftaroline in a 69-year-old male with a history of atrial fibrillation on rivaroxaban and allergies to amoxicillin and sulfa drugs. Following the initiation of ceftaroline for left lower extremity purulent cellulitis, his platelet count dropped from 204,000 to 4,000 x 10³/µL within a day. Given the low platelet levels, anticoagulation therapy, and bleeding risk, immediate interventions and prompt recognition prevented major complications, highlighting the importance of recognizing drug-induced thrombocytopenia in clinical practice.

5.
SAGE Open Med Case Rep ; 12: 2050313X241266766, 2024.
Article in English | MEDLINE | ID: mdl-39071190

ABSTRACT

A 53-year-old otherwise healthy man was referred to our hospital with a fever of unknown origin, headache, and arthralgia. Four days earlier, he had a fever with chills. Treatment with antibiotics and acetaminophen proved ineffective, with the patient subsequently developing headache and joint pain. Blood analysis revealed elevated inflammatory markers, liver impairment, and severe thrombocytopenia (platelet count, 19,000/µL). Subsequent tests revealed elevated levels of anti-cytomegalovirus IgM and IgG. Based on these findings, the patient was diagnosed with severe thrombocytopenia associated with cytomegalovirus infection. Platelet counts increased spontaneously without antiviral therapy. Forty-five days after the initial visit, the symptoms improved, and blood tests revealed resolution of the inflammatory findings, with the platelet count recovering to 155,000/µL. Although the disease may resolve spontaneously, cytomegalovirus infection should be considered as a differential diagnosis in case of severe thrombocytopenia in immunocompetent adults.

6.
Front Vet Sci ; 11: 1404337, 2024.
Article in English | MEDLINE | ID: mdl-39071788

ABSTRACT

Introduction: The objective of this case report is to describe diagnosis and management of life-threatening immune thrombocytopenia (ITP) secondary to pregnancy in a dog with concurrent dystocia. Case summary: A 1-year 11-month old female intact French bulldog was referred for management of severe thrombocytopenia and spontaneous hemorrhage during whelping. The thrombocytopenia was progressive from approximately 32 days of gestation. In the absence of an identifiable cause for the thrombocytopenia, the patient was treated for ITP with immunosuppressive therapies and blood and plasma transfusions. The patient was also supported through dystocia until the platelet count normalized so a Caesarean section and ovariohysterectomy (OVH) could be performed. Discussion: This is the first report documenting ITP in a whelping canine. Pregnancy is a known trigger and can affect the clinical course of autoimmune diseases in women, including ITP. It is suspected that this patient's pregnancy triggered ITP, paralleling what occurs in women.

7.
Expert Rev Hematol ; 2024 Jul 29.
Article in English | MEDLINE | ID: mdl-39072415

ABSTRACT

INTRODUCTION: Immune thrombocytopenia (ITP) is an acquired autoimmune disorder characterized by low platelets and an increased risk of bleeding. Platelet autoantibodies target major platelet glycoproteins and cause Fc-mediated platelet destruction in the spleen and reticuloendothelial systems. As markers of disease, platelet autoantibodies are important therapeutic targets. Neonatal Fc receptor (FcRn) antagonists are a new class of therapeutics that reduce the half-life of immunoglobulin G including platelet autoantibodies. Spleen tyrosine kinase (Syk) inhibitors interfere with Fc-mediated platelet clearance. Bruton's tyrosine kinase (BTK) inhibitors, and inhibitors of B-cell activating factor (BAFF) reduce antibody production. The efficacy of these targeted therapies emphasize the role of platelet autoantibodies in ITP even though they can be difficult to detect. AREAS COVERED: This review includes an in-depth exploration of the pathophysiologic mechanisms of ITP, focusing on autoantibodies. Treatments outlined in this review include a) FcRn antagonists (efgartigimod), b) complement inhibitors (sutimlimab), c) B-cell directed therapies such as BTK inhibitors (rilzabrutinib), anti-BAFF agents (belimumab, ianalumab), and Syk inhibitors (fostamatinib, sovleplenib), d) plasma-cell directed therapies (daratumumab, bortezomib), and e) cellular therapeutic products. EXPERT OPINION: Platelet antibodies are often elusive in ITP; yet novel treatments targeting this pathway reinforce their role in the pathogenesis of this autoimmune platelet disorder.

