The Australian Aplastic Anaemia and other Bone Marrow Failure Syndromes Registry.

The bone marrow failure syndromes (BMFS) are a diverse group of acquired and inherited diseases which may manifest in cytopenias, haematological malignancy and/or syndromic multisystem disease. Patients with BMFS frequently experience poor outcomes, and improved treatment strategies are needed. Collation of clinical characteristics and patient outcomes in a national disease-specific registry represents a powerful tool to identify areas of need and support clinical and research collaboration.

Sickle cell disease in Australia: a snapshot from the Australian Haemoglobinopathy Registry.

Background: Sickle cell disease (SCD) is the most common monogenic disorder worldwide. In deoxygenated conditions, the altered beta chain (haemoglobin S [HbS]) polymerises and distorts the erythrocyte, resulting in pain crises, vasculopathy and end-organ damage. Clinical complications of SCD cause substantial morbidity, and therapy demands expertise and resources.

Role of bone marrow biopsy for fever of unknown origin in the contemporary Australian context.

Background: Bone marrow biopsy (BMB) is an accepted investigation in fever of unknown origin (FUO) to uncover haematological malignancies, such as lymphoma, and sometimes infections. With the advance in imaging modalities, such as 18-fluoro-2-deoxyglucose positron emission tomography (FDG-PET) to identify the focus of lymphoma, BMB may not contribute to the diagnosis when there are no other clinical features to suggest an underlying haematological disease.

Aim: To investigate the utility of BMB in determining the cause of FUO, when there are no other indications for BMB.

Revisiting acquired aplastic anaemia: current concepts in diagnosis and management.

Acquired aplastic anaemia is a rare, serious, immunologically mediated bone marrow failure syndrome, characterised by marrow hypoplasia of varying severity and significant pancytopenia. Careful attention and investigation, including molecular testing, is required to confirm the diagnosis and exclude other mimicking conditions, such as inherited bone marrow failure syndromes. In a proportion of patients, the disease evolves to myelodysplasia or acute myeloid leukaemia and in some there is an association with paroxysmal nocturnal haemoglobinuria.

Combination Treatment of p53-Null HL-60 cells with Histone Deacetylase Inhibitors and Chlorambucil Augments Apoptosis and Increases BCL6 and p21 Gene Expression.

Background: Treatment of hematological malignancies with conventional DNA-damaging drugs, such as chlorambucil (CLB), commonly results in p53-dependent chemo-resistance. Chromatin modifying agents, such as histone deacetylase inhibitors (HDACIs), sodium butyrate (NaBu) and trichostatin A (TSA), may reverse chemo-resistance by modulating the activity of chromatin remodeling enzymes and/or genes that control cell proliferation, differentiation and survival.

Objective: This study examined the potential use of HDACIs and CLB combination therapies in an in vitro chemo-resistant leukemia model.

Platelet transfusion is not associated with increased mortality or morbidity in patients undergoing cardiac surgery.

Background: Transfusion of platelets is common in cardiac surgery, and while there are guidelines for their use, there are concerns about potential risks. We aimed to assess the impact of platelet transfusion on mortality, thrombosis, and infection in this patient group.

Study Design And Methods: A retrospective cohort study of all patients at St Vincent's Hospital Melbourne who underwent a first cardiac surgery procedure from June 2001 to June 2014 was conducted.

Major haemorrhage fatalities in the Australian national coronial database.

Objective: The aim of the study is to describe the epidemiology of major bleeding fatalities.

Methods: A case series analysis of Australia's National Coronial Information System was conducted. Keywords were used to search for closed cases of major haemorrhage in the state of Victoria for the period 1 January 2009 to 31 December 2011.

Introduction of universal prestorage leukodepletion of blood components, and outcomes in transfused cardiac surgery patients.

Objective: To assess whether introduction of universal leukodepletion (ULD) of red blood cells (RBCs) for transfusion was associated with improvements in cardiac surgery patient outcomes.

Methods: Retrospective study (2005-2010) conducted at 6 institutions. Associations between leukodepletion and outcomes of mortality, infection, and acute kidney injury (AKI) were modeled by logistic regression, and intensive care unit length of stay (LOS) in survivors was explored using linear regression.

Predicting blood loss and transfusion requirement in patients undergoing surgery for musculoskeletal tumors.

Background: Few studies have systematically identified factors associated with blood loss in musculoskeletal tumor surgery. We aimed to identify risk factors for requiring large-volume transfusion in musculoskeletal tumor surgery and created an interactive model to predict red blood cell transfusion requirements based on patient characteristics. These data will facilitate planning in hospital blood banks and aid identification of specific groups for future interventions targeted at reducing blood utilization.

Transfusion practice varies widely in cardiac surgery: Results from a national registry.

Objectives: Evidence is accumulating of adverse outcomes associated with transfusion of blood components. If there are differences in perioperative transfusion rates in cardiac surgery, and what hospital factors may contribute, requires further investigation.

Methods: Analysis of 42,743 adult patients who underwent 43,482 procedures from 2005 to 2011 at 25 Australian hospitals, according to the Australian and New Zealand Society of Cardiac and Thoracic Surgeons Cardiac Surgery Database.

Clinical transfusion practice update: haemovigilance, complications, patient blood management and national standards.

Blood transfusion is not without risk. Although the risks of HIV and hepatitis transmission have diminished, haemovigilance programs highlight that other significant transfusion hazards remain. Sepsis from bacterial contamination is the most common residual infectious hazard in developed countries, and events due to clerical error are problematic.

Hospital blood bank information systems accurately reflect patient transfusion: results of a validation study.

Background: Hospital transfusion laboratories collect information regarding blood transfusion and some registries gather clinical outcomes data without transfusion information, providing an opportunity to integrate these two sources to explore effects of transfusion on clinical outcomes. However, the use of laboratory information system (LIS) data for this purpose has not been validated previously.

Study Design And Methods: Validation of LIS data against individual patient records was undertaken at two major centers.

Palliative care and the hemato-oncological patient: can we live together? A review of the literature.

Current evidence suggests that patients with hematological malignancies less frequently access palliative care services, and for those who do, this tends to occur later in their illness than their counterparts with solid malignancies. These patients are also more likely to die in hospital following escalating interventions. This approach to care that considers palliative care referral after most treatments are exhausted has implications for the quality of palliative care intervention possible.

Blood transfusion requirements for patients undergoing chemotherapy for acute myeloid leukemia how much is enough?

Although essential in the management of AML, there is little information quantitating transfusion requirements for these patients. We evaluated 111 consecutive adults treated for AML, showing that approximately 150 blood donors are required to adequately cater for a single patient's complete therapy with little variation for age, prognostic group or intensity of treatment.

Appearance of del(11q) in two patients with acute promyelocytic leukaemia treated with all-trans retinoic acid and combination chemotherapy.

Two acute promyelocytic leukaemia patients, treated with all-trans retinoic acid and combination chemotherapy, acquired a deletion of 11q within 12 months of diagnosis. One patient died in relapse, with both t(15;17) and del(11q) cell lines co-existing. Patient 2 remains in remission with del(11q) in 70% metaphases, despite normal marrow morphology.