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Pediatric Critical Care Medicine : a... Jan 2022To present the recommendations and consensus statements with supporting literature for plasma and platelet transfusions in critically ill neonates and children...
Plasma and Platelet Transfusions Strategies in Neonates and Children Undergoing Cardiac Surgery With Cardiopulmonary Bypass or Neonates and Children Supported by Extracorporeal Membrane Oxygenation: From the Transfusion and Anemia EXpertise Initiative-Control/Avoidance of Bleeding.
OBJECTIVES
To present the recommendations and consensus statements with supporting literature for plasma and platelet transfusions in critically ill neonates and children undergoing cardiac surgery with cardiopulmonary bypass or supported by extracorporeal membrane oxygenation from the Transfusion and Anemia EXpertise Initiative-Control/Avoidance of Bleeding.
DESIGN
Systematic review and consensus conference of international, multidisciplinary experts in platelet and plasma transfusion management of critically ill children.
SETTING
Not applicable.
PATIENTS
Critically ill neonates and children following cardiopulmonary bypass or supported by extracorporeal membrane oxygenation.
INTERVENTIONS
None.
MEASUREMENTS AND MAIN RESULTS
A panel of nine experts developed evidence-based and, when evidence was insufficient, expert-based statements for plasma and platelet transfusions in critically ill neonates and children following cardiopulmonary bypass or supported by extracorporeal membrane oxygenation. These statements were reviewed and ratified by the 29 Transfusion and Anemia EXpertise Initiative-Control/Avoidance of Bleeding experts. A systematic review was conducted using MEDLINE, EMBASE, and Cochrane Library databases, from inception to December 2020. Consensus was obtained using the Research and Development/University of California, Los Angeles Appropriateness Method. Results were summarized using the Grading of Recommendations Assessment, Development, and Evaluation method. We developed one good practice statement, two recommendations, and three expert consensus statements.
CONCLUSIONS
Whereas viscoelastic testing and transfusion algorithms may be considered, in general, evidence informing indications for plasma and platelet transfusions in neonatal and pediatric patients undergoing cardiac surgery with cardiopulmonary bypass or those requiring extracorporeal membrane oxygenation support is lacking.
Topics: Anemia; Blood Component Transfusion; Cardiac Surgical Procedures; Cardiopulmonary Bypass; Child; Critical Care; Critical Illness; Erythrocyte Transfusion; Evidence-Based Medicine; Extracorporeal Membrane Oxygenation; Hemorrhage; Humans; Infant, Newborn; Plasma; Platelet Transfusion
PubMed: 34989703
DOI: 10.1097/PCC.0000000000002856 -
Pediatric Critical Care Medicine : a... Jan 2022To present consensus statements and supporting literature for plasma and platelet transfusions in critically ill children with severe trauma, traumatic brain injury,...
Plasma and Platelet Transfusion Strategies in Critically Ill Children Following Severe Trauma, Traumatic Brain Injury, and/or Intracranial Hemorrhage: From the Transfusion and Anemia EXpertise Initiative-Control/Avoidance of Bleeding.
OBJECTIVES
To present consensus statements and supporting literature for plasma and platelet transfusions in critically ill children with severe trauma, traumatic brain injury, and/or intracranial hemorrhage from the Transfusion and Anemia EXpertise Initiative-Control/Avoidance of Bleeding.
DESIGN
Systematic review and consensus conference of international, multidisciplinary experts in platelet and plasma transfusion management of critically ill children.
SETTING
Not applicable.
PATIENTS
Critically ill neonates and children with severe trauma, traumatic brain injury, and/or intracranial hemorrhage.
INTERVENTIONS
None.
MEASUREMENTS AND MAIN RESULTS
A panel of eight experts developed expert-based statements for plasma and platelet transfusions in critically ill neonates and children with severe trauma, traumatic brain injury, and/or intracranial hemorrhage. These statements were reviewed and ratified by the 29 Transfusion and Anemia EXpertise Initiative-Control/Avoidance of Bleeding experts. A systematic review was conducted using MEDLINE, EMBASE, and Cochrane Library databases, from inception to December 2020. Consensus was obtained using the Research and Development/University of California, Los Angeles Appropriateness Method. Results were summarized using the Grading of Recommendations Assessment, Development, and Evaluation method. We developed one good practice statement and six expert consensus statements.