8.
Br J Haematol ; 2024 Jul 27.
Article in English | MEDLINE | ID: mdl-39072718

ABSTRACT

The Kids ITP Tools (KIT) is a health-related quality of life (HRQoL) questionnaire that evaluates quality of life in children with immune thrombocytopenia (ITP). There are three formats: Child Self-Report, Parent Proxy-Report and Parent Impact-Report. This study aimed to develop a domain structure by grouping-related questions from the questionnaire into domains that independently reflect various aspects of HRQoL. The study was conducted in two phases. Phase 1 involved an online survey distributed to experts to identify conceptual domains for the KIT. Phase 2 utilized a statistical approach to analyse responses from patients with ITP and their families. A revised KIT 2.0 was ultimately developed to aid in treatment decision-making and monitoring of ITP.

9.
Br J Haematol ; 2024 Jul 27.
Article in English | MEDLINE | ID: mdl-39072725

ABSTRACT

International societies have conflicting recommendations on whether bone marrow aspirate/biopsy (BMB) is needed during workup for isolated thrombocytopenia. Our objective was to determine if thrombocytopenia in patients aged ≥60 years is associated with an increased incidence of haematological malignancy. We performed a retrospective population-based cohort study in patients aged ≥60 years between January 1, 2009 to December 31, 2019. Exposed patients had specialist consultation for thrombocytopenia, with platelet count <100 × 109/L, but normal haemoglobin and white blood cell count. Unexposed patients were those who never had specialist consultation for thrombocytopenia and whose platelets were ≥100 × 109/L. The primary outcome was the diagnosis of haematological malignancy using a competing risk of death model. During 4.0 years (IQR 2.2-6.7) of follow-up, 378/4930 exposed (19.1/1000PY, 95% CI 17.1-21.0), and 204/17556 unexposed patients (2.5/1000PY, 95% CI 2.2-2.8) were diagnosed with haematological malignancy (HR 15.5 (95% CI 11.3-21.4, p < 0.0001) in year 1, and 5.3 (95% CI 4.4-6.6, p < 0.0001) in years 2+). This finding persisted in analyses stratified by sex, age, severity, or duration of thrombocytopenia, and treatment with corticosteroids within 2 weeks of consultation. This study found a strong association between isolated thrombocytopenia and haematological malignancy in patients ≥60 years, supporting consideration of diagnostic testing including BMB during outpatient specialist consultation.

10.
Clin Rheumatol ; 2024 Jul 29.
Article in English | MEDLINE | ID: mdl-39073513

ABSTRACT

OBJECTIVES: Severe thrombotic antiphospholipid syndrome (APS) frequently affects the kidney, heart, and central nervous system. The precise frequency, clinical picture, differential diagnoses, and outcome of APS-related hematological involvement are lacking, especially in patients requiring ICU admission. This study aimed to describe the hematological manifestations associated with critically ill thrombotic APS patients and catastrophic antiphospholipid syndrome. METHODS: This French, national, multicenter, retrospective study, conducted, from January 2000 to September 2018, included all APS patients admitted to 24 participating centers' ICUs with any new thrombotic manifestation. The prevalence of hematological manifestations and their associated outcomes were studied. RESULTS: One hundred and thirty-four patients, female 72%, median [IQR] age 45 [34-56] years, with 152 episodes were included. Anemia was present in 95% of episodes and thrombocytopenia in 93%. The lowest values for hemoglobin and platelets were 7.1 [6.3-8.8] g/dL and 38 [21-60] g/L, respectively. The lowest platelet count below 20 g/L was significantly associated with a higher in-ICU mortality rate (50%, p < 0.0001). A thrombotic microangiopathy syndrome (TMA) syndrome was seen in 16 patients (12%) and was associated with higher in-hospital mortality (p = 0.05). Median ADAMTS-13 levels were 44% [27-74]. Anti-ADAMTS13 antibodies were tested in 11 patients and found negative in all. A suspicion of heparin-induced thrombocytopenia (HIT) was raised in 66 patients but only four patients were classified as definite HIT. Disseminated intravascular coagulation (DIC) was seen in 51% of patients. CONCLUSION: Thrombocytopenia is very frequent in severe APS patients and may be related to TMA, HIT, or DIC. Deciphering the mechanisms of thrombocytopenia is decisive in CAPS patients. Key Points • Thrombocytopenia is the hallmark laboratory finding in CAPS. • A complete thrombotic microangiopathy pattern is infrequent in CAPS patients. • Alternate diagnoses of CAPS, especially heparin-induced thrombocytopenia, need to be adequately investigated.

