Reduced Intensity transplantation vs chemotherapy in CR1. A prospective, pseudorandomized study in 50-70 year old AML patients.

The aim of this prospective, international multicenter, pseudorandomized study comparing RICT HCT to standard-of-care chemotherapy in intermediate- or high-risk AML patients 50-70 years using the donor versus no-donor concept. Part 1 included only patients with potential family donors (RD) at the date of HLA-typing of the first potential sibling or CR-date, if later. Part 2 allowed the inclusion of patients without a possible sibling donor using the start of an unrelated donor (URD) search as inclusion date.

Māori and Pacific Peoples With Multiple Myeloma in New Zealand are Younger and Have Inferior Survival Compared to Other Ethnicities: A Study From the Australian and New Zealand Myeloma and Related Diseases Registry (MRDR).

Background: Māori and Pacific peoples (MPP) in New Zealand (NZ) have poorer health outcomes than other ethnicities. However, this has not been clinically investigated in multiple myeloma (MM). Using data from the Australian and NZ Myeloma and Related Diseases Registry for all participating centers in NZ, we compared MPP demographics, clinical characteristics, diagnostics, treatment, and outcomes to non-MPP.

Defining the Optimal Total Number of Chemotherapy Courses in Younger Patients With Acute Myeloid Leukemia: A Comparison of Three Versus Four Courses.

Purpose: The optimum number of treatment courses for younger patients with acute myeloid leukemia (AML) is uncertain. The United Kingdom National Cancer Research Institute AML17 trial randomly assigned patients who were not high risk to a total of three versus four courses.

Patients And Methods: Patients received two induction courses based on daunorubicin and cytarabine (Ara-C), usually with gemtuzumab ozogamicin.

Molecular MRD status and outcome after transplantation in NPM1-mutated AML.

Relapse remains the most common cause of treatment failure for patients with acute myeloid leukemia (AML) who undergo allogeneic stem cell transplantation (alloSCT), and carries a grave prognosis. Multiple studies have identified the presence of measurable residual disease (MRD) assessed by flow cytometry before alloSCT as a strong predictor of relapse, but it is not clear how these findings apply to patients who test positive in molecular MRD assays, which have far greater sensitivity. We analyzed pretransplant blood and bone marrow samples by reverse-transcription polymerase chain reaction in 107 patients with NPM1-mutant AML enrolled in the UK National Cancer Research Institute AML17 study.

An Age Stratified Analysis of the Access to Care Continuum Across Three Tumor Groups: Are There Delays for AYA?

Delays in diagnosis and treatment are regularly discussed as potential poor prognostic factors for adolescent and young adult (AYA) cancer patients. We aimed to determine whether AYA cancer patients (15-24 years of age) in the South Island of New Zealand had longer times to diagnosis and treatment than pediatric (<15 years) and adult patients (>24 years) with the same diagnosis. A retrospective review of medical records was undertaken for 201 recently diagnosed sarcoma, Hodgkin lymphoma (HL), and non-Hodgkin lymphoma (NHL) patients in the South Island.

The burden of cancer in 25-29 year olds in New Zealand: a case for a wider adolescent and young adult age range?

Aims: New Zealand currently defines the adolescent and young adult (AYA) group for cancer services as young people 12-24 years of age, while other countries favour a designation of 15-29 years. This study was undertaken to compare cancer incidence and survival among 25-29 year olds to New Zealand's younger AYA population and to assess survival for our 15-29 year population against international benchmarks.

Methods: Diagnostic and demographic information for cancer registrations between 2000 and 2009 for 25-29 year olds was obtained from the New Zealand Cancer Registry.

Small Numbers, Big Challenges: Adolescent and Young Adult Cancer Incidence and Survival in New Zealand.

Purpose: This study was undertaken to determine cancer survival and describe the unique spectrum of cancers diagnosed among New Zealand's adolescents and young adult (AYA) population.

Methods: Registrations for 1606 15-24 year olds diagnosed with a new primary malignant tumor between 2000 and 2009 were obtained from the New Zealand Cancer Registry and classified according to AYA diagnostic group and subgroup, age, sex, and prioritized ethnicity. Age-standardized incidence rates (IRs) per million person years and 5-year relative survival ratios were calculated.

Patterns and sources of alcohol consumption preceding alcohol-affected attendances to a New Zealand hospital emergency department.

Aim: To perform a descriptive study of the drinking behaviour (amounts, types, sources of alcohol consumed) preceding alcohol-affected presentations to Christchurch Hospital Emergency Department (ED).

Methods: Over 336 hours in the ED, patients with recent alcohol consumption or alcohol-related attendances were identified, classified as alcohol-affected or alcohol- unaffected, and invited to consent to answering questions on types, amounts and sources of alcohol consumed in the drinking session preceding or implicated in their ED attendance. Demographic information and level of intoxication were also recorded.

The impact of alcohol-related presentations on a New Zealand hospital emergency department.

Aim: To determine the impact of alcohol-related presentations on the Christchurch Hospital Emergency Department (ED).

Methods: Over 42 8-hour shifts (2 weeks) between 15 November 2013 and 9 December 2013, patients attending the ED with recent alcohol consumption were classified as screen-positive (consumed alcohol in the 4 hours prior to presentation) or not. A subset of screen-positive patients was classified as impact-positive (alcohol consumption clearly contributed to the reason for presenting).

