Inhibitor development upon switching from plasma-derived to recombinant factor VIII in previously untreated patients with severe hemophilia A: the PUP-SWITCH study.

Background: The SIPPET randomized clinical trial showed that in previously untreated patients (PUPs) with severe hemophilia A, treatment with plasma-derived factor (F)VIII (pdFVIII) within the first 50 exposure days (EDs) was associated with a lower cumulative incidence of inhibitors than with recombinant FVIII (rFVIII). Switching to rFVIII beyond 50 EDs with pdFVIII is a treatment often implemented by many centers. The question is whether or not this switch may induce a risk of inhibitor development.

Naturally occurring anti-P1 with high thermal amplitude complicating ABO blood grouping.

Anti-P1 is immunoglobulin M type and occurs naturally; it is often detected as a weak- and cold-reactive antibody. However, in rare cases, it is reactive at 37°C or shows hemolytic transfusion reactions. The presence of high thermal amplitude of anti-P1 cannot be ignored and requires cross-match compatible red blood cells for transfusion.

Predicting inhibitor development using a random peptide phage-display library approach in the SIPPET cohort.

Inhibitor development is the most severe complication of hemophilia A (HA) care and is associated with increased morbidity and mortality. This study aimed to use a novel immunoglobulin G epitope mapping method to explore the factor VIII (FVIII)-specific epitope profile in the SIPPET cohort population and to develop an epitope mapping-based inhibitor prediction model. The population consisted of 122 previously untreated patients with severe HA who were followed up for 50 days of exposure to FVIII or 3 years, whichever occurred first.

Congenital Skin Rashes in an IVF Baby Progressed to Multisystem Langerhans Cell Histiocytosis with Lung and Bone Involvement: A Case Report and Literature Review.

Langerhans cell histiocytosis is an uncommon proliferative disorder that may influence many organs; so, the clinical presentations vary. Here we describe an 85-day-old female who was born with In vitro fertilization after 10 years of infertility. She referred to us due to severe pulmonary insufficiency and congenital progressive maculopapular rash with desquamation.

Colorimetric detection of fluoride and mercury (II) ions using isatin Schiff base skeleton bearing pyridine-2-carboxamidine moiety: Experimental and theoretical studies.

An isatin-Schiff base ligand (ISS) bearing a pyridine-2-carboxamidine moiety was synthesized through a facile and convenient method for the highly selective colorimetric detection of Hg and F ions. The sensing ability of the synthesized ISS sensor toward Hg and F was established using colorimetric and UV-visible techniques. The developed sensor showed excellent selectivity in the presence of other competing ions for Hg and F, with a color change from yellow to red.

Assessment of Standard Operating Procedures (SOPs) Preparing Hygienic Condition in the Blood Donation Centers during the Outbreak of COVID-19.

The coronavirus disease 2019 (COVID-19) outbreak has led to an alteration in hygienic conditions. In this situation, improving standard operating procedures (SOPs) in blood donation centers is critical. The purpose of this study was the assessment of SOPs in the blood donation centers during the outbreak of COVID-19 by regular blood donors as external audits.

von Willebrand factor neutralizing and non-neutralizing alloantibodies in 213 subjects with type 3 von Willebrand disease enrolled in 3WINTERS-IPS.

Background: Type 3 von Willebrand disease (VWD) is the most severe form of this disease owing to the almost complete deficiency of von Willebrand factor (VWF). Replacement therapy with plasma-derived products containing VWF or recombinant VWF rarely cause the development of alloantibodies against VWF that may be accompanied by anaphylactic reactions.

Objective: The objective of this study was to assess the prevalence of anti-VWF alloantibodies in subjects with type 3 VWD enrolled in the 3WINTERS-IPS.

Allogenic Hematopoietic Stem Cell Transplant in Iranian Patients With Congenital Sideroblastic Anemia: A Single-Center Experience.

Congenital sideroblastic anemia is characterized by anemia and intramitochondrial iron accumulation in erythroid precursors that form ring sideroblasts. The most common recessive forms are caused by sequence variations in the ALAS2 and SLC25A38 genes. In patients with transfusion-dependent and pyridoxine- resistant severe congenital sideroblastic anemia, hematopoietic stem celltransplantis the only curative option.

