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HIV Medicine Nov 2022Thrombotic thrombocytopenic purpura (TTP), a serious thrombotic microangiopathy (TMA), is prevalent in the South African HIV-infected population. The exact pathogenesis... (Review)
Review
BACKGROUND
Thrombotic thrombocytopenic purpura (TTP), a serious thrombotic microangiopathy (TMA), is prevalent in the South African HIV-infected population. The exact pathogenesis of HIV-associated TTP (HIV-TTP) is however still unclear with diagnostic and therapeutic inconsistancies.
METHODS
A systematic review of the published literature regarding HIV-TTP was performed.
RESULTS
HIV-TTP is still associated with significant morbidity and mortality in Africa despite the availability of anti-retroviral therpy (ART). Diagnosis of HIV-TTP requires the presence of a micro-angiopathic haemolytic anaemia with significant red blood cell schistocytes and thrombocytopenia in the absence of another TMA but background activation of the coagulation system and inflammation in HIV infected people can result in diagnostic anbiguity. Plasma therapy in the form of infusion or exchange is successful but expensive, associated with side-effects and not widely available. Adjuvant immunosuppression therapy may of benefit in patients with HIV-TTP and ART must always be optimised. Endothelial dysfunction caused by chronic inflammation and complement activation most likely contributes to the development of HIV-TTP.
CONCLUSION
The role of adjuvant immunomodulating therpy, the therapeutic targets and pathogenic contribution from endothelial dysfunction in HIV-TTP requires further investigation.
Topics: HIV Infections; Humans; Inflammation; Plasma; Purpura, Thrombotic Thrombocytopenic
PubMed: 35373442
DOI: 10.1111/hiv.13305 -
Frontiers in Pharmacology 2024Treatment of glomerulonephritis presents several challenges, including limited therapeutic options, high costs, and potential adverse reactions. As a recognized Chinese... (Review)
Review
Treatment of glomerulonephritis presents several challenges, including limited therapeutic options, high costs, and potential adverse reactions. As a recognized Chinese patent medicine, poly-glycosides (TWP) have shown promising benefits in managing autoimmune diseases. To evaluate clinical effectiveness and safety of TWP in treating glomerulonephritis, we systematically searched PubMed, Cochrane Library, Web of Science, and Embase databases for controlled studies published up to 12 July 2023. We employed weighted mean difference and relative risk to analyze continuous and dichotomous outcomes. This meta-analysis included 16 studies that included primary membranous nephropathy (PMN), type 2 diabetic kidney disease (DKD), and Henoch-Schönlein purpura nephritis (HSPN). Analysis revealed that additional TWP administration improved patients' outcomes and total remission rates, reduced 24-h urine protein (24hUP) and decreased relapse events. The pooled results demonstrated the non-inferiority of TWP to glucocorticoids in achieving total remission, reducing 24hUP, and converting the phospholipase A2 receptor (PLA2R) status to negative. For DKD patients, TWP effectively reduced 24hUP levels, although it did not significantly improve the estimated glomerular filtration rate (eGFR). Compared to valsartan, TWP showed comparable improvements in 24hUP and eGFR levels. In severe cases of HSPN in children, significant clinical remission and a reduction in 24hUP levels were observed with the addition of TWP treatment. TWP did not significantly increase the incidence of adverse reactions. Therefore, TWP could offer therapeutic benefits to patients with PMN, DKD, and severe HSPN, with a minimal increase in the risk of side effects.
PubMed: 38841368
DOI: 10.3389/fphar.2024.1339153 -
Blood Transfusion = Trasfusione Del... Jul 2016Only a few studies have compared solvent/detergent plasma (SD-plasma) to standard fresh-frozen plasma (FFP) in terms of efficacy and safety. (Review)
Review
BACKGROUND
Only a few studies have compared solvent/detergent plasma (SD-plasma) to standard fresh-frozen plasma (FFP) in terms of efficacy and safety.
MATERIALS AND METHODS
A systematic review was performed in order to develop a consensus document on the use of SD-plasma. Moreover, a pharmacoeconomic study was performed in order to assess whether the use of SD-plasma can be cost-effective with respect to the use of FFP. A multidisciplinary panel used the systematic review and the GRADE methodology to develop evidence-based recommendations on this topic.