Burnout in hospital-based medical consultants in the New Zealand public health system.

Aim: To assess the prevalence and severity of burnout in hospital-based medical consultants, and investigate associated demographic and professional characteristics.

Method: Utilising standardised measures of burnout (Maslach Burnout Inventory) and job satisfaction (Job Satisfaction Scale) this cross-sectional study recruited 267 consultants working in a large tertiary hospital in Christchurch, New Zealand.

Results: Seventy-one percent of all eligible participants were recruited.

Written advice can provide a safe and acceptable alternative to new patient assessment for selected referrals to haematologists.

Objective: To measure the safety and acceptability of providing written advice (WA) for selected patients referred to a haematology service, as an alternative to inpatient or outpatient assessment.

Design, Setting And Participants: Review of the initial management and subsequent course of patients newly referred to a tertiary referral hospital in Christchurch, New Zealand, between 16 October 2003 and 8 June 2006. Structured questionnaires were sent to all referring doctors and patients recently managed with WA.

The outcome of clinical parameters in adults with severe Type I Gaucher disease using very low dose enzyme replacement therapy.

Enzyme replacement therapy is now well established as the treatment of choice in Type I Gaucher disease. Historically higher dosage regimens have been used in preference to lower doses despite the little clinical evidence in the way of large controlled clinical trials to support this. Moreover, the extraordinary cost of therapy means that not all eligible patients are able to be treated at the higher dose.

Improved leukemia-free survival after postconsolidation immunotherapy with histamine dihydrochloride and interleukin-2 in acute myeloid leukemia: results of a randomized phase 3 trial.

The primary objective of this phase 3 study was to determine whether postconsolidation immunotherapy with interleukin-2 (IL-2) and histamine dihydrochloride (HDC) improved the leukemia-free survival (LFS) of adult patients with acute myeloid leukemia (AML) in complete remission (CR). Three hundred twenty patients with AML (median age, 57 years; range, 18-84 years) were stratified by CR1 or subsequent CR (CR > 1) and randomly assigned to treatment with HDC/IL-2 or no treatment (control). Treatment comprised 10 21-day cycles with IL-2 (16 400 U/kg) plus HDC (0.

Tacrolimus and mycophenolate mofetil after nonmyeloablative matched-sibling donor allogeneic stem-cell transplantations conditioned with fludarabine and low-dose total body irradiation.

We evaluated tacrolimus/mycophenolate mofetil (MMF) for graft-versus-host disease (GVHD) prophylaxis after a nonmyeloablative stem cell transplantation (NST) from a matched sibling donor (MSD). Thirty-two patients (median age, 57 years) with advanced hematologic malignancies, who were poor candidates for a conventional myeloablative transplantation, received fludarabine (30 mg/m(2), day -4 to day -2), total-body irradiation (TBI) (200 cGy, day 0), infusion of donor peripheral blood progenitor cells (day 0), oral tacrolimus 0.06 mg/kg twice daily (from day 3), and oral MMF at 15 mg/kg twice daily (days 0-+27).

EOG as a monitor of desferrioxamine retinal toxicity.

Iron overload caused by blood transfusion-dependent anaemia usually results in lethal cardiac toxicity unless treated by iron-chelation therapy. Chelation therapy with desferrioxamine (DFO) is well established and widely used to remove excess iron. Unfortunately, visual disorders have been recorded after DFO infusion.

Constitutional t(5;7)(q11;p15) rearranged to acquire monosomy 7q and trisomy 1q in a patient with myelodysplastic syndrome transforming to acute myelocytic leukemia.

We report the case of a 61-year-old woman who presented with a myelodysplastic syndrome (MDS) and a t(5;7)(q11.2;p15) in her bone marrow cells. Subsequent analysis of phytohemagglutinin-stimulated peripheral blood lymphocytes and cultured skin fibroblasts showed that the translocation was constitutional.

Familial hypofibrinogenaemia associated with heterozygous substitution of a conserved arginine residue; Bbeta255 Arg-->His (Fibrinogen Merivale).

Sequencing of all three fibrinogen genes from an individual with hypofibrinogenaemia led to the identification of two new point mutations in the Bbeta gene. Family studies showed the mutations Bbeta255 Arg-->His (Fibrinogen Merivale) and Bbeta148 Lys-->Asn (Fibrinogen Merivale II) were on different alleles and that only the Bbeta255 Arg-->His mutation segregated with hypofibrinogenaemia. Three simple heterozygotes for this mutation had mean fibrinogen concentrations of 1.

A novel inherited mutation of the transcription factor RUNX1 causes thrombocytopenia and may predispose to acute myeloid leukaemia.

The RUNX1 (AML1, CBFA2) gene is a member of the runt transcription factor family, responsible for DNA binding and heterodimerization of other non-DNA binding transcription factors. RUNX1 plays an important part in regulating haematopoiesis and it is frequently disrupted by illegitimate somatic recombination in both acute myeloid and lymphoblastic leukaemia. Germline mutations of RUNX1 have also recently been described and are dominantly associated with inherited leukaemic conditions.