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A sharp-eyed expose of the deadly politics, murderous plots, and cutthroat rivalries behind the first blood transfusions in seventeenth-century Europe.

Purpose To characterize the health-related quality of life (HRQoL) and economic impacts of transfusion-dependent β-thalassemia (TDT). Methods A prospective longitudinal real-world study was conducted in adults with TDT in the US and Europe (France, Germany, Italy, UK) over 6 months. Eligibility criteria included receiving ≥ 8 blood transfusions/year in each of the 2 years before enrollment. Study outcomes assessed at months 0, 3, and 6, included demographics, clinical characteristics, patient-reported outcome measures (EQ-VAS, FACT-G, FACIT-F, 11-point Numerical Rating Scale (NRS) of pain, TranQoL, and WPAI), and responses to bespoke questions on financial burden/distress. Results Overall, 155 adults with TDT participated (mean age [standard deviation (SD)]: 38.5 [10.8] years; 65.8% female). Most participants resided in the US (53.5%) or UK (22.6%). EQ-VAS, FACT-G, and FACIT-F mean (SD) scores at month 0 were lower among participants than the US general population (67.1 [20.4] versus 80.4 [15.6], 70.1 [19.0] versus 77.0 [16.1], and 27.9 [13.7] versus 43.6 [9.4], respectively; all p  < 0.001). Mean (SD) TranQoL total score was 53.9 (18.5) and overall level of pain was “moderate” (NRS mean [SD]: 3.5 [2.8]). Part- or full-time employed participants missed a mean of 5.9 h of work in the preceding 7 days with out-of-pocket expenses being a moderate-to-major burden for 55% of participants. The findings were consistent over time. Conclusions Participants reported substantial humanistic and economic burdens associated with living with TDT. Our findings highlight the need for alternative treatment options that contribute to improvements in HRQoL and reduce economic burden in this patient population. Plain English summary This study explored the impact of living with an inherited blood disorder that limits the body’s ability to make hemoglobin and requires frequent red blood cell transfusions and iron chelation treatment for survival: transfusion-dependent β-thalassemia (TDT). This study was completed over 6 months in the US and Europe, and participants shared their experiences living with TDT by completing survey questions. Our results show the physical, emotional, and financial burden experienced by individuals living with TDT. Compared to published information about the general population, many people with TDT reported experiencing shortness of breath, tiredness, fatigue, pain, and discomfort, all negatively affecting their health-related quality of life. Additionally, a majority reported financial distress due to TDT treatment and management. Together, these results highlight the burden of living with TDT and the argument for curative treatment options.

Tunneled central venous catheters (CVC) were developed five decades ago. Since then, several clinician-inventors have created a variety of catheters with different functions. Indeed, many catheters have been named after their inventor. Many have wondered who the inventors were of each catheter, and what specifically inspired their inventions. Many of these compelling stories have yet to be told. A literature review of common catheters and personal communication with inventors. Only first person accounts from inventors or those close to the invention were used. CVCs are now essential devices that have saved countless lives. Though the inventors have earned the honor of naming their catheters, it may be reasonable to consider more consistent terminology to describe these catheters to avoid confusion. •Tunneled central venous catheters have now been available for patients for exactly fifty years.•Many variations have been developed.•This review provides the reader first person accounts of the development of these life saving devices.

This fifteenth annual European Group for Blood and Marrow Transplantation activity report lists the transplant activity in Europe in 2004 and documents the changes in indication over the past 15 years. In 2004, there were 22 216 first hematopoetic stem cells (HSCT), 7407 allogeneic (33%), 14 809 autologous (67%) and 4378 additional re- or multiple transplants reported from 592 centres in 38 European and five affiliated countries. Main indications were leukemias (7045 (32%; 78% allogeneic)); lymphomas (12 310 (55%; 94% autologous)); solid tumors (1759 (8%; 93% autologous)) and nonmalignant disorders (1015 (5%; 92% allogeneic)). In comparison, 145 teams from 20 countries performed 4234 HSCT (2137 allogeneic, 50%; 2097 autologous, 50%) in 1990. The overall increase was accompanied by major changes. Stem cell source changed from bone marrow to peripheral blood. More than one-third of allogeneic HSCT are now from unrelated donors. Reduced intensity conditioning is employed for one-third of allogeneic HSCT. Leukemias for allogeneic and lymphoproliferative disorders for autologous HSCT continue to increase. The decline in HSCT for chronic myeloid leukemia appears to level off for the first time since 1999. These data are informative for patient counselling and decision making for health care professionals.

