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Follicular lymphoma (FL) is the most common indolent non-Hodgkin lymphoma subtype, accounting for 15-20% of all lymphoma diagnoses. Although typically slow-growing and responsive to frontline therapies, advanced-stage FL remains incurable with current treatments and typically follows a chronic relapsing/remitting course with increasingly shorter responses to subsequent lines of therapy. Outcomes are highly variable; some patients experience prolonged first remissions that may approximate a 'functional cure'. By contrast, a significant minority of patients experience disease progression shortly after frontline treatment resulting in high rates of lymphoma-related mortality. Reflecting on the heterogeneous natural history of FL, clinical practice varies widely, particularly in controversial areas, including appropriate disease staging, selection of management strategies and duration of clinical follow-up. This position statement presents an evidence-based synthesis of the literature for application in Australasian practice., (© 2024 Royal Australasian College of Physicians.)

Despite widespread vaccination rates, we are living with high transmission rates of SARS-CoV-2. Although overall hospitalisation rates are falling, the risk of serious infection remains high for patients who are immunocompromised because of haematological malignancies. In light of the ongoing pandemic and the development of multiple agents for treatment, representatives from the Haematology Society of Australia and New Zealand and infectious diseases specialists have collaborated on this consensus position statement regarding COVID-19 management in patients with haematological disorders. It is our recommendation that both patients with haematological malignancies and treating specialists be educated regarding the preventive and treatment options available and that patients continue to receive adequate vaccinations, keeping in mind the suboptimal vaccine responses that occur in haematology patients, in particular, those with B-cell malignancies and on B-cell-targeting or depleting therapy. Patients with haematological malignancies should receive treatment for COVID-19 in accordance with the severity of their symptoms, but even mild infections should prompt early treatment with antiviral agents. The issue of de-isolation following COVID-19 infection and optimal time to treatment for haematological malignancies is discussed but remains an area with evolving data. This position statement is to be used in conjunction with advice from infectious disease, respiratory and intensive care specialists, and current guidelines from the National COVID-19 Clinical Evidence Taskforce and the New Zealand Ministry of Health and Cancer Agency Te Aho o Te Kahu COVID-19 Guidelines., (© 2023 The Authors. Internal Medicine Journal published by John Wiley & Sons Australia, Ltd on behalf of Royal Australasian College of Physicians.)

Waldenström macroglobulinaemia (WM) is an indolent B-cell malignancy characterised by the presence of IgM paraprotein, bone marrow infiltration by clonal small B lymphocytes with plasmacytic differentiation and the MYD88 L265P mutation in >90% of cases. Traditionally, WM has been treated with chemoimmunotherapy. Recent trials have demonstrated the efficacy and safety of Bruton tyrosine kinase inhibitors in WM, both as monotherapy and in combination with other drugs. There is emerging evidence on the use of other agents including B-cell lymphoma 2 inhibitors and on the treatment of rare presentations of WM. In this update, the Medical and Scientific Advisory Group of Myeloma Australia reviews the available evidence on the treatment of WM since the last publication in 2017 and provides specific recommendations to assist Australian clinicians in the management of this disease., (© 2022 The Authors. Internal Medicine Journal published by John Wiley & Sons Australia, Ltd on behalf of Royal Australasian College of Physicians.)

Patients with post-haemopoietic stem cell transplant or chimeric antigen receptor T -cell (CAR-T) therapy face a significant risk of morbidity and mortality from coronavirus disease 2019 because of their immunosuppressed state. As case numbers in Australia and New Zealand continue to rise, guidance on management in this high-risk population is needed. Whilst we have learned much from international colleagues who faced high infection rates early in the pandemic, guidance relevant to local health system structures, medication availability and emerging therapies is essential to equip physicians to manage our patients optimally., (© 2022 The Authors. Internal Medicine Journal published by John Wiley & Sons Australia, Ltd on behalf of Royal Australasian College of Physicians.)

The management of Hodgkin lymphoma (HL) has undergone significant changes in recent years. Due to the predilection of HL to affect younger patients, balancing cure and treatment-related morbidity is a constant source of concern for physicians and patients alike. Positron emission tomography adapted therapy has been developed for both early and advanced stage HL to try and improve the outcome of treatment, while minimising toxicities. The aim of this review is to digest the plethora of studies recently conducted and provide some clear, evidence-based practice statements to simplify the management of HL., (© 2021 Royal Australasian College of Physicians.)