11.
Ann Hematol ; 2024 Jul 29.
Article in English | MEDLINE | ID: mdl-39073589

ABSTRACT

Recurrent problems of patients with myelofibrosis (MF) are cytopenias, debiliating disease-related symptoms and splenomegaly. Whereas the latter are usually addressed by the JAK1/2 inhibitors ruxolitinib and fedratinib, cytopenias often remain critical. Momelotinib, a JAK1/2 inhibitor recently approved for the treatment of anemic MF patients, was shown to improve anemia via a direct inhibition of activin A receptor type I. In this German-wide, multicenter, retrospective analysis the safety and efficacy profile of momelotinib was evaluated in a real world setting within a cohort of 60 MF patients independent of pre-treatment. The median duration of treatment was 12 weeks. As a new, but manageable safety finding, creatinine increase (CTC°1-2) was detected in 10/60 patients (17%). Interestingly, not only hemoglobin levels increased in 84% of patients, but also platelet values (67%). In the cohort of transfusion-dependent individuals (n = 38), transfusion requirement improved in 15 patients (39%) with 8 reaching transfusion independency (21%). Transfusion independency was achieved within a median of 4 weeks (range 2-12). Spleen size decreased in 13/53 individuals (25%) with a median response time of 6 weeks. Thereof, 11 patients had been pre-treated with JAK inhibitor(s) (85%). Clinical improvement was detected in 24/51 symptomatic individuals (47%) with a median response time of 4 weeks. 5 patients stopped treatment due to side effects (8%), 6 patients due to a worsening of clinical symptoms (10%). Taken together, the MoReLife analysis identifies momelotinib as potent and safe therapeutic option also for heavily pre-treated cytopenic MF patients under real world conditions.

12.
Medicina (Kaunas) ; 60(7)2024 Jul 17.
Article in English | MEDLINE | ID: mdl-39064581

ABSTRACT

Background and Objectives: Immune thrombocytopenia (ITP) is an autoimmune disease characterized by the autoantibody-mediated destruction of platelets. The treatment of ITP aims to maintain a sufficient platelet count to prevent bleeding. First-line treatment options include corticosteroids and intravenous immunoglobulin (IVIg), while second-line treatments include splenectomy, rituximab and other immunosuppressive agents, and thrombopoietin (TPO) receptor agonists. This study aims to discuss the treatment methods and results from 100 patients with ITP at the Mugla Training and Research Hospital through a pharmacological approach. Materials and Methods: Demographic characteristics, clinical findings, bone marrow aspiration and biopsy results, and treatments and treatment responses at the time of diagnosis of the 100 patients with ITP who were treated and followed up in the period 2015-2023 were evaluated retrospectively. Results: In the third month after treatment, the overall response percentage was 100% in patients who received steroids only and 88% in patients who received IVIg treatment alone or in combination with steroids (p > 0.05). The most preferred second-line treatments were splenectomy (41%), eltrombopag (26%), and rituximab (10%). Bone marrow biopsy was performed in 54% of patients, where 35.1% showed increased megakaryocytes, 44.4% adequate megakaryocytes, and 14.8% decreased megakaryocytes. It is noted that eltrombopag and rituximab, in particular, yield higher complete remission rates than immunosuppressive drugs. Conclusions: Considering the side effects of immunosuppressive medications, IVIg, splenectomy, and steroid therapy, the use of new agents such as eltrombopag, which are easily tolerated and have a lower risk of side effects, is expected to increase.


