Thrombosis risk with haemoglobin C trait and haemoglobin C disease: A systematic review.

The thrombotic risk with haemoglobin C trait (HbAC) or haemoglobin C disease (HbCC) is unclear. However, individuals with HbCC have demonstrated chronic haemolysis, higher blood viscosity and altered rheology when compared to individuals with wild-type haemoglobin (HbAA). These physiological alterations may theoretically translate to increased risk of thrombosis; therefore, a systematic literature review was performed to investigate the possible association between HbAC and/or HbCC and thrombosis. Twenty-two studies met inclusion criteria representing 782 individuals with HbAC (n = 694) or HbCC (n = 88). Fifteen studies described the presence/absence of venous thromboembolism (VTE) in patients with HbAC (n = 685) or HbCC (n = 79), while seven studies described patients with HbAC (n = 9) or HbCC (n = 9) and arterial thrombosis. Most (n = 20) studies were case reports or case series; however, two studies suggested a potential increased VTE risk with HbAC compared to HbAA in (i) all patients (OR 2.2, 95% CI: 0.9-5.5) and in (ii) pregnant individuals (RR 3.7, 95% CI 0.9-16). This review is the largest assessment of patients with HbC trait or disease and thrombosis to date; despite its limitations, the findings suggest HbC may be a predisposing risk factor to thrombosis. Prospective cohort studies are warranted to definitively elucidate the risk of thrombosis in this population.

How the United States syphilis epidemic may portend a resurgence of an unusual hematologic condition: The connection between syphilis and paroxysmal cold hemoglobinuria.

The connection between syphilis and paroxysmal cold hemoglobinuria.

The seasonal distribution of immune thrombotic thrombocytopenic purpura is influenced by geography: Epidemiologic findings from a multi-center analysis of 719 disease episodes.

Prior studies have suggested that immune thrombotic thrombocytopenic purpura (iTTP) may display seasonal variation; however, methodologic limitations and sample sizes have diminished the ability to perform a rigorous assessment. This 5-year retrospective study assessed the epidemiology of iTTP and determined whether it displays a seasonal pattern. Patients with both initial and relapsed iTTP (defined as a disintegrin and metalloprotease with thrombospondin type motifs 13 activity <10%) from 24 tertiary centers in Australia, Canada, France, Greece, Italy, Spain, and the US were included. Seasons were defined as: Northern Hemisphere-winter (December-February); spring (March-May); summer (June-August); autumn (September-November) and Southern Hemisphere-winter (June-August); spring (September-November); summer (December-February); autumn (March-May). Additional outcomes included the mean temperature in months with and without an iTTP episode at each site. A total of 583 patients experienced 719 iTTP episodes. The observed proportion of iTTP episodes during the winter was significantly greater than expected if equally distributed across seasons (28.5%, 205/719, 25.3%-31.9%; p = .03). Distance from the equator and mean temperature deviation both positively correlated with the proportion of iTTP episodes during winter. Acute iTTP episodes were associated with the winter season and colder temperatures, with a second peak during summer. Occurrence during winter was most pronounced at sites further from the equator and/or with greater annual temperature deviations. Understanding the etiologies underlying seasonal patterns of disease may assist in discovery and development of future preventative therapies and inform models for resource utilization.

New threats from an old foe: Evaluating the risk to the blood supply due to increasing incidence and endemicity of leprosy in the United States.

Leprosy (i.e., Hansen's disease) is a chronic disease secondary to infection with either Mycobacterium leprae or M. lepromatosis. While the incidence of this disease is decreasing across the world, there is mounting evidence that it might be increasing, and becoming endemic, in the United States. Leprosy was once considered a potential threat to the blood supply, and while this threat has not borne out, it is worth revisiting the available data to assess whether it may pose a threat in the future. Herein, we discuss the evidence for and against the potential for transfusion-transmission of leprosy, and highlight future areas of research to further elucidate this possibility.

A multi-institutional survey of apheresis services among institutions in the United States.

INTRODUCTION: Apheresis practices in the United States (US) have not been comprehensively characterized to date. This study aimed to address this gap by evaluating apheresis therapy through a national survey. METHODS: A multi-institutional survey was conducted between April and July 2023. The survey, comprising 54 questions, focused on institutional demographics, procedures, equipment, staffing, training, and impacts of the Coronavirus Disease 2019 (COVID-19) pandemic. Responses from 22 institutions, primarily academic medical centers, were analyzed. RESULTS: Therapeutic plasma exchange (TPE) was the most common procedure, followed by hematopoietic progenitor cell collection (HPC-A) and red blood cell exchange (RCE). CAR-T cell collections were widespread, with some institutions supporting over 30 protocols concurrently. Most sites used the Spectra Optia Apheresis System, were managed by a transfusion medicine service, and employed internal apheresis providers. Insufficient staffing levels, exacerbated by the COVID-19 pandemic, were common and most often addressed using overtime. DISCUSSION: The survey highlighted the ubiquity of TPE, expanding cellular collections and staffing challenges. The role of apheresis in supporting cellular therapy, particularly in newly developing cell and gene therapies and clinical trials, was evident. Staffing issues during the pandemic emphasized the need for innovative recruitment strategies. CONCLUSION: This nationwide survey provides the most comprehensive analysis to date of apheresis practices in large US academic centers.

