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Journal of Oncology Practice Jun 2016The unexpected occurrence of thrombotic microangiopathy (TMA), characterized by microangiopathic hemolytic anemia and thrombocytopenia, in a patient with cancer requires... (Review)
Review
The unexpected occurrence of thrombotic microangiopathy (TMA), characterized by microangiopathic hemolytic anemia and thrombocytopenia, in a patient with cancer requires urgent diagnosis and appropriate management. TMA is a term used to describe multiple syndromes caused by microvascular thrombosis, including thrombotic thrombocytopenic purpura (TTP), Shiga toxin-mediated hemolytic uremic syndrome, and complement-mediated TMA. In patients with cancer, systemic microvascular metastases and bone marrow involvement can cause microangiopathic hemolytic anemia and thrombocytopenia. This occurs most often in patients with known metastatic cancer, but microangiopathic hemolytic anemia and thrombocytopenia may occur unexpectedly in patients without known metastatic disease or be the presenting features of undiagnosed cancer. TMA may also be caused by commonly used chemotherapy agents, either through dose-dependent toxicity or an acute immune-mediated reaction. These causes of TMA must be distinguished from TTP, which results from a severe deficiency of ADAMTS13 and is the most common cause of TMA among adults without cancer. The importance of this distinction is to avoid inappropriate use of plasma exchange, which is associated with major complications. Plasma exchange is the essential treatment for TTP, but it has no known benefit for patients with cancer-induced or drug-induced TMA. We will describe cancer-induced and drug-induced TMA using the experience of the Oklahoma TTP-Hemolytic Uremic Syndrome Registry and data from a systematic review of all published reports of drug-induced TMA. We will illustrate the principles of evaluation and management of these disorders with patients' stories.
Topics: Anemia, Hemolytic; Antineoplastic Agents; Humans; Neoplasms; Thrombocytopenia
PubMed: 27288467
DOI: 10.1200/JOP.2016.012096 -
Epilepsia Mar 2022This is a systematic review aimed at summarizing the evidence related to instruments that have been developed to measure stigma or attitudes toward epilepsy and on...
Epilepsy-related stigma and attitudes: Systematic review of screening instruments and interventions - Report by the International League Against Epilepsy Task Force on Stigma in Epilepsy.
OBJECTIVE
This is a systematic review aimed at summarizing the evidence related to instruments that have been developed to measure stigma or attitudes toward epilepsy and on stigma-reducing interventions.
METHODS
This review followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) standards. A broad literature search (1985-2019) was performed in 13 databases. Articles were included if they described the development and testing of psychometric properties of an epilepsy-related stigma or attitude scale or stigma-reducing interventions. Two reviewers independently screened abstracts, reviewed full-text articles, and extracted data. Basic descriptive statistics are reported.
RESULTS
We identified 4234 abstracts, of which 893 were reviewed as full-text articles. Of these, 38 met inclusion criteria for an instrument development study and 30 as a stigma-reduction intervention study. Most instruments were initially developed using well-established methods and were tested in relatively large samples. Most intervention studies involved educational programs for adults with pre- and post-evaluations of attitudes toward people with epilepsy. Intervention studies often failed to use standardized instruments to quantify stigmatizing attitudes, were generally underpowered, and often found no evidence of benefit or the benefit was not sustained. Six intervention studies with stigma as the primary outcome had fewer design flaws and showed benefit. Very few or no instruments were validated for regional languages or culture, and there were very few interventions tested in some regions.
SIGNIFICANCE
Investigators in regions without instruments should consider translating and further developing existing instruments rather than initiating the development of new instruments. Very few stigma-reduction intervention studies for epilepsy have been conducted, study methodology in general was poor, and standardized instruments were rarely used to measure outcomes. To accelerate the development of effective epilepsy stigma-reduction interventions, a paradigm shift from disease-specific, siloed trials to collaborative, cross-disciplinary platforms based upon unified theories of stigma transcending individual conditions will be needed.
Topics: Adult; Advisory Committees; Attitude; Epilepsy; Humans; Psychometrics; Social Stigma
PubMed: 34985766
DOI: 10.1111/epi.17133 -
Seminars in Arthritis and Rheumatism Apr 2019To characterize levamisole-induced vasculopathy.