CONCLUSIONS
The lack of evidence precludes proposing recommendations on monitoring of the coagulation system and on plasma and platelets transfusion in critically ill pediatric patients with severe trauma, severe traumatic brain injury, or nontraumatic intracranial hemorrhage.
Topics: Anemia; Blood Component Transfusion; Brain Injuries, Traumatic; Child; Critical Care; Critical Illness; Erythrocyte Transfusion; Evidence-Based Medicine; Hemorrhage; Humans; Infant, Newborn; Intracranial Hemorrhages; Plasma; Platelet Transfusion
PubMed: 34989702
DOI: 10.1097/PCC.0000000000002855 -
Pediatric Critical Care Medicine : a... Jan 2022To present consensus statements and supporting literature for plasma and platelet product variables and related laboratory testing for transfusions in general critically...
What Laboratory Tests and Physiologic Triggers Should Guide the Decision to Administer a Platelet or Plasma Transfusion in Critically Ill Children and What Product Attributes Are Optimal to Guide Specific Product Selection? From the Transfusion and Anemia EXpertise Initiative-Control/Avoidance of...
OBJECTIVES
To present consensus statements and supporting literature for plasma and platelet product variables and related laboratory testing for transfusions in general critically ill children from the Transfusion and Anemia EXpertise Initiative-Control/Avoidance of Bleeding.
DESIGN
Systematic review and consensus conference of international, multidisciplinary experts in platelet and plasma transfusion management of critically ill children.
SETTING
Not applicable.
PATIENTS
Critically ill pediatric patients at risk of bleeding and receiving plasma and/or platelet transfusions.
INTERVENTIONS
None.
MEASUREMENTS AND MAIN RESULTS
A panel of 10 experts developed evidence-based and, when evidence was insufficient, expert-based statements for laboratory testing and blood product attributes for platelet and plasma transfusions. These statements were reviewed and ratified by the 29 Transfusion and Anemia EXpertise Initiative - Control/Avoidance of Bleeding experts. A systematic review was conducted using MEDLINE, EMBASE, and Cochrane Library databases, from inception to December 2020. Consensus was obtained using the Research and Development/University of California, Los Angeles Appropriateness Method. Results were summarized using the Grading of Recommendations Assessment, Development, and Evaluation method. We developed five expert consensus statements and two recommendations in answer to two questions: what laboratory tests and physiologic triggers should guide the decision to administer a platelet or plasma transfusion in critically ill children; and what product attributes are optimal to guide specific product selection?
CONCLUSIONS
The Transfusion and Anemia EXpertise Initiative-Control/Avoidance of Bleeding program provides some guidance and expert consensus for the laboratory and blood product attributes used for decision-making for plasma and platelet transfusions in critically ill pediatric patients.
Topics: Anemia; Blood Component Transfusion; Child; Critical Care; Critical Illness; Erythrocyte Transfusion; Evidence-Based Medicine; Hemorrhage; Humans; Plasma; Platelet Transfusion
PubMed: 34989701
DOI: 10.1097/PCC.0000000000002854 -
Asian Journal of Surgery Feb 2022As the number of fusion levels increases, the complexity of spinal correction surgery also increases. Thus, we conducted this study to determine the safety and efficacy... (Meta-Analysis)
Meta-Analysis Review
As the number of fusion levels increases, the complexity of spinal correction surgery also increases. Thus, we conducted this study to determine the safety and efficacy of tranexamic acid (TXA) involving eight or more spinal fusion levels. According to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) and Assessing the Methodological Quality of Systematic Reviews (AMSTAR) guidelines, a search of the PubMed, Embase, CENTRAL, Web of Science, and ClinicalTrials.gov databases was conducted for relevant studies published prior to May 30, 2019. The primary outcomes, including blood loss and transfusion requirement, and the secondary outcomes, including general indices, postoperative hemoglobin, and coagulation function, were analyzed using Rev Man 5.3.5 software and STATA version 12.0. Eight randomized controlled trials (473 participants) were included in the study. Compared to the control treatments, TXA reduced intraoperative blood loss, total blood loss, transfusion volume, and prothrombin time. There were no significant differences between the TXA and non-TXA groups in transfusion rate, operative time, hospital stay, complications, hemoglobin level, and other coagulation function parameters. In the pediatric subgroup analysis, TXA additionally improved hemoglobin levels, platelet count, and prothrombin time international normalized ratio. The present meta-analysis showed that TXA reduced blood loss and transfusion volume in both adults and children. In pediatric patients, TXA led to a greater benefit in postoperative hemoglobin levels and coagulation function. Intravenous TXA is safe and effective in children with eight or more spinal corrective levels.
