ANZTCT practice statement: sinusoidal obstruction syndrome/veno-occlusive disease diagnosis and management.

Sinusoidal obstruction syndrome/veno-occlusive disease (SOS/VOD) is a life-threatening complication which can develop after haemopoietic stem cell transplantation (HSCT) and some antibody-drug conjugates. Several SOS/VOD diagnostic and management guidelines exist, with the most recent and refined being the European Society for Blood and Marrow Transplantation adult and paediatric guidelines. Timely diagnosis and effective management (including the availability of therapeutic options) significantly contribute to improved patient outcomes. In Australia and New Zealand, there is variability in clinical practice and access to SOS/VOD therapies. This review aims to summarise the current evidence for SOS/VOD diagnosis, prevention and treatment and to provide recommendations for SOS/VOD in the context of contemporary Australasian HSCT clinical practice.

Clinicopathological characteristics and long-term outcomes of plasmablastic lymphoma in British Columbia.

Plasmablastic lymphoma (PBL) is an aggressive and rare subtype of non-Hodgkin lymphoma with no standard-of-care therapy. We reviewed all patients diagnosed with histologically confirmed PBL in British Columbia, Canada between 1997 and 2019. Overall, 42 patients were identified, including 15 (36%) positive for HIV and nine (21%) on chronic immunosuppression. Curative-intent treatment consisting primarily of cyclophosphamide, doxorubicin, vincristine and prednisone was administered to 31 patients, of which 74% achieved response, however 61% relapsed after a median of 7.5 months. At a median follow-up of eight years for the whole cohort, five-year progression-free survival (PFS) and overall survival (OS) were 18% [95% confidence interval (CI): 6%, 30%] and 22% (95% CI: 8%, 36%) with median eight and 15 months respectively. There were no differences in relapse rate (p = 0.962), PFS (p = 0.228) or OS (p = 0.340) according to immune status. For those treated with curative intent, five-year PFS and OS were 24% (95% CI: 8%, 40%) and 31% (95% CI: 13%, 49%) with median 18 and 27 months respectively. In this population-based cohort of PBL patients spanning 20 years, survival outcomes were poor. Ultimately, further research is needed to develop more effective treatment strategies and to improve survival for patients.

Coparenting and children's school readiness: a mediational model.

We examined the long-term direct and indirect links between coparenting (conflict, communication, and shared decision-making) and preschoolers' school readiness (math, literacy, and social skills). The study sample consisted of 5,650 children and their biological mothers and fathers who participated in the Early Childhood Longitudinal Study-Birth Cohort. Using structural equation modeling and controlling for background characteristics, we found that our conceptual model of the pathways from coparenting to child outcomes is structurally the same for cohabiting and married families. Controlling for a host of background characteristics, we found that coparenting conflict and shared decision-making were negatively and positively, respectively, linked to children's academic and social skills and co-parental communication was indirectly linked to academic and social skills through maternal supportiveness. Coparenting conflict was also indirectly linked to children's social skills through maternal depressive symptoms. The overall findings suggest that for both cohabiting and married families, the context of conflicted coparenting may interfere with the development of children's social competencies and academic skills, whereas collaborative coparenting promotes children's school readiness because mothers are more responsive to their children's needs. These findings have implications for programs aimed at promoting positive family processes in cohabiting and married families.

The improvement in overall survival from unrelated donor transplantation in Australia and New Zealand is driven by a reduction in non-relapse mortality: A study from the ABMTRR.

Unrelated donors (UDs) are the commonest source for allogeneic transplantation (alloSCT), with higher non-relapse mortality (NRM) than siblings. We analyzed data from the Australasian Bone Marrow Transplant Recipient Registry from adults receiving a first UD alloSCT during 2001-2015, to determine whether and how NRM has changed. Predictors of outcome were determined using cox regression, accounting for time-interactions and competing risks. A total of 2308 patients met inclusion criteria. Changes over time included increasing age, utilization of peripheral blood cells, reduced intensity conditioning, and T-cell depletion. Three-year OS increased significantly from 44% in 2001-2005 to 58% in 2011-2015 (p < 0.001). This was attributed to a reduction in NRM from 35% to 24% (p < 0.001) with no change in relapse. Factors associated with increased NRM included age, male sex, CMV seropositivity, HLA mismatch, transplant more than 6 months from diagnosis, and T-cell depletion when administered during 2001-2005. Survival following UD SCT has improved by almost 15% over the past decade, driven by improvements in NRM. This has occurred despite increasing recipient age and appears to be due to better donor selection, reduced delays to transplantation, and improved prevention and management of GVHD.

Ethnic differences in acute promyelocytic leukaemia between New Zealand Polynesian and European patients.