Gender Representation on Editorial Boards of JAMA Network Journals.

Objective: Underrepresentation of women on editorial boards of biomedical journals has occurred for decades. The JAMA Network Journals have substantial and broad impact on advances in the biomedical sciences. We sought to determine the current status of gender representation on editorial boards of the 12 JAMA Network Journals. Methods: The gender of each editorial board member of the 12 JAMA Network Journals was classified based on review of online sources. The percentage of women on each board (i.e., number of women relative to total members) was calculated and compared to gender equity and parity benchmarks. The gender equity benchmark for each journal was defined as the percentage of women physicians in the medical specialty reflecting the journal's content based on Association of American Medical Colleges data. The gender parity benchmark for all journals was defined as 50% women. Results: There was considerable variation in the representation of women on the editorial boards of the JAMA Network Journals relative to gender equity and parity benchmarks. Women were underrepresented on 50% (6 of 12) of boards relative to gender equity and 67% (8 of 12) of boards relative to gender parity. Conclusions: Women were found to be underrepresented on 50% or more of the editorial boards of the JAMA Network Journals. This finding reflects gender inequities in academic publishing and the broader biomedical enterprise, which limits advances in the biomedical sciences and health care. Those JAMA Network Journals that continue to underrepresent women on their editorial boards are urged to remediate this longstanding issue.

Transfusion Support in Hematopoietic Stem Cell Transplantation: A Contemporary Narrative Review.

Hematopoietic stem cell transplantation (HSCT) is a cornerstone of modern medical practice, and can only be performed safely and effectively with appropriate transfusion medicine support. Patients undergoing HSCT often develop therapy-related cytopenia, necessitating differing blood product requirements in the pre-, peri-, and post-transplant periods. Moreover, ensuring optimal management for patients alloimmunized to human leukocyte antigens (HLA) and/or red blood cell (RBC) antigens, as well as for patients receiving ABO-incompatible transplants, requires close collaboration with transfusion medicine and blood bank professionals. Finally, as updated transfusion guidelines and novel blood product modifications emerge, the options available to the transplant practitioner continue to expand. Herein, we detail contemporary blood transfusion and transfusion medicine practices for patients undergoing HSCT.

Analysis of editor in chief gender and associated journal variables among 126 pathology journals.

OBJECTIVES: Gender equity studies have shown that women are underrepresented in journal editor in chief positions, which confer major professional opportunities and influence. We sought to systematically investigate editor in chief gender and journal attributes within pathology. METHODS: We constructed a journal data set using the Scimago Journal & Country Rank and Clarivate Journal Citation Reports databases. We also included official journals of the major medical societies for the 12 pathology subspecialties recognized by the Association of American Medical Colleges. The final data set included 126 journals. We obtained editor in chief gender, impact factor, publication model (ie, hybrid access vs open access), year of founding, and geographic location for all included pathology journals. RESULTS: Women made up only 18% of the 141 total editor in chief positions. This inequity was present irrespective of all pathology journal variables studied. Among 10 journals with 2 editor in chief positions, 5 had only men and 5 had 1 man and 1 woman. All 3 journals with 3 editor in chief positions had 2 men and 1 woman. CONCLUSIONS: Women are significantly underrepresented among editor in chiefs across pathology journals. Journals and affiliated members should advocate for diversity among these influential positions, given their impact on research, science, and medicine.

Analysis of Pediatric Clinical Practice Guideline Authors by Gender, Race, and Ethnicity.