OBJECTIVE
To characterize levamisole-induced vasculopathy.
METHODS
We performed a systematic review searching MEDLINE for articles published from 1972 to 2016.
RESULTS
We retrieved 357 references and abstracts and selected 111 articles. Levamisole-induced vasculopathy was reported in 192 patients, with a female predominance (n = 122, 63.5%). Median [interquartile range] age was 44 [38-50]. Skin was the most frequently involved organ (n = 182, 94.8%). Cutaneous lesions were mostly on the face (n = 136, 70.8%), especially the ears. Purpura (n = 131, 68.2%) was the most reported cutaneous lesion. Organ involvement included acute renal failure (n = 24, 12.5%), and pulmonary involvement (n = 20, 10.4%). Anti-neutrophil cytoplasmic antibodies (ANCAs) were found in 167/178 patients (93.8%), with both anti-myeloperoxydase and anti-proteinase 3 specificity reported in 51/118 patients (43.2%). Anti-phospholipid (APL) antibodies were found in 93/137 patients (67.9%). Leukopenia was detected in 69/138 patients (50%). Skin biopsies identified vasculitis and thrombotic vasculopathy in 73/148 (49.3%) and 62/148 (41.9%) patients, respectively. The outcome was favourable in 116/134 patients (86.6%), but relapses were reported in 33 (28.4%), mainly on levamisole re-exposure.
CONCLUSION
Levamisole-induced vasculopathy is characterized by a female predominance, skin involvement, ANCA and/or APL antibody positivity, leukopenia, vasculitis or vascular thrombotic histological lesions, and despite possible systemic involvement, a favourable outcome with levamisole interruption.
Topics: Acute Kidney Injury; Adult; Antibodies, Antineutrophil Cytoplasmic; Antibodies, Antiphospholipid; Antirheumatic Agents; Female; Humans; Leukopenia; Levamisole; Male; Middle Aged; Purpura; Skin; Vasculitis
PubMed: 30166200
DOI: 10.1016/j.semarthrit.2018.07.010 -
International Journal of Surgery... Sep 2017An accessory spleen (AS) is a lobule of splenic tissue found in ectopic locations. Identification of AS is particularly important in patients with immune... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
An accessory spleen (AS) is a lobule of splenic tissue found in ectopic locations. Identification of AS is particularly important in patients with immune thrombocytopenia (ITP) requiring splenectomy as unrecognized AS can later cause refractory symptoms. The AS can also be a source of significant intraabdominal hemorrhage. The aim of this meta-analysis was to systematically analyze the data on the prevalence, number, location, and morphometry of AS.
MATERIALS AND METHODS
An extensive search of the major electronic databases was conducted to identify all studies that reported relevant data on the AS. No date or language restrictions were applied. Data on the study type, the prevalence of AS, location, morphometry and number of AS per patient were extracted from the eligible studies and pooled into a meta-analysis.
RESULTS
A total of 81 studies (n = 22,487 subjects) were included into the quantitative analysis. The overall pooled prevalence of AS was 14.5% (95%CI: 12.4-16.7), while the pooled prevalence of AS in ITP patients was 16.7% (95%CI: 12.1-21.7). The majority of accessory spleens were located in the splenic hilum (62.1% [95%CI:51.5-76.3]). Moreover, 26% of ITP patients with an AS have more than one.
CONCLUSIONS
The findings of this study provide an evidence-based foundation of anatomical knowledge about the AS. Surgeons should take particular caution in identifying an AS, as unnoticed AS during splenectomy can lead to recurrence of hematological diseases or can be a potential source of bleeding in the future.
Topics: Adult; Choristoma; Female; Humans; Prevalence; Purpura, Thrombocytopenic, Idiopathic; Spleen; Splenectomy
PubMed: 28716661
DOI: 10.1016/j.ijsu.2017.07.045 -
Epilepsia Jan 2016The International League Against Epilepsy (ILAE) Epilepsy Guidelines Task Force, composed of 14 international members, was established in 2011 to identify, using... (Review)
Review
OBJECTIVE
The International League Against Epilepsy (ILAE) Epilepsy Guidelines Task Force, composed of 14 international members, was established in 2011 to identify, using systematic review methodology, international epilepsy clinical care guidelines, assess their quality, and determine gaps in areas of need of development.