Source Plasma Donation: The Experience of the Iranian Blood Transfusion Organization.

A declining need for red blood cells coupled with strengthening demand for plasma-derived medicines has led to a strong focus on moving whole blood donors to plasmapheresis. The purpose of this study was to evaluate the four-year policies of the Iranian Blood Transfusion Organization (IBTO) in terms of plasmapheresis recruitment of first-time donors and its effect on plasmapheresis outcome.   Plasmapheresis data related to 16 centers from 2016 to 2019 was obtained from IBTO software.

Patient and Physician Perspectives in the Management of Immune Thrombocytopenia in Iran: Responses from the ITP World Impact Survey (I-WISh).

Data describing physicians' and patients' perspectives towards immune thrombocytopenia (ITP) management and impact of disease in Iran are limited. This ITP World Impact Survey was conducted between October 2019 and October 2020. Of the 114 patients included in the survey, 17 were aged ≤18 years.

The impact of COVID-19 on blood safety and availability in the Islamic Republic of Iran.

Background: The COVID-19 pandemic has affected blood inventory and donation worldwide. The Islamic Republic of Iran was among the first countries to report the COVID-19 pandemic and it faced a significant blood shortage during the first weeks of the pandemic.

Aims: We aimed to evaluate the impact of COVID-19 pandemic on the number, type, and safety of blood donations, comparing the periods before and during the pandemic.

Investigation of the potential of leukoreduction filters in the creation of anti-inflammatory compound.

Background: Some viruses such as SARS, SARS-CoV-2, and MERS cause an imbalance in immune responses and leads to an acute inflammatory reaction named cytokine storm. In this situation, an anti-inflammatory component can modulate the immune system and decrease mortality. The aim of this study was investigate the potential of leukoreduction filters (LRFs) in creating an anti-inflammatory compound.

Estimate of anti-SARS-CoV-2 spike IgG antibodies prevalence among Iranian population based on blood donations: A serial cross-sectional study during the third wave of the pandemic.

Objectives: Iran is one of the countries that have been confronted with the SARS-CoV-2 epidemic since February 2020. This study aimed to determine the levels of specific IgG antibodies against SARS-CoV-2 among healthy blood donors to estimate the burden of the epidemic.

Material And Methods: A serial cross-sectional study was conducted on blood donors who referred to 31 main blood donation centers in different provinces during the third weeks of September, October, and November 2020.

Re-positive PCR of SARS-CoV-2 in health care persons during COVID-19 pandemic.

Reinfection rate with SARS-CoV-2 and degree of protection by the induced antibody after the first episode of the infection is not well known, so it makes a big dilemma for health care personnel (HCP) who work in the front line of combating SARS-CoV-2. In this study, we investigated the frequency of SARS-CoV-2 redetection among HCP after the initial onset of the infection in a children's hospital during one year. Out of 131 seropositive HCP, 13.

Von Willebrand factor propeptide and pathophysiological mechanisms in European and Iranian patients with type 3 von Willebrand disease enrolled in the 3WINTERS-IPS study.

Background: Type 3 von Willebrand disease (VWD) is a severe bleeding disorder caused by the virtually complete absence of von Willebrand factor (VWF). Pathophysiological mechanisms of VWD like defective synthesis, secretion, and clearance of VWF have previously been evaluated using ratios of VWF propeptide (VWFpp) over VWF antigen (VWF:Ag) and factor (F)VIII coagulant activity (FVIII:C) over VWF:Ag.

Objective: To investigate whether the VWFpp/VWF:Ag and FVIII:C/VWF:Ag ratios may also be applied to understand the pathophysiological mechanism underlying type 3 VWD and whether VWFpp is associated with bleeding severity.

Clinical and genetic characteristics of hemoglobin H disease in Iran.

Hemoglobin H (Hb H) disease is a subtype of α-thalassemia caused by deletional and/or non-deletional mutations in three alpha-globin genes in which the various genotypes determine the disease severity. This study was aimed to investigate the frequency of alpha gene mutations and genotypes and their correlation with hematological and clinical characteristics in Iran. Among 202 patients diagnosed with Hb H disease through a national study in Iran according to standard methods, we had access to the hematologic and clinical findings and genetic data of 101 patients in whom genetic study was performed.