Australia and New Zealand have achieved excellent community control of COVID-19 infection. In light of the imminent COVID-19 vaccination roll out in both countries, representatives from the Haematology Society of Australia and New Zealand and infectious diseases specialists have collaborated on this consensus position statement regarding COVID-19 vaccination in patients with haematological disorders. It is our recommendation that patients with haematological malignancies, and some benign haematological disorders, should have expedited access to high-efficacy COVID-19 vaccines, given that these patients are at high risk of morbidity and mortality from COVID-19 infection. Vaccination should not replace other public health measures in these patients, given that the effectiveness of COVID-19 vaccination, specifically in patients with haematological malignancies, is not known. Given the limited available data, prospective collection of safety and efficacy data of COVID-19 vaccination in this patient group is a priority., (© 2021 Royal Australasian College of Physicians.)

The COVID-19 pandemic poses a unique challenge to the care of patients with haematological malignancies. Viral pneumonia is known to cause disproportionately severe disease in patients with cancer, and patients with lymphoma, myeloma and chronic lymphocytic leukaemia are likely to be at particular risk of severe disease related to COVID-19. This statement has been developed by consensus among authors from Australia and New Zealand. We aim to provide supportive guidance to clinicians making individual patient decisions during the COVID-19 pandemic, in particular during periods that access to healthcare resources may be limited. General recommendations include those to minimise patient exposure to COVID-19, including the use of telehealth, avoidance of non-essential visits and minimisation of time spent by patients in infusion suites and other clinical areas. This statement also provides recommendations where appropriate in assessing indications for therapy, reducing therapy-associated immunosuppression and reducing healthcare utilisation in patients with specific haematological malignancies during the COVID-19 pandemic. Specific decisions regarding therapy of haematological malignancies will need to be individualised, based on disease risk, risks of immunosuppression, rates of community transmission of COVID-19 and available local healthcare resources., (© 2020 Royal Australasian College of Physicians.)

Background: Clinical trial participants receiving investigational new drugs, which subsequently become approved by a medicines regulatory authority for its trialled indication, effectively gain free early access to efficacious treatment. Participants may also benefit from receiving approved, but unsubsidised medicines. These financial benefits of clinical trial participation have not previously been defined or quantified. Additionally, there are limited Australian pharmaceutical cost avoidance studies quantifying government savings through sponsored clinical trials., Aims: To calculate pharmaceutical financial benefits and cost avoidance of clinical trial participation at a single Clinical Research Unit., Methods: Recruiting clinical trials between 1 January 2006 and 31 December 2017 conducted at the Haematology Clinical Research Unit, Concord Repatriation General Hospital, Sydney were reviewed. Dispensing records were used to quantitate the pharmaceuticals dispensed to every participant. Financial calculations were based on Pharmaceutical Benefits Scheme (PBS) pricing, or from UpToDate for non-PBS listed agents., Results: Thirty-six eligible clinical trials involving 245 participants accrued AU$3 971 357 in financial benefit from early access to subsequently approved investigational new drugs, AU$12 209 538 in financial benefit from accessing approved medications not PBS listed, and AU$6 728 576 in government cost avoidance. These findings totalled AU$22 909 471, 89% of which was derived in the past 5 years., Conclusion: Pharmaceutical financial benefit is a previously unquantified aspect of clinical trial participation, its assigned value reflecting a measure of the quality and quantity of life delivered to patients. These data, albeit from a single discipline and institution, suggest that financial benefit represents a greater value than cost avoidance, and that its inclusion in cost-analyses may better reflect the monetary benefits of accessing efficacious pharmaceutical agents through clinical trials., (© 2019 Royal Australasian College of Physicians.)

Mantle cell lymphoma (MCL) and the marginal zone lymphoma (MZL) subtypes (nodal MZL, extra-nodal MZL of mucosa-associated lymphoid tissue (MALT lymphoma) and splenic MZL) are uncommon lymphoma subtypes, accounting for less than 5-10% of all non-Hodgkin lymphoma. The evidence base for therapy is therefore limited and enrolment into clinical trials is preferred. Outcomes for patients with MCL have been steadily improving mainly due to the adoption of more intense strategies in younger patients, the use of rituximab maintenance and the recent introduction of bendamustine in older patients. MZL is a more heterogeneous group of cancer with both nodal, extra-nodal and splenic subtypes. Extranodal MZL may be associated with autoimmune or infectious aetiologies, and can respond to eradication of the causative pathogen. Proton pump inhibitor plus dual antibiotics in Helicobacter pylori positive gastric MALT lymphoma is curative in many patients. Watchful waiting is appropriate in most patients with asymptomatic advanced stage disease, which tends to behave in a particularly indolent manner. Other options for symptomatic disease include splenectomy, chemoimmunotherapy with rituximab and, more recently, targeted therapies., (© 2019 Royal Australasian College of Physicians.)