METHODS
A systematic review of the literature (1985-2014) was performed in six electronic databases (e.g. Medline, Embase) using a broad search strategy without initial limits to language or study design. Six gray literature databases (e.g., American Academy of Neurology [AAN], ILAE) were also searched to minimize publication bias. Two independent reviewers screened abstracts, reviewed full text articles, and performed data abstraction. Descriptive statistics and a meta-analysis were generated.
RESULTS
The search identified 10,926 abstracts. Of the 410 articles selected for full text review, 63 met our eligibility criteria for a guideline. Of those included, 54 were in English and 9 were in other languages (French, Spanish, and Italian). Of all guidelines, 29% did not specify the target age groups, 27% were focused on adults, 22% included only children, and 6% specifically addressed issues related to women with epilepsy. Guidelines included in the review were most often aimed at guiding clinical practice for status epilepticus (n = 7), first seizure (n = 6), drug-resistant epilepsy (n = 5), and febrile seizures (n = 4), among others. Most of the guidelines were therapeutic (n = 35) or diagnostic (n = 16) in nature. The quality of the guidelines using a 1-7 point scale (7 = highest) varied and was moderate overall (mean = 4.99 ± 1.05 [SD]).
SIGNIFICANCE
We identified substantial gaps in topics (e.g., epilepsy in the elderly) and there was considerable heterogeneity in methodologic quality. The findings should offer a valuable resource for health professionals caring for people with epilepsy, since they will help guide the prioritization, development, and dissemination of future epilepsy-related guidelines.
Topics: Advisory Committees; Databases, Factual; Epilepsy; Evidence-Based Medicine; Humans; Practice Guidelines as Topic; Societies, Medical
PubMed: 26659723
DOI: 10.1111/epi.13273 -
Vaccines Sep 2022With the recent outbreak of the COVID-19 pandemic and emergency use authorization of anti-SARS-CoV-2 vaccines, reports of post-vaccine immune thrombocytopenia (ITP) have... (Review)
Review
With the recent outbreak of the COVID-19 pandemic and emergency use authorization of anti-SARS-CoV-2 vaccines, reports of post-vaccine immune thrombocytopenia (ITP) have gained attention. With this systematic review, we aim to analyze the clinical characteristics, therapeutic strategies, and outcomes of patients presenting with ITP after receiving COVID-19 vaccination. Medline, Embase, and Ebsco databases were systematically explored from inception until 1 June 2022. Case reports and case series investigating the association between the anti-SARS-CoV-2 vaccine and ITP were included. We found a total of 66 patients. The mean age of presentation was 63 years with a female preponderance (60.6%). Sixteen patients had pre-existing ITP. The mean time from vaccine administration to symptom onset was 8.4 days. More ITP events were triggered by mRNA vaccines (BNT162b2 (n = 29) > mRNA-1273 (n = 13)) than with adenoviral vaccines (ChAdOx1-S AstraZeneca (n = 15) > Ad26.COV2-S (n = 9)). Most of the patients were treated with steroids or IVIG, or both. The overall outcome was promising, with no reported deaths. Our review attempts to increase awareness among physicians while evaluating patients presenting with thrombocytopenia after receiving the vaccine. In our solicited opinion, the rarity of these events and excellent outcomes for patients should not change views regarding the benefits provided by immunization.
PubMed: 36146522
DOI: 10.3390/vaccines10091444 -
International Journal of Molecular... Feb 2023Patients with IgA nephropathy (IgAN), including Henoch-Schönlein purpura nephritis (HSP), who present with rapidly progressive glomerulonephritis (RPGN) have a poor...