RESULTS
Based on moderate to very low quality evidence, the panel developed the following consensus statements: (i) the panel suggested that SD-plasma is safer than FFP; (ii) the panel could not express for or against a greater efficacy of SD-plasma as compared to FFP; (iii) the panel suggested that in patients undergoing liver transplantation SD-plasma can be preferred over FFP; (iv) the panel suggested that SD-plasma can be preferred over FFP in patients with thrombotic thrombocytopenic purpura undergoing plasma-exchange procedures; (v) the panel could not recommend for or against preferring SD-plasma over FFP in critical care patients; and (vi) the panel suggested that the use of SD-plasma can be cost-effective with respect to the use of FFP.
DISCUSSION
Data from additional randomised studies are needed to establish more definitive guidelines on the use of SD-plasma.
PubMed: 27136429
DOI: 10.2450/2016.0168-15 -
PeerJ 2024Immune disorders and autoantibodies has been noted in both primary immune thrombocytopenia (ITP) and systemic lupus erythematosus (SLE). Whether the two disorders are... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Immune disorders and autoantibodies has been noted in both primary immune thrombocytopenia (ITP) and systemic lupus erythematosus (SLE). Whether the two disorders are correlated is unclear. The lack of evidence on the incidence of and risk factors for SLE in primary ITP patients poses a challenge for prediction in clinical practice. Therefore, we conducted this study.
METHODS
The protocol was registered with PROSPERO (CRD42023403665). Web of Science, Cochrane, PubMed, and EMBASE were searched for articles published from inception to 30 September 2023 on patients who were first diagnosed with primary ITP and subsequently developed into SLE. Furthermore, the risk factors were analyzed. Study quality was estimated using the Newcastle-Ottawa Scale. The statistical process was implemented using the R language.
RESULTS
This systematic review included eight articles. The incidence of SLE during the follow-up after ITP diagnosis was 2.7% (95% CI [1.3-4.4%]), with an incidence of 4.6% (95% CI [1.6-8.6%]) in females and 0 (95% CI [0.00-0.4%]) in males. Older age (OR = 6.31; 95% CI [1.11-34.91]), positive antinuclear antibody (ANA) (OR = 6.64; 95% CI [1.40-31.50]), hypocomplementemia (OR = 8.33; 95% CI [1.62-42.91]), chronic ITP (OR = 24.67; 95% CI [3.14-100.00]), organ bleeding (OR = 13.67; 95% CI [2.44-76.69]), and female (OR = 20.50; 95% CI [4.94-84.90]) were risk factors for subsequent SLE in ITP patients.
CONCLUSION
Patients with primary ITP are at higher risk of SLE. Specific follow-up and prevention strategies should be tailored especially for older females with positive ANA, hypocomplementemia, or chronic ITP. In subsequent studies, we need to further investigate the risk factors and try to construct corresponding risk prediction models to develop specific prediction strategies for SLE.
Topics: Humans; Lupus Erythematosus, Systemic; Incidence; Risk Factors; Purpura, Thrombocytopenic, Idiopathic; Female; Male
PubMed: 38666084
DOI: 10.7717/peerj.17152 -
Thrombosis Research Jun 2022With the advent of COVID-19 vaccines, hospitalization rates and progression to severe COVID-19 disease have reduced drastically. Most of the adverse events reported by... (Review)
Review
INTRODUCTION
With the advent of COVID-19 vaccines, hospitalization rates and progression to severe COVID-19 disease have reduced drastically. Most of the adverse events reported by the vaccine recipients were minor. However, autoimmune hematological complications such as vaccine-induced immune thrombotic thrombocytopenia (VITT), immune thrombocytopenic purpura (ITP) and TTP have also been reported post-COVID-19 vaccination. Given this, we sought to reflect on the existing cases of TTP, whether de novo or relapsing, reported after COVID-19 vaccination to further gain insight into any association, if present, and outcomes.
METHODS
We searched PubMed, Embase, and Ebsco databases for published individual case reports on the occurrence or relapse of TTP after receiving any COVID-19 vaccine. A total of 23 articles (27 patients) were included in this qualitative analysis.
RESULTS
The mean age for the patients who developed de novo TTP post-COVID-19 vaccination was 51.3 years. TTP episodes were seen mostly after BNT162b2 vaccine, followed by mRNA-1273 vaccine. All patients with immune TTP except one received plasma exchange (PLEX) and steroids. One patient passed away after two days of hospitalization, likely due to a sudden cardiovascular event.