Background and Objectives: Clinical practice guidelines (CPGs) have significantly influenced medical practice worldwide. Nevertheless, the authorship of CPGs produced by several medical societies has not been representative of the field and population they address, as women and individuals from racial and ethnic minority groups have been underrepresented as authors. We hypothesized that women and individuals from minoritized racial and ethnic groups would also be underrepresented as authors of CPGs produced by the American Academy of Pediatrics (AAP). Methods: In this cross-sectional study, the gender, race, and ethnic composition of authors and subcommittee participants of AAP-produced CPGs published from January 2010 through May 2023 were analyzed and compared to the 2010 and 2021 U.S. population and 2010 and 2022 U.S. medical school pediatric faculty. Results: Women (39.7%, 127/320 of all positions, and 42.5%, 85/200 of named author positions) and women physicians (35.2%, 101/287 of all positions, and 36.4%, 64/176 of named author positions) were significantly underrepresented-while men and men physicians were significantly overrepresented-from their respective composition in the U.S. Census and pediatric faculty. Women and women physicians from all racial and ethnic groups and men and men physicians from minority racial and ethnic groups were significantly underrepresented-from their respective composition in the U.S. Census and pediatric faculty. No Black man was identified as an author. Conclusions: Medical societies that produce CPGs should be cognizant of these inequities and ensure appropriate authorship diversity.

Assessing Recommendations for Determining Fetal Risk in Alloimmunized Pregnancies in the United States: Is It Time to Update a Decades-Old Practice?

The current recommended testing algorithm for assessing the alloimmunized pregnancy utilized by many obstetricians in the United States (US) fails to consider the most recent evidence, placing fetuses, and mothers at unnecessary risk of poor outcome or death. This narrative review of the current landscape of fetal red blood cell (RBC) antigen testing evaluates the history of hemolytic disease of the fetus and newborn (HDFN) and how its discovery has continued to influence practices in the US today. We compare current US-based HDFN practice guidelines with those in Europe. We also provide transfusion medicine and hematology perspectives and recommendations addressing the limitations of US practice, particularly regarding paternal RBC antigen testing, and discuss the most valuable alternatives based on decades of data and evidence-based recommendations from Europe.

Heidenhain variant of Creutzfeldt-Jakob disease masquerading as neuromyelitis optica spectrum disorder: recognizing when apheresis is not the answer.

The Heidenhain variant of Creutzfeld-Jakob disease (CJD) is a rare form that initially presents with visual disturbances. In early stages, the presentation can mimic neuromyelitis optica spectrum disorders (NMOSD) and lead to unnecessary treatment modalities. Herein, we describe a case of a 66-year-old man who presented with bilateral vision loss and retro-orbital discomfort. In addition to immunosuppressive therapy, he received 4 rounds of therapeutic plasma exchange after his preliminary diagnosis of NMOSD. We were surprised to note that his condition did not show improvement but deteriorated, with severe neurocognitive symptoms. Eventually, CJD was suspected, and real-time quaking-induced conversion (RT-QuIC) was performed. By the time the diagnosis of Heidenhain variant of CJD was confirmed, the patient was discharged to hospice care and died shortly after.

Gender Inequities in Transfusion Medicine Society Recognition Awards.

Award recognition by medical societies contributes to professional development, career networking, and academic rank promotion. Previous research has demonstrated that men are the predominant recipients of medical society awards across multiple medical specialties; as such, we sought to understand whether women are underrepresented as award recipients amongst blood banking and transfusion medicine (BBTM) medical societies. We examined recipients of 10 total awards from the Association for the Advancement of Blood and Biotherapies (AABB) and the American Society for Apheresis. Additional evaluation of AABB's National Blood Foundation Hall of Fame inductees was conducted. Gender was determined via online review of pronouns, online photographs, and a web-based gender identification application. Award recipient gender was analyzed and coded independently by two authors, and any discrepancies were adjudicated by author consensus. Of the 330 AABB awards since 1954, significantly more have been conferred to men (81.5%, 269/330; P < .001). Of the 51 American Society for Apheresis awards presented since 1993, 64.7% (33/51; P = .23) have been conferred to men. Compared to the first 10 years of the AABB awards (1954-1964), there has been a significant increase in the proportion of women award recipients in the most recent decade (2010-2021) (18.5%, 5/27 vs 29.4%, 30/102; P < .001). However, additional temporal analysis of the modern era (2000-2021) revealed men have received significantly more AABB awards than women (77.4%, 144/186 vs 22.6%, 42/186; P < .001). Our findings highlight both historic and contemporary inequity for recognition of women within BBTM. Without improvement, gender parity among BBTM award recipients will take approximately 120 years (11% increase in women awardees in 60 years); thus, to ensure the BBTM field continues to progress, we must advocate for equity among all members, including but not limited to gender, race, and ethnicity. Strategies to enhance equity include transparency in the identities of award nominees, award recipients, and individuals on selection committees, the gender ratios of both award nominees and recipients, and implementation of methods for tracking individual demographics over time. These strategies would permit temporal analysis of the ratio of award nominee gender to award recipient gender, and assessment as to whether potential gender inequities improve over time.