Topics: Adult; Antifibrinolytic Agents; Blood Loss, Surgical; Blood Transfusion; Child; Humans; Spine; Tranexamic Acid
PubMed: 34930653
DOI: 10.1016/j.asjsur.2021.06.065 -
Journal of Vascular Surgery Mar 2022Thromboelastography (TEG) is diagnostic modality that analyzes real-time blood coagulation parameters. Clinically, TEG primarily allows for directed blood component...
OBJECTIVE
Thromboelastography (TEG) is diagnostic modality that analyzes real-time blood coagulation parameters. Clinically, TEG primarily allows for directed blood component resuscitation among patients with acute blood loss and coagulopathy. The utilization of TEG has been widely adopted in among other surgical specialties; however, its use in vascular surgery is less prominent. We aimed to provide an up-to-date review of TEG utilization in vascular and endovascular surgery.
METHODS
Using Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines, a literature review with the Medical Subject Headings (MeSH) terms "TEG and arterial events", "TEG and vascular surgery", "TEG and vascular", "TEG and endovascular surgery", "TEG and endovascular", "TEG and peripheral artery disease", "TEG and prediction of arterial events", "TEG and prediction of complications ", "TEG and prediction of thrombosis", "TEG and prediction of amputation", and "TEG and amputation" was performed in Cochrane and PubMed databases to identify all peer-reviewed studies of TEG utilization in vascular surgery, written between 2000 and 2021 in the English language. The free-text and MeSH subheadings search terms included diagnosis, complications, physiopathology, surgery, mortality, and therapy to further restrict the articles. Studies were excluded if they were not in humans or pertaining to vascular or endovascular surgery. Additionally, case reports and studies with limited information regarding TEG utilization were excluded. Each study was independently reviewed by two researchers to assess for eligibility.
RESULTS
Of the 262 studies identified through the MeSH strategy, 15 studies met inclusion criteria and were reviewed and summarized. Literature on TEG utilization in vascular surgery spanned cerebrovascular disease (n = 3), peripheral arterial disease (n = 3), arteriovenous malformations (n = 1), venous thromboembolic events (n = 7), and perioperative bleeding and transfusion (n = 1). In cerebrovascular disease, TEG may predict the presence and stability of carotid plaques, analyze platelet function before carotid stenting, and compare efficacy of antiplatelet therapy after stent deployment. In peripheral arterial disease, TEG has been used to predict disease severity and analyze the impact of contrast on coagulation parameters. In venous disease, TEG may predict hypercoagulability and thromboembolic events among various patient populations. Finally, TEG can be utilized in the postoperative setting to predict hemorrhage and transfusion requirements.
CONCLUSIONS
This systematic review provides an up-to-date summarization of TEG utilization in multiple facets of vascular and endovascular surgery.
Topics: Blood Coagulation; Blood Loss, Surgical; Blood Transfusion; Endovascular Procedures; Humans; Monitoring, Intraoperative; Postoperative Hemorrhage; Predictive Value of Tests; Thrombelastography; Treatment Outcome; Vascular Diseases; Vascular Surgical Procedures
PubMed: 34788649
DOI: 10.1016/j.jvs.2021.11.037 -
Blood Advances Jan 2022Idiopathic purpura fulminans (IPF) is a rare but severe prothrombotic coagulation disorder that can occur after chickenpox or human herpesvirus 6 (HHV-6) infection. IPF...
Idiopathic purpura fulminans (IPF) is a rare but severe prothrombotic coagulation disorder that can occur after chickenpox or human herpesvirus 6 (HHV-6) infection. IPF leads to an autoantibody-mediated decrease in the plasma concentration of protein S. We conducted a retrospective multicenter study involving patients with IPF from 13 French pediatric centers and a systematic review of cases in published literature. Eighteen patients were included in our case series, and 34 patients were included as literature review cases. The median age was 4.9 years, and the diagnostic delay after the first signs of viral infection was 7 days. The lower limbs were involved in 49 patients (94%) with typical lesions. In all, 41 patients (78%) had a recent history of varicella-zoster virus infection, and 7 patients (14%) had been infected by HHV-6. Most of the patients received heparin (n = 51; 98%) and fresh frozen plasma transfusions (n = 41; 79%); other treatment options were immunoglobulin infusion, platelet transfusion, corticosteroid therapy, plasmapheresis, and coagulation regulator concentrate infusion. The antithrombin level and platelet count at diagnosis seemed to be associated with severe complications. Given the rarity of this disease, the creation of a prospective international registry is required to consolidate these findings.