CONCLUSION
Our review underscores the importance of in-depth anamnesis before vaccination and outlines characteristics of predisposed individuals. Evaluation of post-vaccine thrombocytopenia must include the possibility of TTP given the associated fatality with this condition.
Topics: 2019-nCoV Vaccine mRNA-1273; BNT162 Vaccine; COVID-19; COVID-19 Vaccines; Humans; Middle Aged; Purpura, Thrombocytopenic, Idiopathic; Purpura, Thrombotic Thrombocytopenic; SARS-CoV-2; Thrombosis; Vaccination
PubMed: 35533526
DOI: 10.1016/j.thromres.2022.04.020 -
International Journal of Environmental... Jul 2021Vulvar lichen sclerosus (VLS) is a chronic inflammatory condition affecting the anogenital region, which may present in a prepubertal or adolescent patient. The most...
Vulvar lichen sclerosus (VLS) is a chronic inflammatory condition affecting the anogenital region, which may present in a prepubertal or adolescent patient. The most popular theories are its autoimmune and genetic conditioning, although theories concerning hormonal and infectious etiology have also been raised. The most common presenting symptoms of VLS is vulva pruritus, discomfort, dysuria and constipation. In physical examination, a classic "Figure 8" pattern is described, involving the labia minora, clitoral hood, and perianal region. The lesions initially are white, flat-topped papules, thin plaques, or commonly atrophic patches. Purpura is a hallmark feature of VLS. The treatment includes topical anti-inflammatory agents and long-term follow-up, as there is a high risk of recurrence and an increased risk of vulvar cancer in adult women with a history of lichen sclerosus. This article reviews vulvar lichen sclerosus in children and provides evidence-based medicine principles for treatment in the pediatric population. A systematic search of the literature shows recurrence of VLS in children. Maintenance regimens deserve further consideration.
Topics: Adolescent; Adult; Anti-Inflammatory Agents; Child; Female; Humans; Lichen Sclerosus et Atrophicus; Vulvar Diseases; Vulvar Lichen Sclerosus
PubMed: 34281089
DOI: 10.3390/ijerph18137153 -
F1000Research 2018The Zika virus (ZIKV) outbreak in the Americas has caused international concern due to neurological sequelae linked to the infection, such as microcephaly and...
The Zika virus (ZIKV) outbreak in the Americas has caused international concern due to neurological sequelae linked to the infection, such as microcephaly and Guillain-Barré syndrome (GBS). The World Health Organization stated that there is "sufficient evidence to conclude that Zika virus is a cause of congenital abnormalities and is a trigger of GBS". This conclusion was based on a systematic review of the evidence published until 30.05.2016. Since then, the body of evidence has grown substantially, leading to this update of that systematic review with new evidence published from 30.05.2016 - 18.01.2017, update 1. We review evidence on the causal link between ZIKV infection and adverse congenital outcomes and the causal link between ZIKV infection and GBS or immune-mediated thrombocytopaenia purpura. We also describe the transition of the review into a living systematic review, a review that is continually updated. Between 30.05.2016 and 18.01.2017, we identified 2413 publications, of which 101 publications were included. The evidence added in this update confirms the conclusion of a causal association between ZIKV and adverse congenital outcomes. New findings expand the evidence base in the dimensions of biological plausibility, strength of association, animal experiments and specificity. For GBS, the body of evidence has grown during the search period for update 1, but only for dimensions that were already populated in the previous version. There is still a limited understanding of the biological pathways that potentially cause the occurrence of autoimmune disease following ZIKV infection. This systematic review confirms previous conclusions that ZIKV is a cause of congenital abnormalities, including microcephaly, and is a trigger of GBS. The transition to living systematic review techniques and methodology provides a proof of concept for the use of these methods to synthesise evidence about an emerging pathogen such as ZIKV.
