The EASEMENT study: A multicentre, observational, cross-sectional study to evaluate patient preferences, treatment satisfaction, quality of life, and healthcare resource use in patients with multiple myeloma receiving injectable-containing or fully oral therapies.

OBJECTIVE: As multiple myeloma (MM) therapies advance, understanding patients', caregivers', and physicians' perspectives on, and satisfaction with, available treatment options and their impact on quality of life (QoL), is important. METHODS: EASEMENT is a real-world, observational, cross-sectional study conducted in 19 sites within the UK, Canada, and Italy using retrospective chart reviews and surveys. Enrolled patients had clinical history available since diagnosis and had received ≥1 cycle of their current line of therapy. Primary objectives were to describe patient/caregiver QoL (EQ-5D-5L questionnaire), patient preference for oral/injectable therapies (single discrete-choice question), and patient satisfaction (TSQM-9 questionnaire). RESULTS: Between October 2018 and March 2020, 399 patients were enrolled (n = 192 newly diagnosed multiple myeloma [NDMM], n = 206 relapsed/refractory multiple myeloma [RRMM], n = 1 missing). Among NDMM and RRMM patients, 78%/22% and 42%/58% were receiving injectables/orals, respectively. Both NDMM and RRMM patients significantly preferred orals versus injectables (p < .0001). No significant differences were reported in treatment satisfaction or QoL, but treatment convenience favoured orals over injectables with near significance (p = .053). CONCLUSION: MM patients perceived greater convenience and preference for orals versus injectables. Oral treatments are useful for patients who cannot or prefer not to travel to clinics, or cannot perform self-injection within the community.

Thrombus Distribution in Vaccine-induced Immune Thrombotic Thrombocytopenia after ChAdOx1 nCov-19 Vaccination.

Vaccination strategies have been at the forefront of controlling the COVID-19 pandemic. An association between vaccine-induced immune thrombotic thrombocytopenia (VITT) and one of these vaccines, the ChAdOx1 nCov-19 vaccine, is now recognized. The purpose of this study was to investigate the frequency and location of thrombosis in each vascular system using CT, MRI, and US to identify additional sites of thrombus in a United Kingdom-wide sample of patients with confirmed VITT. Thirty-two radiology centers identified through the national collaborative Radiology Academic Network for Trainees were invited from the United Kingdom; seven of these contributed to this study. All patients with confirmed VITT ¬between February 3 and May 12, 2021, who met the inclusion criteria were included. The location and extent of thrombi were evaluated using CT, MRI, and US. A total of 40 patients (median age, 41 years [IQR, 32-52]; 22 [55%] men) with confirmed vaccine-induced immune thrombotic thrombocytopenia after administration of their first ChAdOx1 nCov-19 vaccine were included. Thirty-two patients (80%) developed symptoms within the first 14 days, and eight (20%) developed symptoms within 14-28 days. Twenty-nine patients (72%) experienced neurologic symptoms and were confirmed to have cerebral venous sinus thrombosis, 12 (30%) had clinical deterioration and repeat imaging demonstrated extension of their primary thrombus, and eight (20%) died. Twenty-five of 30 patients (83%) who underwent additional imaging had occult thrombosis. In conclusion, patients with VITT are likely to have multiple sites of thrombosis, with the most frequent being cerebral venous sinus thrombosis in combination with pulmonary embolism and portomesenteric venous thrombosis. Whole-body imaging with contrast-enhanced CT can be used to identify occult thrombosis.

Imaging and Hematologic Findings in Thrombosis and Thrombocytopenia after ChAdOx1 nCoV-19 (AstraZeneca) Vaccination.

This case series reports six patients (four men and two women; median age, 38 years; interquartile range, 26-48 years) who presented with vaccine-induced thrombocytopenia and thrombosis beginning 3-26 days after receiving the first dose of the ChAdOx1 nCoV-19 (AstraZeneca) vaccine for COVID-19. The patients were admitted to a general hospital between 9 and 31 days after the first dose. All patients had strongly detected antiplatelet factor 4 antibodies and severe thrombosis. Laboratory features included thrombocytopenia and elevated d-dimer levels. Thrombotic events were predominantly venous; two patients had arterial or mixed arterial and venous thrombosis. All patients recovered after receiving intravenous immunoglobulin and nonheparin-based anticoagulation. © RSNA, 2021 An earlier incorrect version appeared online. This article was corrected on August 18, 2021.

Adult T cell leukaemia/lymphoma (ATL) in pregnancy: A UK case series.

Introduction: Chronic infection with human T-cell lymphotropic virus type-1 (HTLV-1) may result in aggressive adult T-cell leukaemia/lymphoma (ATL) in 4-6% carriers. The majority of this risk arises in carriers infected during infancy, and so each infant has ∼25% lifetime risk. Other risk factors include a family history of ATL. Antenatal HTLV-1 screening is not undertaken in the UK. Methods: Here we describe four cases of ATL diagnosed during pregnancy and describe strategies to minimise HTLV-1 transmission to neonates. Results/conclusion: These cases highlight undiagnosed HTLV-1 in pregnancy which allows ongoing mother to child vertical transmission and risk of future ATL. We recommend the UK National Screening Committee incorporate HTLV-1 serology into antenatal screening.

Lesson of the month 1: Massive spontaneous haematomas in an elderly man.

A 73-year-old man presented with bilateral leg pain and swelling, and no history of trauma or bleeding disorders. Clinical examination, biochemistry and magnetic resonance imaging of the thighs were suggestive of muscle haematomas. These progressed significantly during the admission, requiring blood transfusion. Normal vascular anatomy on computed tomography, renal and liver function, and absence of infection made a bleeding diathesis more likely. This may be caused by coagulation defects, platelet disorders and vascular fragility. An undetectable serum ascorbic acid level confirmed the clinical suspicion of scurvy, and administration of vitamin C resulted in rapid improvement. Our case provides a structured approach to the diagnosis of bleeding disorders and scurvy, a treatable and potentially fatal disease which is often forgotten.

Gaucher disease and myeloma.

Gaucher disease (GD), the commonest lysosomal storage disorder, is an autosomal recessive condition, caused by deficiency in lysosomal glucocerebrosidase. Skeletal disease, peripheral blood cytopenias and hepato-splenomegaly are common at presentation. Several reports describe an increased risk of cancer in GD; in particular multiple myeloma. Tumor Associated Macrophages (TAMs) may promote cancerous expansion in non-GD patients, but it is unknown whether such an occurrence is present in GD. GD is traditionally described as a disorder of macrophages, associated with numerical and functional lymphoid abnormalities which could contribute to a state of impaired 'tumor surveillance'. These abnormalities include disturbances in invariant NK-T cells that depend on sphingolipid metabolism to deliver a physiological response. Glucosylceramide deposition, chronic antigenic stimulation, increased free radical production, impaired antigen presentation, reduced intra-cellular ceramide levels and disturbed autophagy have all been postulated to facilitate the growth of malignant clones in the GD microenvironment. At present, it is unknown whether carrier status for a GBA1 mutation confers an increased risk of malignancy. A cancer diagnosis is not currently an indication for GD-specific therapy in the absence of other features of the disease This article reviews cancer epidemiology, potential biological mechanisms of carcinogenesis and highlights areas of future research regarding malignancy in GD.