Subject(s)
Benzoates , Hydrazines , Immunoglobulins, Intravenous , Purpura, Thrombocytopenic, Idiopathic , Rituximab , Splenectomy , Humans , Female , Male , Retrospective Studies , Middle Aged , Purpura, Thrombocytopenic, Idiopathic/drug therapy , Purpura, Thrombocytopenic, Idiopathic/mortality , Adult , Cross-Sectional Studies , Rituximab/therapeutic use , Benzoates/therapeutic use , Hydrazines/therapeutic use , Immunoglobulins, Intravenous/therapeutic use , Splenectomy/statistics & numerical data , Aged , Prognosis , Pyrazoles/therapeutic use , Adolescent , Immunosuppressive Agents/therapeutic use , Adrenal Cortex Hormones/therapeutic use , Survival Analysis
13.
Front Pharmacol ; 15: 1407894, 2024.
Article in English | MEDLINE | ID: mdl-38953101

ABSTRACT

Introduction: An increasing number of immune-related adverse events (irAEs) induced by immune checkpoint inhibitors (ICIs) have been reported during clinical treatment. We aimed to explore the clinical characteristics of patients with ICIs-induced ITP under different therapeutic strategies based on the FAERS database and explore the potential biological mechanisms in combination with TCGA pan-cancer data. Methods: Data from FAERS were collected for ICIs adverse reactions between January 2012 and December 2022. Disproportionality analysis identified ICIs-induced ITP in the FAERS database using the reporting odds ratio (ROR), proportional reporting ratio (PRP), Bayesian confidence propagation neural network (BCPNN), and multi-item gamma Poisson shrinker algorithms (MGPS). The potential biological mechanisms underlying ITP induced by ICIs were examined using TCGA transcriptome data on cancers. Results: In the FAERS, 345 ICIs-induced ITP reports were retrieved, wherein 290 (84.06%) and 55 (15.94%) were reported as monotherapy and combination therapy, respectively. The median age of the reported patients with ICIs-induced ITP was 69 years (IQR 60-76), of which 62 (18%) died and 47 (13.6%) had a life-threatening outcome. The majority of reported indications were lung, skin, and bladder cancers, and the median time to ITP after dosing was 42 days (IQR 17-135), with 64 patients (43.5%) experiencing ITP within 30 days of dosing and 88 patients experiencing ITP in less than 2 months (59.9%). The occurrence of ICIs-induced ITP may be associated with ICIs-induced dysregulation of the mTORC1 signaling pathway and megakaryocyte dysfunction. Conclusion: There were significant reporting signals for ITP with nivolumab, pembrolizumab, cemiplimab, atezolizumab, avelumab, durvalumab, ipilimumab, nivolumab/ipilimumab, and pembrolizumab/ipilimumab. Patients treated with anti-PD-1 in combination with anti-CTLA-4 are more likely to have an increased risk of ICIs-induced ITP. Patients with melanoma are at a higher risk of developing ITP when treated with ICI and should be closely monitored for this risk within 60 days of treatment. The potential biological mechanism of ICIs-induced ITP may be related to the dysfunction of megakaryocyte autophagy through the overactivation of the mTOR-related signaling pathway. This study provides a comprehensive understanding of ICIs-induced ITP. Clinicians should pay attention to this potentially fatal adverse reaction.

14.
Front Med ; 2024 Jul 03.
Article in English | MEDLINE | ID: mdl-38958922

ABSTRACT

Corona virus disease 2019 (COVID-19) due to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection has affected the whole world. Acquired thrombotic thrombocytopenic purpura (TTP) has been reported after administration of mRNA- or adenoviral vector-based COVID-19 vaccines, including Ad26.COV2-S, BNT162b2, mRNA-1273, and ChAdOx1 nCov-19. However, whether inactivated vaccines, such as CoronaVac, could cause TTP and whether the symptoms in TTPs caused by inactivated vaccines are different from previously reported cases are unknown. In this study, two cases were reported. Both cases developed TTP after the second CoronaVac vaccination shot, but not the first. They demonstrated symptoms of fever, neurological abnormalities, renal dysfunction, thrombocytopenia, and hemolysis. Both patients achieved complete remission through several sessions of plasma exchanges and immune suppression. The incidence of TTP in Nanjing area was analyzed. The number of patients with TTP was 12 in 2019, 6 in 2020, 16 in 2021, and 19 in 2022. To the authors' knowledge, this report is the first report of TTP associated with inactivated COVID-19 vaccine (CoronaVac). The rarity and delayed onset may be due to the relatively milder immune response caused by the inactivated vaccines than mRNA-based ones. Timely plasma exchange is a vital treatment for CoronaVac-related TTP, similar to activated vaccine-related TTP.