Topics: Animals; Brain; Female; Fetus; Global Health; Guillain-Barre Syndrome; Humans; Nervous System Malformations; Pregnancy; Pregnancy Complications, Infectious; Zika Virus; Zika Virus Infection
PubMed: 30631437
DOI: 10.12688/f1000research.13704.1 -
Frontiers in Immunology 2024The objective of this study is to evaluate the risk of secondary autoimmune diseases in multiple sclerosis (MS) patients treated with alemtuzumab (ALZ) through a... (Meta-Analysis)
Meta-Analysis
BACKGROUND AND PURPOSE
The objective of this study is to evaluate the risk of secondary autoimmune diseases in multiple sclerosis (MS) patients treated with alemtuzumab (ALZ) through a meta-analysis.
METHODS
PubMed, Web of Science, OVID, EMBASE, and Cochrane central register of controlled trials were searched. Information and data were screened and extracted by 2 researchers. The obtained data were analyzed using the R software meta package. Quality assessment was conducted using the Newcastle-Ottawa Scale (NOS). The causes of heterogeneity were analyzed using subgroup analysis and sensitivity analysis. Publication bias was evaluated using funnel plots and Egger's test.
RESULTS
The search retrieved a total of 3530 papers from the databases. After screening, a total of 37 studies were included in the meta-analysis. The analysis results indicate that the pooled incidence rate of overall secondary autoimmune events (SAEs) in the included studies was 0.2824 [0.2348, 0.3300] (I²=94%, p<0.01). The overall incidence of autoimmune thyroid events (ATE) was 0.2257 [0.1810, 0.2703] (I²=94%, p<0.01). Among them, the rate of serious autoimmune thyroid events (SATE) was 0.0541 [0.0396, 0.0687] (I²=0%, p=0.44). The incidence rates of different thyroid events were as follows: Graves' disease (GD), 0.2266 [0.1632, 0.2900] (I²=83%, p<0.01); Hashimoto thyroiditis (HT), 0.0844 [0.0000, 0.2262] (I²=81%, p=0.02); Hashimoto thyroiditis with hypothyroidism (HTwH), 0.0499 [0.0058, 0.0940] (I²=37%, p=0.21); fluctuating thyroid dysfunction (FTD), 0.0219 [0.0015, 0.0424] (I²=0%, p=0.40); transient thyroiditis (TT), 0.0178 [0.0062, 0.0295] (I²=0%, p=0.94). The overall incidence of hematological events was 0.0431 [0.0274, 0.0621] (I²=70%, p<0.01). The incidence rates from high to low were as follows: lymphopenia, 0.0367 [0.0000, 0.0776] (I²=81%, p=0.02); Idiopathic thrombocytopenic purpura (ITP), 0.0258 [0.0199, 0.0323] (I²=25%, p=0.15); Hemolytic anemia (HA), 0.0177 [0.0081, 0.0391] (I²=29%, p=0.23); pancytopenia, 0.0136 [0.0000, 0.0314] (I²=0%, p=0.67); Neutropenia, 0.0081 [0.0000, 0.0183] (I²=0%, p=0.42). After excluding thyroid and hematological diseases, the combined incidence of other related SAEs was 0.0061 [0.0014, 0.0109] (I²=50%, p=0.02). The incidence of each disease ranked from highest to lowest as: skin psoriasis (SP), 0.0430 [0.0000, 0.0929] (I²=0%, p=0.57); alopecia areata (AA), 0.0159 [0.0024, 0.0372] (I²=19%, p=0.29); vitiligo, 0.0134 [0.0044, 0.0223] (I²=0%, p=0.81); inflammatory atrichia (IA), 0.0103 [0.0000, 0.0232] (I²=0%, p=0.43); chronic urticaria (CU), 0.0107 [0.0000, 0.0233] (I²=0%, p=0.60); and nephropathy, 0.0051 [0.0000, 0.0263] (I²=62%, p=0.02).
CONCLUSION
The occurrence of secondary autoimmune diseases in patients with MS treated with ALZ is noteworthy, particularly in the form of thyroid events and hematological events. Clinicians should monitor the overall condition of patients promptly for early management and avoid delayed diagnosis and treatment.
SYSTEMATIC REVIEW REGISTRATION
inplasy.com/inplasy-2024-4-0048/, identifier INPLASY202440048.
Topics: Humans; Alemtuzumab; Multiple Sclerosis; Autoimmune Diseases; Incidence; Hashimoto Disease
PubMed: 38690271
DOI: 10.3389/fimmu.2024.1343971 -
Hematology/oncology and Stem Cell... Jun 2019Cutaneous immunoglobulin (Ig) amyloid light-chain (AL) amyloidosis associated with overt multiple myeloma (MM) is rare and optimal treatment is not well defined. The...