Topics: Child; Child, Preschool; Humans; Chickenpox; Delayed Diagnosis; Prospective Studies; Protein S; Purpura Fulminans; Retrospective Studies
PubMed: 34788405
DOI: 10.1182/bloodadvances.2021005126 -
The Cochrane Database of Systematic... Nov 2021Acute limb ischaemia usually is caused by a blood clot blocking an artery or a bypass graft. Severe acute ischaemia will lead to irreversible damage to muscles and... (Review)
Review
BACKGROUND
Acute limb ischaemia usually is caused by a blood clot blocking an artery or a bypass graft. Severe acute ischaemia will lead to irreversible damage to muscles and nerves if blood flow is not restored in a few hours. Once irreversible damage occurs, amputation will be necessary and the condition can be life-threatening. Infusion of clot-busting drugs (thrombolysis) is a useful tool in the management of acute limb ischaemia. Fibrinolytic drugs are used to disperse blood clots (thrombi) to clear arterial occlusion and restore blood flow. Thrombolysis is less invasive than surgery. A variety of techniques are used to deliver fibrinolytic agents. This is an update of a review first published in 2004.
OBJECTIVES
To compare the effects of infusion techniques during peripheral arterial thrombolysis for treatment of patients with acute limb ischaemia.
SEARCH METHODS
The Cochrane Vascular Information Specialist searched the Cochrane Vascular Specialised Register, CENTRAL, MEDLINE, Embase, and CINAHL databases and World Health Organization International Clinical Trials Registry Platform and ClinicalTrials.gov trials registries to 20 October 2020. We undertook reference checking to identify additional studies.
SELECTION CRITERIA
We included all randomised controlled trials (RCTs) comparing infusion techniques for fibrinolytic agents in the treatment of acute limb ischaemia.
DATA COLLECTION AND ANALYSIS
We used standard methodological procedures as recommended by Cochrane. We assessed the risk of bias in included trials using the Cochrane 'Risk of bias' tool. We evaluated certainty of evidence using GRADE. For dichotomous outcomes, we calculated the odds ratio (OR) with the corresponding 95% confidence interval (CI). We were not able to carry out meta-analyses due to clinical heterogeneity, so we have reported the results and performed the comparisons narratively. The main outcomes of interest were amputation-free survival or limb salvage, amputation, mortality, vessel patency, duration of thrombolysis, and complications such as cerebrovascular accident and major and minor bleeding.
MAIN RESULTS
Nine studies with a total of 671 participants are included in this update. Trials covered a variety of infusion techniques, dosage regimens, and adjunctive agents. We grouped trials according to types of techniques assessed (e.g. intravenous and intra-arterial delivery of the agent, 'high-' and 'low-dose' regimens of the agent, continuous infusion and 'forced infusion' of the agent, use of adjunctive antiplatelet agents). We assessed the certainty of evidence as very low to low due to the limited power of individual studies to deliver clinically relevant results, small and heterogeneous study populations, use of different inclusion criteria by each study in terms of severity and duration of ischaemia, considerably different outcome measures between trials, and use of different fibrinolytic agents. This heterogeneity prevented pooling of data in meta-analyses. No regimen has been shown to confer benefit in terms of amputation-free survival (at 30 days), amputation, or death. For vessel patency, complete success was more likely with intra-arterial (IA) than with intravenous (IV) infusion (odds ratio (OR) 13.22, 95% confidence interval (CI) 2.79 to 62.67; 1 study, 40 participants; low-certainty evidence); radiological failure may be more likely with IV infusion (OR 0.02, 95% CI 0.00 to 0.38; 1 study, 40 participants; low-certainty evidence). Due to the small numbers involved in each arm and design differences between arms, it is not possible to conclude whether any technique offered any advantage over another. None of the treatment strategies clearly affected complications such as cerebrovascular accident or major bleeding requiring surgery or blood transfusion. Minor bleeding complications were more frequent in systemic (intravenous) therapy compared to intra-arterial infusion (OR 0.03, 95% CI 0.00 to 0.56; 1 study, 40 participants), and in high-dose compared to low-dose therapy (OR 0.11, 95% CI 0.01 to 0.96; 1 study, 63 participants). Limited evidence from individual trials appears to indicate