15.
Avicenna J Phytomed ; 14(1): 112-125, 2024.
Article in English | MEDLINE | ID: mdl-38948177

ABSTRACT

Objective: Medicago sativa (M. sativa) has been traditionally used for treating anemia; therefore, M. sativa hydro-ethanolic extract therapeutic effects against cyclophosphamide (CP) -induced hematologic and liver toxicity were examined. Materials and Methods: Thirty male Wistar rats were randomly divided to control (saline); CP (100 mg/kg, day 1-3, subcutaneously); CP+ M. sativa 200 mg/kg (MS 200); CP+ M. sativa 400 mg/kg (MS 400); CP+ dexamethasone (0.1 mg/kg), (all groups n=6). Treated animals received M. sativa or dexamethasone by gavage from days 7-14. On days 0, 7, and 14, hematologic parameters, and on the 14th day, serum and liver tissue oxidative stress markers including nitric oxide, malondialdehyde (MDA) and total thiol levels, superoxide dismutase (SOD) and catalase (CAT) activities, serum lipids, and liver enzymes were measured. Results: Animal weight, platelet, white blood cells, and red blood cells counts, hemoglobin and hematocrit as well as thiol, SOD, and CAT activities in serum and liver tissue were significantly reduced, but serum nitric oxide, MDA, total cholesterol, triglycerides, low-density lipoproteins levels, and liver enzymes were increased in the CP group compared to the control group (p<0.05 to p<0.001). Administering M. sativa extract (400 mg/kg) significantly enhanced platelet count, and SOD and CAT activities and inhibited all of the CP toxic effects, while dexamethasone improved platelet count and oxidative stress markers compared to the CP group (p<0.05 to p<0.001). Conclusion: The extract of M. sativa (400 mg/kg) showed therapeutic effects against the CP-induced myelosuppression and thrombocytopenia and improved oxidative stress markers which were comparable to the effect of dexamethasone.

16.
J Family Med Prim Care ; 13(5): 1787-1792, 2024 May.
Article in English | MEDLINE | ID: mdl-38948621

ABSTRACT

Introduction: Systemic lupus erythematosus (SLE), the commonest type of lupus, is an autoimmune multisystemic disorder that can affect any organ system of the body, especially blood vessels and connective tissues, causing widespread inflammation. Pediatric onset of SLE is a rare condition with more hematological involvement. Aim: This study was undertaken to observe various hematological abnormalities and their association with various autoantibodies present in pediatric SLE in Eastern India. Methodology: It was a single-centered, cross-sectional, observational, hospital-based study conducted in the Department of Pediatric Medicine in collaboration with the Department of Rheumatology in IPGME and R and SSKM Hospital, Kolkata. The duration of the study was 1.5 years, and a total of 30 children up to 12 years of age of either gender were enrolled. Study participants were evaluated for various parameters like demographic, hematological (anemia, neutropenia, leucopenia, lymphopenia, and thrombocytopenia), biochemical (CRP, Lactate dehydrogenase (LDH), and bilirubin), autoantibodies (anti-dsDNA, anti-Ro 52, and anti-Ribonucleoprotein [RNP]), and SLE related pathologies (Cutaneous, nephritis, serositis). Results: In the present study, most of the participants had arthritis, muscle pain (86.66%), and hematological involvement (80%). Among cytopenias, anemia was the commonest. dsDNA autoantibody was positive in most of the patients (83%), and about one-third suffered from autoimmune hemolytic anemia (AIHA). No association was observed between autoantibodies and various hematological manifestations. Conclusion: It can be concluded from the present study that anemia is the most common cytopenia in pediatric SLE, but there is no association between autoantibodies and these cytopenias. However, study on larger population may give better results.

