Hemophagocytic lymphohistiocytosis (HLH) is rarely described in association with chronic lymphocytic leukemia (CLL), mostly triggered by disease progression or concurrent infection. A 68-year-old male received 4 cycles of fludarabine, cyclophosphamide, and rituximab (FCR) for CLL and achieved a complete response. Twenty-four days after the last chemotherapy, he presented with febrile neutropaenia and was diagnosed with HLH. The diagnosis was based upon persistent fever, pancytopenia, hyperferritinemia, splenomegaly, and hemophagocytosis on bone marrow aspirate. He began treatment with dexamethasone, cyclosporine, and etoposide. Fever resolved and hyperferritinemia improved but pancytopenia persisted. He died 13 days later from septic shock with positive blood cultures. A limited postmortem examination was performed and biopsies were taken from bone marrow, liver, and spleen. Biopsies demonstrated abundant hemophagocytosis by the activated macrophage as stained by CD68. There was no evidence of residual CLL as demonstrated by the lack of lymphocytes which was confirmed by the negative staining of CD79a. Chemotherapy appears to be responsible for the development of HLH in this patient. This is the second reported case of HLH developing after a rituximab-containing chemotherapy.
Acute Rheumatic Fever (ARF) is an autoimmune condition caused by untreated Group A Streptococcal (GAS) infection of the upper respiratory tract (and possibly skin). Multiple or severe attacks of ARF can cause cardiac damage, known as Rheumatic Heart Disease (RHD) which remains a significant cause of morbidity and mortality globally and is rare in developed countries. In Australia, New Zealand (NZ) and the Pacific Region, the disease burden of ARF and RHD amongst Indigenous and Pacific communities is one of the highest in the world, usually affecting children and young adults. A GAS vaccine remains years from being available and until then, the most effective recommended preventative measure for ARF requires painful monthly intramuscular injections of Benzathine Penicillin G (BPG) for 10 years or more, known as secondary prophylaxis (SP). Adherence to SP remains a challenge in many settings and improved understanding of barriers and novel approaches to BPG delivery are urgently needed. To create a reformulation of BPG that encourages SP adherence, insight is needed into the reformulation preferences of those with ARF/RHD. This work seeks to explore the experiences and investigate children/teen on SP, family, and healthcare professional and practitioner preferences for potential BPG reformulation characteristics in Tonga. Three software applications will be adapted from pre-developed applications already piloted and pretested, and optimized for use to ensure age, cultural appropriateness and efficient data collection. This is the first time software applications have been successfully developed and utilized to collect qualitative and quantitative data on individual preferences for BPG formulations and dosing regimens in Tonga.
Acute Rheumatic Fever (ARF) is an autoimmune condition caused by untreated Group A Streptococcal (GAS) infection of the upper respiratory tract (and possibly skin). Multiple or severe attacks of ARF can cause cardiac damage, known as Rheumatic Heart Disease (RHD) which remains a significant cause of morbidity and mortality globally and is rare in developed countries. In Australia, New Zealand (NZ) and the Pacific Region, the disease burden of ARF and RHD amongst Indigenous and Pacific communities is one of the highest in the world, usually affecting children and young adults. A GAS vaccine remains years from being available and until then, the most effective recommended preventative measure for ARF requires painful monthly intramuscular injections of Benzathine Penicillin G (BPG) for 10 years or more, known as secondary prophylaxis (SP). Adherence to SP remains a challenge in many settings and improved understanding of barriers and novel approaches to BPG delivery are urgently needed. To create a reformulation of BPG that encourages SP adherence, insight is needed into the reformulation preferences of those with ARF/RHD. This work seeks to explore the experiences and investigate children/teen on SP, family, and healthcare professional and practitioner preferences for potential BPG reformulation characteristics in Tonga. Three software applications will be adapted from pre-developed applications already piloted and pretested, and optimized for use to ensure age, cultural appropriateness and efficient data collection. This is the first time software applications have been successfully developed and utilized to collect qualitative and quantitative data on individual preferences for BPG formulations and dosing regimens in Tonga.
Circulating tumour DNA (ctDNA) refers to small fragments of tumour DNA found in the blood circulation. These DNA fragments can be isolated and quantified to obtain data about a cancer’s size and progression. The possibility of utilizing a simple blood sample to allow early detection and monitoring of cancer growth is a highly desirable application for this ground breaking technology. Significant health inequalities exist for Pacific peoples with cancer living in New Zealand and in the Pacific region, where the burden of cancer remains a public health concern. The application of this simple ctDNA method of detecting and monitoring cancer to enhance the precision of early cancer diagnostics and surveillance to improve cancer health outcomes for Pacific peoples in Tonga was investigated. Consultation was sought with senior Government officials, Medical, Nursing, Health and Community research staff concerning the development and implementation of ctDNA as a diagnostic tool within the health care setting throughout Tonga. Preliminary assessment of the on-the-ground laboratory requirements needed for the implementation of the ctDNA technology was also undertaken. Strong support for the development of ctDNA as an early diagnostic tool within the clinical setting was expressed. A key feature of the ctDNA technology within this resource constrained environment was the anticipated detection of cancers at an earlier stage with a greater chance of being treatable given that most cancer presentations in Tonga are late. This work sought to explore and progress the implementation of ctDNA as an early cancer diagnostic tool within the Pacific setting, and remains a highly feasible early cancer detection tool within Tonga.
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