that high-dose and forced-infusion regimens reduce the duration of thrombolysis. In one trial, the median duration of infusion was 4 hours (range 0.25 to 46) for the high-dose group and 20 hours (range 2 to 46) for the low-dose group. In a second trial, treatment using pulse spray was continued for a median of 120 minutes (range 40 to 310) compared with low-dose infusion for a median of 25 hours (range 2 to 60). In a third trial, the median duration of therapy was reduced with pulse spray at 195 minutes (range 90 to 1260 minutes) compared to continuous infusion at 1390 minutes (range 300 to 2400 minutes). However, none of the studies individually showed improvement in limb salvage at 30 days nor benefit for the amputation rate related to the technique of drug delivery. Similarly, no studies reported a clear difference in occurrence of cerebrovascular accident or major bleeding. Although 'high-dose' and 'forced-infusion' techniques achieved vessel patency in less time than 'low-dose' infusion, more minor bleeding complications may be associated (OR 0.11, 95% CI 0.01 to 0.96; 1 study, 72 participants; and OR 0.48, 95% CI 0.17 to 1.32; 1 study, 121 participants, respectively). Use of adjunctive platelet glycoprotein IIb/IIIa antagonists did not improve outcomes, and results were limited by inclusion of participants with non-limb-threatening ischaemia.
AUTHORS' CONCLUSIONS
There is insufficient evidence to show that any thrombolytic regimen provides a benefit over any other in terms of amputation-free survival, amputation, or 30-day mortality. The rate of CVA or major bleeding requiring surgery or blood transfusion did not clearly differ between regimens but may occur more frequently in high dose and IV regimens. This evidence was limited and of very low certainty. Minor bleeding may be more common with high-dose and IV regimens. In this context, thrombolysis may be an acceptable therapy for patients with marginally threatened limbs (Rutherford grade IIa) compared with surgery. Caution is advised for patients who do not have limb-threatening ischaemia (Rutherford grade I) because of risks of major haemorrhage, cerebrovascular accident, and death from thrombolysis.
Topics: Amputation, Surgical; Arteries; Fibrinolytic Agents; Humans; Ischemia; Thrombolytic Therapy
PubMed: 34786692
DOI: 10.1002/14651858.CD000985.pub3 -
Annals of Palliative Medicine Oct 2021To investigate the prevention of platelet transfusion refractoriness (PTR) by platelet antigen gene matching using literature search and meta-analysis. (Meta-Analysis)
Meta-Analysis
BACKGROUND
To investigate the prevention of platelet transfusion refractoriness (PTR) by platelet antigen gene matching using literature search and meta-analysis.
METHODS
PubMed (2000.1-2021.8), Embase (2000.1-2021.8), Cochrane (2010.1-2021.8), and the Chinese Biomedical Literature Database CBM (2010.1-2021.8) were selected as the search database platform. The keywords (HLA/Human leukocyte antigen), (HPA/Human platelet alloantigens), (genotyping/cross-match), platelet transfusion (PLT), and (CCI/Corrected Count Increment) were used for the joint search. After the literature was screened for inclusion and exclusion criteria, the Cochrane intervention handbook was used for bias risk assessment, and Revman 5.3.5 software was used for analysis to obtain the statistical forest plot and funnel plot.
RESULTS
The preliminary results revealed 255 publications, and seven (297 patients in total) were finally included in the quantitative analysis. A total of five publications reported comparison of the 1 h CCI index of HLA or HPA gene matching and PLT after random selection, and the heterogeneity test showed statistical difference (I2=49%, P=0.10). The combined statistical analysis results were: (MD =8.57, 95% CI: 7.30-9.80, Z=13.30, P<0.00001), and while six publications reported the effective rate index of PLT, and the heterogeneity test showed no statistical difference (I2=43%, P=0.12). The fixed effect mode was used to compare the effective rate of the two intervention methods (OR =4.90, 95% CI: 3.50-6.86, Z=9.23, P<0.00001).
DISCUSSION
HLA or HPA gene matching can improve the increment after PLT and reduce the incidence of ineffective PLT.
Topics: Antigens, Human Platelet; Blood Platelets; HLA Antigens; Humans; Platelet Transfusion; Thrombocytopenia
PubMed: 34763457
DOI: 10.21037/apm-21-2603 -
Journal of Acute Medicine Sep 2021Optimal management for trauma-induced coagulopathy (TIC) is a clinical conundrum. In conjunction with the transfusion of fresh-frozen plasma (FFP), additional...