Patients with IgA nephropathy (IgAN), including Henoch-Schönlein purpura nephritis (HSP), who present with rapidly progressive glomerulonephritis (RPGN) have a poor prognosis despite aggressive immunosuppressive therapy. The utility of plasmapheresis/plasma exchange (PLEX) for IgAN/HSP is not well established. This systematic review aims to assess the efficacy of PLEX for IgAN and HSP patients with RPGN. A literature search was conducted using MEDLINE, EMBASE, and through Cochrane Database from inception through September 2022. Studies that reported outcomes of PLEX in IgAN or HSP patients with RPGN were enrolled. The protocol for this systematic review is registered with PROSPERO (no. CRD42022356411). The researchers systematically reviewed 38 articles (29 case reports and 9 case series articles) with a total of 102 RPGN patients (64 (62.8%) had IgAN and 38 (37.2%) had HSP). The mean age was 25 years and 69% were males. There was no specific PLEX regimen utilized in these studies, but most patients received at least 3 PLEX sessions that were titrated based on the patient's response/kidney recovery. The number of PLEX sessions ranged from 3 to 18, and patients additionally received steroids and immunosuppressive treatment (61.6% of patients received cyclophosphamide). Follow-up time ranged from 1 to 120 months, with the majority being followed for at least 2 months after PLEX. Among IgAN patients treated with PLEX, 42.1% ( = 27/64) achieved remission; 20.3% ( = 13/64) achieved complete remission (CR) and 18.7% ( = 12/64) partial remission (PR). 60.9% ( = 39/64) progressed to end-stage kidney disease (ESKD). Among HSP patients treated with PLEX, 76.3% (n = 29/38) achieved remission; of these, 68.4% ( = 26/38) achieved CR and 7.8% achieved ( = 3/38) PR. 23.6% ( = 9/38) progressed to ESKD. Among kidney transplant patients, 20% (n = 1/5) achieved remission and 80% ( = 4/5) progressed to ESKD. Adjunctive plasmapheresis/plasma exchange with immunosuppressive therapy showed benefits in some HSP patients with RPGN and possible benefits in IgAN patients with RPGN. Future prospective, multi-center, randomized clinical studies are needed to corroborate this systematic review's findings.
Topics: Adult; Female; Humans; Male; Glomerulonephritis, IGA; IgA Vasculitis; Kidney Failure, Chronic; Plasma Exchange
PubMed: 36835388
DOI: 10.3390/ijms24043977 -
Expert Opinion on Pharmacotherapy Oct 2017We conducted a systematic review to assess the efficacy and safety of Thrombopoietin-receptor agonists (TPOras) for pediatric immune thrombocytopenia (ITP). (Review)
Review
OBJECTIVE
We conducted a systematic review to assess the efficacy and safety of Thrombopoietin-receptor agonists (TPOras) for pediatric immune thrombocytopenia (ITP).
METHODS
We searched PubMed, Embase and Cochrane Library from their earliest records to January 2017. Randomized controlled trials (RCTs) were included. Primary outcomes were durable response and clinically significant bleeding. Secondary outcomes were overall response, overall bleeding events, the use of rescue medication and adverse events (AEs).
RESULTS
Five randomized RCTs (261participants) were included. Compared with placebo group, the proportion of patients achieving durable platelet response was significantly higher in Eltrombopag (P = 0.0004) or Romiplostim (P = 0.002) group, so was the overall response in Eltrombopag [RR = 2.64, 95% CI (1.58, 4.44)] or Romiplostim [RR = 5.05, 95% CI (2.21, 11.53)] group. Both clinically significant bleeding (P = 0.04) and total bleeding (P = 0.01) in Eltrombopag group were significantly less frequent than those in placebo group, while no significant difference between Romiplostim and placebo group. The proportion of patients receiving rescue medication, the incidence of overall AEs and serious AEs between TPO-receptor agonists and placebo group were not significantly different.
CONCLUSION
TPOras might improve both durable and overall platelet response in pediatric ITP, compared with placebo.