There have been four types of drug treatment of asthma that have been used over the past 100 years. Belladonna alkaloids, derived from the thorn-apple plant were used in 1905, and chemically synthesized entities in this class are still in use today. Western medicine began to use adrenergic stimulants approximately 100 years ago, but they were likely used in Asian medicine long before that. Systemic treatment with corticosteroids was introduced into the treatment of asthma in the mid-20th century; inhaled corticosteroids have been in use for over 35 years. The last 40 years have also seen the development of the first targeted asthma treatments: cromones, antileukotrienes, and anti-IgE. As we learn more of the biology of asthma, we anticipate that more effective targeted asthma treatments will be developed.

Vitamin B12 deficiency is a recognised pathology in several populations, with a particular prevalence in an older adult population. We present two cases whereby vitamin B12 deficiency is the causative factor in marked pancytopaenia. Oval macrocytosis and hypersegmented neutrophils were noted on both peripheral blood samples, which are a characteristic finding in macrocytic anaemia due to B12 deficiency. Distinct underlying pathologies were identified in both cases; food-cobalamin malabsorption and pernicious anaemia. Parenteral vitamin B12 supplementation resulted in a marked reticulocytosis and rapid improvement of haematological indices in both cases. We present this series to serve as a reminder that B12 deficiency can present as life-threatening pancytopaenia.It has has multiple underlying pathologies,defined risk populations and has characteristic blood film findings which can guide investigations, diagnosis and treatment.

A 34-year-old woman presented to our service with chest pain, a troponin rise and dynamic ECG changes. Of note, she had complained of fatigue, feeling constitutionally unwell and a generalised rash in the days prior to her presentation. Her echocardiogram showed normal wall motion and preserved ejection fraction. Her eosinophil count, normal at presentation, rose to a peak of 12.21×105/L. She was haemodynamically stable throughout with no evidence clinically of congestive cardiac failure. CT coronary angiogram showed no obstructive coronary artery disease. Cardiac MRI demonstrated areas of late gadolinium enhancement consistent with myocarditis. A diagnosis of eosinophilic myocarditis was made. No tissue biopsy was performed due to the patchy myocardial involvement and high potential for low-yield biopsy. Our patient was treated conservatively and has made an excellent recovery.

We report on the obstetric outcome of a woman aged 27 years with Hermansky-Pudlak syndrome (HPS). She underwent a caesarean section after failed induction of labour. Platelet transfusion was administered in a set schedule for 36 hours, starting 2 hours before delivery. The child had good Apgar scores and there were no significant problems of prolonged bleeding during the procedure. 72 hours postpartum, a haematoma developed at the site of the wound, subsequently complicated by a secondary infection for which she received antibiotics. Wound care was provided in an outpatient setting during 2 weeks, in which the infection stabilised and responded to the treatment. Mother and child could leave the hospital after 6 days.

Thrombotic thrombocytopenic purpura (TTP) affects essentially all organ systems. Myocardial injury in TTP is often attributed to microthrombi formation. We present the first case report in the literature of an acute TTP patient with concomitant obstructive coronary artery disease (CAD) and acute myocardial infarction who underwent successful percutaneous coronary intervention (PCI). A 70-year-old female patient who was diagnosed with acute TTP required plasma exchange. The patient also experienced episodes of angina pectoris, elevated cardiac enzymes and global ST segment depressions on ECG. A subsequent non-invasive ischaemia workup revealed significant ischaemia. Coronary angiography revealed obstructive CAD in her right coronary artery, requiring PCI with a bare metal stent placement and dual antiplatelet therapy. The patient tolerated antiplatelet therapy well. At 6 months of follow-up, she had no recurrent angina. This case highlights the potential co-existence of obstructive CAD and acute TTP requiring careful differential diagnosis and treatment.

This article examines the introduction of blood transfusion into general practice from the end of the First World War to the Second World War. Developments during most of this period were not the result of new discoveries but rather the spread of ideas and the establishment of donor organizations to secure an adequate blood supply. The identification, testing, and organization of potential donors were done in a wide variety of settings that reflected differences in political and cultural experiences. At the end of the 1930s, with war approaching, the resolution of problems with storage of blood and the discovery of new techniques for separating and storing plasma dramatically changed transfusion practice. Thus, the innovations of the Second World War were very much based on the development of broad donor organizations plus the new technical discoveries that had occurred during the interwar period.