Outcomes with follicular lymphoma (FL) have improved in the modern era and median survival is now beyond 15 years. Therapeutic decisions need to consider this increased survival as well as recent clinical trial data and emerging treatments. In this context, we present here current approaches to front-line management of FL in Australia. Treatment choices depend on the disease stage, tumour burden, the patient's age, symptoms, comorbidities and preferences. Only about 10-15% of patients with FL are diagnosed with early stage disease. For patients with low-grade, early stage disease, radiotherapy (RT) is recommended. The addition of chemotherapy has been shown to increase progression-free survival (PFS) but without demonstrated overall survival advantage. For patients with low-tumour-burden, advanced-stage FL, immediate treatment may not be required and we recommend considering active monitoring. For stage III/IV disease that is symptomatic and/or with high tumour burden, established first-line treatment is chemotherapy in combination with rituximab, often followed by rituximab maintenance. The listing of bendamustine and now obinutuzumab on the Pharmaceutical Benefits Scheme has expanded the first-line treatment options in Australia to include bendamustine in combination with rituximab (without rituximab maintenance permitted) or with obinutuzumab plus 2 years obintuzumab maintenance. In the FL subgroup of the Study group indolent Lymphomas (StiL) trial, therapy with bendamustine plus rituximab significantly increased PFS compared with rituximab in combination with cyclophosphamide, doxorubicin, vincristine and prednisolone, without rituximab maintenance. Initial tolerability may be more favourable with bendamustine in combination with anti-CD20 antibody therapy than other therapies overall, but clinical vigilance is still required because of concerns of late infectious toxicities associated with prolonged T-cell depletion., (© 2018 Royal Australasian College of Physicians.)

Background: Multidisciplinary team (MDT) meetings aimed at facilitating peer review have become standard practice in oncology. However, there is scant literature on the optimal structure and conduct of such meetings., Aims: To develop a process for formal peer review of patients with haematological malignancies and to audit any resulting changes made to the management recommendations of the treating physician., Methods: A standard operating procedure (SOP) for MDT meetings was developed essentially to integrate clinical peer review with weekly pathology and radiology meetings. The centrepiece is the electronic submission of a patient-specific proforma (Microsoft InfoPath) prior to the meeting. It serves as the template for presentation, discussion and recording of recommendations and conclusions. The final verified document is stored in the electronic patient record, and a copy is sent to the general practitioner. The proposed management plans were compared to the consensus recommendations of the meeting for the first 4 years since inception., Results: Both SOP and proforma underwent continual improvements. These provided the framework for the conduct of a robust weekly MDT meeting for peer review of the management of patients with haematological malignancies. On 20% of occasions, patient management plans were altered to optimise patient care as a direct consequence on peer review at the MDT., Conclusion: Our streamlined process, in its ultimate format, has provided a mature and efficient forum for formal peer review in a genuine multidisciplinary environment. Both initial data and informal feedback support its ongoing activity as an integral component of delivering quality patient care., (© 2016 Royal Australasian College of Physicians.)

Waldenström macroglobulinaemia (WM) is an indolent B-cell malignancy characterised by the presence of immunoglobulin M (IgM) paraprotein and bone marrow infiltration by clonal small B lymphocytes, plasmacytoid lymphocytes and plasma cells. The symptoms of WM are protean, often follow an asymptomatic phase and may include complications related to the paraneoplastic effects of IgM paraprotein. The revised 2016 World Health Organization classification includes the MYD88 L265P mutation, which is seen in >90% of cases, within the diagnostic criteria for WM. While treatment of WM has often been considered together with other indolent B cell lymphomas, there are unique aspects of WM management that require specific care. These include the unreliability of IgM and paraprotein measurements in monitoring patients prior to and after treatment, the lack of correlation between disease burden and symptoms and rituximab-induced IgM flare. Moreover, while bendamustine and rituximab has recently been approved for reimbursed frontline use in WM in Australia, other regimens, including ibrutinib- and bortezomib-based treatments, are not funded, requiring tailoring of treatment to the regional regulatory environment. The Medical and Scientific Advisory Group of the Myeloma Foundation Australia has therefore developed clinical practice guidelines with specific recommendations for the work-up and therapy of WM to assist Australian clinicians in the management of this disease., (© 2017 Royal Australasian College of Physicians.)