BACKGROUND
Optimal management for trauma-induced coagulopathy (TIC) is a clinical conundrum. In conjunction with the transfusion of fresh-frozen plasma (FFP), additional administration of prothrombin complex concentrate (PCC) was proposed to bring about further coagulative benefit. However, investigations evaluating the efficacy as well as corresponding side effects were scarce and inconsistent. The aim of this study was to systematically review current literature and to perform a meta-analysis comparing FFP+PCC with FFP alone.
METHODS
Web search followed by manual interrogation was performed to identify relevant literatures fulfilling the following criteria, subjects as TIC patients taking no baseline anticoagulants, without underlying coagulative disorders, and reported clinical consequences. Those comparing FFP alone with PCC alone were excluded. Comprehensive Meta-analysis software was utilized, and statistical results were delineated with odd ratio (OR), mean difference (MD), and 95% confidence interval (CI). I was calculated to determine heterogeneity. The primary endpoint was set as all-cause mortality, while the secondary endpoint consisted of international normalized ratio (INR) correction, transfusion of blood product, and thrombosis rate.
RESULTS
One hundred and sixty-four articles were included for preliminary evaluation, 3 of which were qualified for meta-analysis. A total of 840 subjects were pooled for assessment. Minimal heterogeneity was present in the comparisons (I < 25%). In the PCC + FFP cohort, reduced mortality rate was observed (OR: 0.631; 95% CI: 0.450-0.884, = 0.007) after pooling. Meanwhile, INR correction time was shorter under PCC + FFP (MD: -608.300 mins, < 0.001), whilst the rate showed no difference ( = 0.230). The PCC + FFP group is less likely to mandate transfusion of packed red blood cells ( < 0.001) and plasma ( < 0.001), but not platelet ( = 0.615). The incidence of deep vein thrombosis was comparable in the two groups ( = 0.460).
CONCLUSIONS
Compared with FFP only, PCC + FFP demonstrated better survival rate, favorable clinical recovery and no elevation of thromboembolism events after TIC.
PubMed: 34595091
DOI: 10.6705/j.jacme.202109_11(3).0001 -
Blood Transfusion = Trasfusione Del... Sep 2022Acquired platelet function disorders (PFD) are rare bleeding diseases that should be suspected in all patients with unexplained mucocutaneous bleedings of recent onset,... (Review)
Review
Acquired platelet function disorders (PFD) are rare bleeding diseases that should be suspected in all patients with unexplained mucocutaneous bleedings of recent onset, with no previous history of haemorrhages, and with normal coagulation test and platelet count. Drug-induced platelet function bleeding disorders are the most frequent PFDs and can easily be identified on the basis of recent administration of platelet-inhibiting drugs. Apart from these, the most challenging acquired PFDs are those caused by autoimmune mechanisms. In fact, demonstration of autoantibodies inhibiting platelet function may be difficult in most non-specialised centres. Among autoimmune PFDs (aPFDs), acquired Glanzmann thrombasthenia (aGT), which is caused by autoantibodies that bind to platelet αIIbβ3 integrin, inhibiting its function, is the most frequent. aGT can be associated with underlying haematological malignancies or autoimmune diseases but can also be idiopathic. More rarely, other immune-mediated PFDs can occur, such as acquired delta storage pool disease (aδSPD). Treatment of aPFDs must rely on the control of acute and chronic bleedings, treatment of the underlying disease in secondary forms, and immunosuppressive treatment for autoantibody reduction or eradication. aPFDs may completely resolve upon treatment of any underlying disease that may be present. In primary aPFDs, and in the majority of secondary forms, treatment relies on immunosuppressive therapies.Here we present a systematic review of previously described immune-mediated aGT and aδSPD cases. Clinical and laboratory characteristics, treatments for the control of bleedings and for the eradication of autoantibodies, and responses to treatments are also discussed. Although no guidelines are available for the management of these very rare conditions, presentation of all cases reported so far can help clinicians in the diagnosis and treatment of these life-threatening diseases.
Topics: Albinism; Autoantibodies; Autoimmune Diseases; Hemorrhagic Disorders; Hermanski-Pudlak Syndrome; Humans; Platelet Glycoprotein GPIIb-IIIa Complex; Thrombasthenia
PubMed: 34369869
DOI: 10.2450/2021.0119-21