Topics: Benzoates; Child; Hemorrhage; Humans; Hydrazines; Incidence; Platelet Count; Purpura, Thrombocytopenic, Idiopathic; Pyrazoles; Randomized Controlled Trials as Topic; Receptors, Fc; Receptors, Thrombopoietin; Recombinant Fusion Proteins; Thrombopoietin
PubMed: 28845713
DOI: 10.1080/14656566.2017.1373091 -
Scientific Reports Dec 2016Immune thrombocytopenia (ITP) is an autoimmune disease characterized by increased platelet destruction and impaired platelet production. In this study, we conducted a... (Meta-Analysis)
Meta-Analysis Review
Immune thrombocytopenia (ITP) is an autoimmune disease characterized by increased platelet destruction and impaired platelet production. In this study, we conducted a systematic review and meta-analysis to determine the efficacy and safety of thrombopoietin receptor agonists (TPO-RAs) in primary ITP patients. Thirteen randomized controlled trials were included in this study, the pooled results of which demonstrated that TPO-RAs significantly increased platelet response (R) and durable response (DR) rates [risk ratio (RR): 2.77, 95% confidence interval (CI): 2.01-3.82, P = 5.9 × 10; RR: 7.52, 95% CI: 3.94-14.35, P = 9.2 × 10; respectively] and that TPO-RAs significantly reduced the incidences of any or severe bleeding events (RR: 0.80, 95% CI: 0.67-0.95, P = 0.013; RR: 0.52, 95% CI: 0.27-0.99, P = 0.048; respectively). Moreover, our results indicated that there was a significant reduction in the proportion of patients needing rescue medications in the TPO-RA groups compared with the control groups (RR: 0.50, 95% CI: 0.42-0.59, P = 2.0 × 10) and that the rates of any or severe adverse events were similar between the TPO-RA and control regimens (RR: 1.01, 95% CI: 0.92-1.10; RR: 0.74, 95% CI: 0.54-1.01; respectively). These findings demonstrate that TPO-RAs are an effective and safe second-line treatment option for primary ITP patients.
Topics: Blood Platelets; Humans; Purpura, Thrombocytopenic, Idiopathic; Randomized Controlled Trials as Topic; Receptors, Thrombopoietin
PubMed: 27991534
DOI: 10.1038/srep39003 -
Journal of the European Academy of... Jul 2018Acne vulgaris is a multifaceted skin disorder, affecting more than 85% of young individuals worldwide. Pharmacological therapy is not always desirable because of the... (Review)
Review
BACKGROUND
Acne vulgaris is a multifaceted skin disorder, affecting more than 85% of young individuals worldwide. Pharmacological therapy is not always desirable because of the development of antibiotic resistance or the potential risk of adverse effects. Non-pharmacological therapies can be viable alternatives for conventional therapies. However, sufficient evidence-based support in the efficacy and safety of non-pharmacological therapies is lacking.
OBJECTIVE
To assess the efficacy and safety of several non-pharmacological therapies in the treatment of acne vulgaris.
METHODS
A systematic literature review, including a best-evidence synthesis, was performed to identify literature. Three electronic databases were accessed and searched for studies published between January 2000 and May 2017.
RESULTS
Thirty-three eligible studies were included in our systematic review. Three main types of non-pharmacological therapies were identified laser- and light-based therapies, chemical peels and fractional microneedling radiofrequency. The majority of the included studies demonstrated a significant reduction in acne lesions. However, only seven studies had a high methodologic quality. Based on these seven trials, a best-evidence synthesis was conducted. Strong evidence was found for glycolic acid (10-40%). Moderate evidence was found for amino fruit acid (20-60%), intense pulsed light (400-700 and 870-1200 nm) and the diode laser (1450 nm). Initially, conflicting evidence was found for pulsed dye laser (585-595 nm). The most frequently reported side-effects for non-pharmacological therapies included erythema, tolerable pain, purpura, oedema and a few cases of hyperpigmentation, which were in most cases mild and transient.
CONCLUSION
Circumstantial evidence was found for non-pharmacological therapies in the treatment of acne vulgaris. However, the lack of high methodological quality among included studies prevented us to draw clear conclusions, regarding a stepwise approach. Nevertheless, our systematic review including a best-evidence synthesis did create order and structure in resulting outcomes in which a first step towards future research is generated.
Topics: Acne Vulgaris; Chemexfoliation; Evidence-Based Medicine; Humans; Laser Therapy; Needles; Phototherapy; Radiofrequency Therapy
PubMed: 29444375
DOI: 10.1111/jdv.14881