OBJECTIVE/BACKGROUND
Cutaneous immunoglobulin (Ig) amyloid light-chain (AL) amyloidosis associated with overt multiple myeloma (MM) is rare and optimal treatment is not well defined. The recently developed highly efficacious MM therapy has brought on a new set of challenges to this field for consideration. The goal of this paper is to describe the characteristics of cutaneous manifestations of systemic AL amyloidosis associated with MM according to age, sex, race, Ig type, plasma cell percentage, and cytogenetic and fluorescent in situ hybridization studies along with their outcomes.
METHODS
An electronic search of the PubMed database was performed to obtain key literature in AL amyloidosis and MM, using the following search terms: multiple myeloma, immunoglobulin light chain amyloidosis, and cutaneous amyloidosis. The search results were narrowed by selecting studies in English. Results were confined to the following articles types: case reports, case series, and systematic reviews.
RESULTS
We identified 32 cases from the PubMed database search and examined their potential relevance. We found the following: (a) higher prevalence in women (two-thirds) and white population; (b) IgG and IgA were equally distributed with lambda (λ) light chain occurring in 53-66% of cases; (c) majority of cases (56%) presented as hemorrhagic bullous lesions, followed by purpura/ecchymosis in 25% of cases; and (d) majority (64%) died within 6 months since diagnosis.
CONCLUSIONS
We reviewed the constellation of the cutaneous manifestations of AL amyloidosis with concurrent MM. We found a female predominance, and more than half presented as hemorrhagic bullous lesions. There is a preponderance of λ light chains over kappa (κ) light chains, both as a free light chain (15% vs. 4%) and as an intact Ig (38% vs. 24%; absolute number of 14 vs. 7 patients, respectively). In the subgroup of patients with bullous skin lesions, λ light chain was present in eight cases and κ light chain in seven cases. All κ light chain subtypes presented with bullous lesions and no other cutaneous types of lesions. They carried very poor prognosis with majority of cases surviving only 6 months, much worse than overall patients with AL amyloidosis without myeloma or myeloma without amyloidosis.
Topics: Adult; Female; Humans; Immunoglobulin Light-chain Amyloidosis; Male; Middle Aged; Multiple Myeloma; Neoplasm Proteins; Sex Factors; Skin Neoplasms
PubMed: 30261180
DOI: 10.1016/j.hemonc.2018.09.003 -
The Journal of International Medical... Oct 2020We reviewed relevant research on rituximab (RTX) treatment for pediatric immune thrombocytopenia (ITP) to elucidate the efficacy and safety of RTX. (Meta-Analysis)
Meta-Analysis
OBJECTIVE
We reviewed relevant research on rituximab (RTX) treatment for pediatric immune thrombocytopenia (ITP) to elucidate the efficacy and safety of RTX.
METHODS
Prospective clinical trials of RTX for the treatment of pediatric ITP were collected by searching the PubMed, Cochrane Library, Web of Science, and OVID: EMBASE databases and ClinicalTrials.gov. We examined rates of overall response (OR), complete response (CR), partial response (PR), sustained response (SR), relapse (R), and adverse drug reaction (ADR). The Methodological Index for Nonrandomized Studies scale was used, and sensitivity analyses were performed.
RESULTS
For five studies, including 100 patients, the pooled OR, CR, PR, SR, R, and ADR rates were 52% (95% CI: 0.36-0.77, = 78%), 52% (95% CI: 0.41-0.67, = 45%), 18% (95% CI: 0.10-0.33, = 33%), 43% (95% CI: 0.29-0.63, = 0%), 25% (95% CI: 0.06-0.96, = 52%), and 30% (95% CI: 0.15-0.58, = 64%), respectively.
CONCLUSION
There is evidence, albeit low quality, that RTX may be a better second-line therapy than splenectomy for children with ITP; however, its efficacy and safety need to be validated by further high-quality clinical trials, such as randomized controlled trials.
Topics: Child; Humans; Minors; Prospective Studies; Purpura, Thrombocytopenic, Idiopathic; Rituximab; Thrombocytopenia
PubMed: 33115308
DOI: 10.1177/0300060520962348