Ethnic differences in haematologic malignancies remain poorly elucidated, hence research in this area is important. This was a retrospective study into potential ethnic disparity in the presentation and outcomes of acute promyelocytic leukaemia (APL) between New Zealand (NZ) Polynesian and European patients. Data were analysed for patients treated at Auckland City Hospital (ACH; n = 55) and recorded in the New Zealand Cancer Registry (NZCR; n = 173), both for the period 2000-2017. We found that Polynesian patients treated at ACH presented at a younger age than European (P = 0.005), showed higher blast counts (P = 0.033), and a marginally higher prothrombin ratio (P = 0.02). Treatment with all-trans retinoic acid (ATRA) was started faster in Polynesian patients than European (P = 0.021), suggesting Polynesians were sicker at presentation but were managed accordingly. There were no differences in bleeding events, transfusion requirements and early deaths during the first month of treatment. Long-term survival was also similar. Data extracted from the NZCR confirmed NZ Polynesian patients with APL were younger than European (P < 0.001), but long-term survival was similar (P = 0.920). In summary, this study indicates a discrepancy in the presentation and severity of APL between NZ Polynesian and European patients but treatment initiation was rapid with no difference in outcomes. The distinctive features of APL in NZ Polynesians raise the possibility of a predisposing genetic factor or a different risk factor profile, elucidation of which is important for all patients with APL.

Consensus statement on the treatment of transplant-eligible patients with newly diagnosed multiple myeloma in New Zealand.

Multiple myeloma is the second most common blood cancer in New Zealand with higher incidence in Maori and Pacific Island populations. It remains an incurable disease but the rapidly changing treatment landscape has led to improved outcome. In response to recent changes in funding of anti-myeloma therapy in New Zealand, the New Zealand Myeloma Interest Group has reviewed the latest literature and updated the treatment pathway of transplant-eligible patients with newly diagnosed multiple myeloma.

Treatment outcomes of patients with acute promyelocytic leukaemia between 2000 and 2017, a retrospective, single centre experience.

All-trans retinoic acid (ATRA) and arsenic trioxide (ATO) are effective induction therapy for acute promyelocytic leukaemia (APL). However, early thrombo-haemorrhagic complications and mortality remain high. We aimed to investigate how the timing of ATRA initiation and the inclusion of ATO influence patient outcomes. Clinical records were retrospectively reviewed for all patients treated for APL in a single, tertiary centre during 2000-2017. Among 70 patients with APL, 36 (51.4%) presented with thrombo-haemorrhagic complications, and four (5.8%) died within 30 days. The median time to ATRA initiation was 11.2 (range 0-104) h from the time of admission. Patients requiring more transfusions started on ATRA sooner (P = 0.04). Patients with adverse early events did not start ATRA later (P = 0.99). Nevertheless, patients that required additional tests for diagnosis (PML immunofluorescence or molecular) started on ATRA later (28.5 versus 5.3 h; P < 0.0001), and had more thrombo-haemorrhagic complications (P = 0.04). Long-term survival was actually better in patients who started ATRA later (P = 0.03), which is likely explained by higher proportion of low risk patients in this group. Patients treated with ATO (n = 23) maintained higher fibrinogen levels and required less transfusions during induction (P < 0.05), with no disease-related deaths in this group over a median follow-up time of 37.8 months (interquartile range 44.9 months). In summary, fast ATRA initiation reduces early but not late adverse events in APL patients, and the inclusion of ATO helps further improve both early and late outcomes in APL.

Early treatment of acute promyelocytic leukaemia is accurately guided by the PML protein localisation pattern: real-life experience from a tertiary New Zealand centre.

Current guidelines recommend that a rapid test be used to assist diagnosis of acute promyelocytic leukaemia (APL), but the choice of an assay is discretionary. PML immunofluorescence (PML IF) identifies the microparticulate pattern of the PML protein localisation, highly specific for APL. The aim of this study was to evaluate clinical utility of PML IF in a real-life setting based on a retrospective records review for all patients who had PML IF performed in our centre between 2000 and 2017. Final analysis included 151 patients, 70 of whom had APL. PML IF was reported on average 3 days faster than cytogenetics. Compared with genetic results, PML IF showed sensitivity of 96% and specificity of 100%. PML IF accurately predicted APL in four APL cases with cryptic karyotype/FISH and excluded APL in 98% cases tested based on the suspicious immunophenotype alone, 21/28 of whom had mutated NPM1. Results of PML IF influenced decision to start ATRA in 25 (36%) APL patients and led to its termination in six non-APL patients. In conclusion, PML IF is a fast and reliable test that facilitates accurate treatment decisions when APL is suspected. This performance of PML IF remains hard to match in a real-life setting.