Effects and treatment applications of polymeric nanoparticles on improving platelets' storage time: a review of the literature from 2010 to 2020.

Maintaining the quality of platelet products and increasing their storage time are priorities for treatment applications. The formation of platelet storage lesions that limit the storage period and preservation temperature, which can prepare a decent environment for bacterial growth, are the most important challenges that researchers are dealing with in platelet preservation. Nanotechnology is an emerging field of science that has introduced novel solutions to resolve these problems.

Early high-titer convalescent plasma therapy in patients with moderate and severe COVID-19.

Background And Objectives: The use of COVID-19 convalescent plasma (CCP) has been approved by the FDA. We assessed the outcome of patients with moderate and severe COVID-19 following convalescent plasma therapy and the association with variables such as antibody titer in CCP units and transfusion time.

Materials And Methods: In this prospective cohort study, 3097 patients with moderate and severe COVID-19 (according to WHO Progression Scale) had heterogeneous demographic and clinical characteristics received plasma with an unknown titer at the transfusion time.

An efficient and effective ambulatory service model for severe hemophilia-A patients; an introduction to a novel home care model.

Introduction: Hemophilia A (HA) is an inherited deficiency in blood coagulation factors. Starting the treatment based merely on patients' hemorrhage feelings results in more than 63.6% mistakes in joint bleeding diagnosis.

The demographic and serological characteristics of COVID-19 convalescent plasma donors: Identification of basic criteria for optimal donor selection.

Background And Objectives: Convalescent plasma has attracted significant attention as a therapeutic option against infectious agents for more than a century. In March 2020, the use of Convalescent COVID-19 plasma (CCP) as a new research drug for COVID-19 treatment was approved by the FDA. The development of SARS-CoV-2 IgG antibodies following infection or vaccination is likely to be essential to provide adequate immunity for the population to halt the COVID19 pandemic.

A comparison between on-demand usage of rFVIIa vs prophylaxis use of emicizumab in high titer inhibitory hemophilia A patients in Iran: A cost-utility analysis.

Background: Hemophilia A (HA) is an inherited X-linked bleeding disease with costly treatment, especially for high titer inhibitory patients. Emicizumab, a new humanized bispecific antibody, has been approved for use to prevent or reduce the frequency of bleeding episodes in HA patients with inhibitors. This study evaluated the cost-utility of emicizumab prophylaxis (EP) in comparison with recombinant factor VII activated on-demand treatment in HA patients with inhibitors.

Positive anti-SARS-CoV-2 rapid serological test results among asymptomatic blood donors.

Objectives: The world is fighting with a COVID-19 pandemic, some of the uncertainties have been revealed. To figure out an estimation of asymptomatic patients and seropositive SARS-COV-2 blood donors in Iran, a national survey was conducted to find the prevalence of asymptomatic blood donors with positive SARS-COV-2 IgM/IgG test results at the end of May 2020.

Material And Methods: From all 31 provinces, 1339 blood donors were included.

Genotypes of European and Iranian patients with type 3 von Willebrand disease enrolled in 3WINTERS-IPS.

Type 3 von Willebrand disease (VWD3) is a rare and severe bleeding disorder characterized by often undetectable von Willebrand factor (VWF) plasma levels, a recessive inheritance pattern, and heterogeneous genotype. The objective of this study was to identify the VWF defects in 265 European and Iranian patients with VWD3 enrolled in 3WINTERS-IPS (Type 3 Von Willebrand International Registries Inhibitor Prospective Study). All analyses were performed in centralized laboratories.

Performance of a clinical risk prediction model for inhibitor formation in severe haemophilia A.

Background: There is a need to identify patients with haemophilia who have a very low or high risk of developing inhibitors. These patients could be candidates for personalized treatment strategies.

Aims: The aim of this study was to externally validate a previously published prediction model for inhibitor development and to develop a new prediction model that incorporates novel predictors.