Background: Elderly patients with diffuse large B-cell lymphoma (DLBCL) have an inferior prognosis, due in part to advanced age and pre-existing comorbidities, with reduced tolerability and deliverability of standard R-CHOP chemotherapy., Aims: To examine the deliverability, toxicity and efficacy of R-CHOP and the prevalence of the germinal and non-germinal phenotype DLBCL in an elderly Australian cohort., Methods: This retrospective analysis included patients ≥75 years diagnosed with DLBCL. Comprehensive chemotherapy and toxicity data were collected for patients treated with R-CHOP. Baseline demographics and chemotherapy characteristics were compared with progression-free (PFS) and overall survival (OS). Immunohistochemical staining identified the prevalence of the non-germinal centre (non-GCB) phenotype., Results: Of the 111 patients, 92 (83%) commenced R-CHOP with 26/92 (28%) receiving ≤4 cycles. Median average relative dose (ARD) was 0.80 (0.07-1.17). Median average relative dose intensity (ARDI) was 0.89 (0.33-1.18). Serious adverse events occurred in 77% of patients with ≥Gd3 adverse events in 74%. Overall response rate was 85%. Two-year PFS was 63% and OS 74%. ARD and performance status ≥2 were significant prognostic factors for PFS and OS but not ARDI. Non-GCB-phenotype was identified in 44/72 (61%) of patients with immunohistochemical data., Conclusion: Despite high response rates and respectable survival estimates, the absence of standard therapy in 17% of patients, and dose reductions and serious toxicity of R-CHOP in this Australian cohort highlights the need for the development of less toxic yet efficacious treatments for very elderly patients with DLBCL. The high prevalence of the non-GCB phenotype highlights the potential value of targeted biological therapy for this demographic., (© 2015 Royal Australasian College of Physicians.)

Background: Anticancer drugs are often expensive and are contributing to the growing cost of cancer care. Concerns have been raised about the effect rising costs may have on availability of new anticancer drugs., Aim: This study aims to determine the recent changes in the costs of anticancer drugs in Australia., Methods: Publicly available expenditure and prices paid by the Australian Pharmaceutical Benefits Scheme (PBS) for anticancer drugs from 2000 to 2012 were reviewed. The measures used to determine changes in cost were total PBS expenditure and average price paid by the PBS per prescription for anticancer drugs and for all PBS listed drugs. An estimated monthly price paid for newly listed anticancer drugs was also calculated., Results: Annual PBS expenditure on anticancer drugs rose from A$65 million in 1999-2000 to A$466 million in 2011-2012; an average increase of 19% per annum. The average price paid by the PBS per anticancer drug prescription, adjusted for inflation, increased 133% from A$337 to A$786. The real average annual increase in the price per anticancer drug prescription was more than double that for all other PBS drugs combined (7.6% vs 2.8%, difference 4.8%, 95% confidence interval -0.4% to 10.1%, P = 0.07). The median price for a month's treatment of the new anticancer drugs listed was A$4919 (range A$1003 to A$12 578, 2012 prices)., Conclusions: PBS expenditure and the price of anticancer drugs in Australia rose substantially from 2000 to 2012. Dealing with these burgeoning costs will be a major challenge for our health system and for those affected by cancer., (© 2014 The Authors; Internal Medicine Journal © 2014 Royal Australasian College of Physicians.)

Autologous stem cell transplantation (ASCT) has a well-established role in the treatment of haematological malignancies. Stem cells are commonly collected following salvage chemotherapy although there may be advantages in collecting earlier in the disease course. A 'rainy day' harvest (RDH) refers to the collection of autologous haemopoietic stem cells for long-term storage. Although there are few data to support RDH, there is increasing evidence that such harvests are being carried out, creating storage pressures in stem cell laboratories across New South Wales. The Bone Marrow Transplant Network New South Wales conducted a three-staged exercise to develop consensus-based RDH guidelines. Using available evidence, guidelines were developed supporting RDH for specific patients with acute and chronic myeloid leukaemias, follicular and other lymphomas, and multiple myeloma. Physician agreement with these disease-specific guidelines ranged between 58 and 100%. These consensus guidelines will improve equity of access to appropriate RDH and assist the planning of future storage requirements in New South Wales.