17.
Pediatr Cardiol ; 2024 Jul 01.
Article in English | MEDLINE | ID: mdl-38951145

ABSTRACT

After the Fontan procedure, patients require lifelong follow-up due to significant late morbidity and mortality. Thrombocytopenia is seen frequently post-Fontan, likely due to secondary hypersplenism from elevated Fontan pressure. We investigated platelet counts in patients with a Fontan circulation and assessed associations with catheterization data and clinical outcomes. This retrospective study included 92 patients (33% female) post-Fontan who had a complete blood count performed between January 2011 and July 2023. The age at evaluation was 24.0 ± 8.9 years. Outcomes measured included elevated Fontan pressure (≥ 15 mmHg), Fontan-associated liver disease (FALD), unscheduled admissions, transplant, and death. Participants with thrombocytopenia (≤ 150,000/µL) had significantly higher rates of elevated Fontan pressure (OR 8.1, 95% CI 1.3-52.7, p = 0.03), FALD (OR 4.1, 95% CI 1.6-10.6, p = 0.004), and unscheduled admissions (362 ± 577 versus 115 ± 185 admissions per 1000 patient-years, p = 0.02). Thrombocytopenia post-Fontan is associated with elevated Fontan pressure, FALD, and increased morbidity. Platelet count could serve as a non-invasive factor in identifying patients at risk of decompensation.

18.
Rev Med Interne ; 2024 Jul 02.
Article in English | MEDLINE | ID: mdl-38960844

ABSTRACT

The CARMEN-France registry is a prospective, multicenter registry in France including adult patients with a new diagnosis of immune thrombocytopenia or of autoimmune immune hemolytic anemia (2402 patients included in December 31, 2023). The recording of clinical, biological and treatment data allows detailed epidemiological and pharmacoepidemiological real-world studies. This review summarizes the CARMEN-France registry protocol, gives examples of studies conducted in the registry, and indicates future directions such as inclusion of patient reported outcomes, linkage with the French national health insurance database and linkage with other registries in Europe.

19.
Zhonghua Gan Zang Bing Za Zhi ; 32(6): 489-492, 2024 Jun 20.
Article in Chinese | MEDLINE | ID: mdl-38964889

ABSTRACT

Thrombocytopenia is one of the common complications of cirrhotic patients, which can induce an increasing bleeding risk and closely correlate with bleeding following invasive procedures. Consequently, how to respond to thrombocytopenia is crucial for improving the prognosis of patients with cirrhosis. This article reviews the main mechanisms of cirrhosis concurrent with thrombocytopenia, as well as the corresponding clinical management strategies.


Subject(s)
Liver Cirrhosis , Thrombocytopenia , Humans , Thrombocytopenia/therapy , Thrombocytopenia/etiology , Liver Cirrhosis/complications , Liver Cirrhosis/therapy
20.
Zhonghua Gan Zang Bing Za Zhi ; 32(6): 481-483, 2024 Jun 20.
Article in Chinese | MEDLINE | ID: mdl-38964887

ABSTRACT

Managing cirrhosis complications is an important measure for improving patients' clinical outcomes. Therefore, in order to provide a complete disease assessment and comprehensive treatment, improve quality of life, and improve the prognosis for patients with cirrhosis, it is necessary to pay attention to complications such as thrombocytopenia and portal vein thrombosis in addition to common or severe complications such as ascites, esophagogastric variceal bleeding, hepatic encephalopathy, and hepatorenal syndrome. The relevant concept that an effective albumin concentration is more helpful in predicting the cirrhosis outcome is gradually being accepted; however, the detection method still needs further standardization and commercialization.


Subject(s)
Hepatic Encephalopathy , Liver Cirrhosis , Humans , Liver Cirrhosis/complications , Liver Cirrhosis/diagnosis , Hepatic Encephalopathy/etiology , Hepatic Encephalopathy/diagnosis , Hepatic Encephalopathy/therapy , Hepatorenal Syndrome/etiology , Hepatorenal Syndrome/diagnosis , Hepatorenal Syndrome/therapy , Ascites/etiology , Ascites/therapy , Ascites/diagnosis , Thrombocytopenia/etiology , Thrombocytopenia/diagnosis , Thrombocytopenia/therapy , Esophageal and Gastric Varices/diagnosis , Esophageal and Gastric Varices/etiology , Esophageal and Gastric Varices/therapy , Gastrointestinal Hemorrhage/etiology , Gastrointestinal Hemorrhage/diagnosis , Gastrointestinal Hemorrhage/therapy
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