Recognition, Investigation and Management of Acute Transfusion Reactions: Consensus guidelines for Oman.

The recognition and management of transfusion reactions (TRs) are critical to ensure patient safety during and after a blood transfusion. Transfusion reactions are classified into acute transfusion reactions (ATRs) or delayed transfusion reactions, and each category includes different subtypes. Different ATRs share common signs and symptoms which can make categorisation difficult at the beginning of the reaction.

Microdeletion of exon 3 in the HBA2 gene associated with mild α-thalassemia trait.

A mother and son presented with mild symptoms of thalassemia trait. Polymerase chain reaction (PCR) amplification of their globin genes revealed a previously unreported 203 bp microdeletion in the HBA2 gene (NG_000006.1:g.

Congenital thrombotic thrombocytopenic purpura (cTTP) with two novel mutations.

Congenital thrombotic thrombocytopenic purpura (cTTP) is a rare disorder of childhood that has clinical and laboratory similarities to other, more common conditions. Prompt recognition is required as delays in therapy are associated with significant morbidity and failure to treat may lead to death. While the principles of treatment have not changed, enormous progress in the genetic and molecular understanding has taken place.

In Utero diagnosis and management of a fetus with homozygous α-Thalassemia in the second trimester: a case report and literature review.

Alpha thalassemia with the absence of 4 α-globin genes leads to fetal hydrops and fetal death from anemia. Historically considered a lethal condition, optimal in utero management of homozygous α-thalassemia is unclear. A fetus of Filipino descent at 26 weeks gestation presented with ultrasound evidence of anemia.

Two new examples of Hb St. Etienne [beta 92(F8)HisGln] in association with venous thrombosis.

Hb St. Etienne [beta92(F8)HisGln] (also known as Hb Istanbul) is a rare unstable beta-globin chain variant that has been described in only three reports involving four patients. We report two individuals in a family of Scottish extraction whose members had been erroneously diagnosed to have hereditary spherocytosis (HS) and have now been shown to be heterozygotes for Hb St.

Combined neutrophil and erythrocyte agglutination in a 7-year-old boy.

Leukoagglutination is a rare in vitro phenomenon, with demonstration of both temperature and/or ethylenediaminetetraacetic acid dependence. We report a case of combined leukocyte and erythrocyte agglutination in a 7-year-old male with Mycoplasma pneumoniae and Epstein-Barr virus coinfection. To our knowledge, this morphologic finding has not previously been described.

Guidelines on the use of intravenous immune globulin for neurologic conditions.

Canada's per capita use of intravenous immune globulin (IVIG) grew by approximately 115% between 1998 and 2006, making Canada one of the world's highest per capita users of IVIG. It is believed that most of this growth is attributable to off-label usage. To help ensure IVIG use is in keeping with an evidence-based approach to the practice of medicine, the National Advisory Committee on Blood and Blood Products (NAC) and Canadian Blood Services convened a panel of national experts to develop an evidence-based practice guideline on the use of IVIG for neurologic conditions.

Recognition and management of methemoglobinemia and hemolysis in a G6PD-deficient patient on experimental anticancer drug Triapine.

We report occurrence of severe methemoglobinemia in a patient on novel experimental anti-cancer drug, Triapine. Treatment with methylene blue led to massive hemolysis due to concomitant G6PD deficiency. We recommend that G6PD screening be done in high-risk populations prior to commencement of Triapine therapy.

Severe FVII deficiency caused by a new point mutation combined with a previously undetected gene deletion.

A 3-week-old Caucasian female presented with severe unprovoked parenchymal cerebral haemorrhage. Her plasma factor VII (FVII) activity was <0.01 units/ml.

Utility of anti-xa monitoring in children receiving enoxaparin for therapeutic anticoagulation.

Although enoxaparin is used to treat thromboembolism in children, current treatment guidelines are largely extrapolated from adults. The objectives of this study were to determine: i) correlation between enoxaparin dose and anti-factor Xa (anti-Xa) level, ii) intra-patient variability, and iii) whether dose or anti-Xa level is a predictor of outcomes. A retrospective chart review was conducted on all hospitalized patients receiving enoxaparin in a tertiary care pediatric institution.

Hyperuricemia and reticulocytopenia in association with autoimmune hemolytic anemia in two children.

Hyperuricemia developed in 2 children with autoimmune hemolytic anemia with reticulocytopenia at a time of hemolytic crisis. One likely cause of hyperuricemia is the destruction of nucleated RBC precursors by autoantibodies. It is advised that patients with autoimmune hemolytic anemia with reticulocytopenia be examined for hyperuricemia.

An assessment of published pediatric dosage guidelines for enoxaparin: a retrospective review.

Objective: To evaluate the ability of published dosage guidelines for enoxaparin to achieve therapeutic anticoagulation and to determine whether the routine monitoring of anti-Xa levels is still necessary at a tertiary care pediatric institution.

Methods: Consecutive charts and laboratory records were reviewed for all patients receiving treatment doses of enoxaparin for thrombosis in the authors institution over a 4-year period (1998-2002).

Results: Sixty-six percent (25/38) of the anti-Xa levels were within the recommended therapeutic range (0.

Element of caution: a case of reversible cytopenias associated with excessive zinc supplementation.

Zinc is a common supplement and is widely available as a standard component of many over-the-counter products. A number of reports have identified an association between excessive zinc intake and severe cytopenia. We report a case of zinc-induced copper deficiency in a young adult to illustrate this under-recognized cause of anemia and neutropenia.

Permanent and panerythroid correction of murine beta thalassemia by multiple lentiviral integration in hematopoietic stem cells.

Achieving long-term pancellular expression of a transferred gene at therapeutic level in a given hematopoietic lineage remains an important goal of gene therapy. Advances have recently been made in the genetic correction of the hemoglobinopathies by means of lentiviral vectors and large locus control region (LCR) derivatives. However, panerythroid beta globin gene expression has not yet been achieved in beta thalassemic mice because of incomplete transduction of the hematopoietic stem cell compartment and position effect variegation of proviruses integrated at a single copy per genome.