Measurement of the major ignored burden of multiple myeloma, pernicious anaemia and of other haematological conditions on partners and family members: A cross-sectional study.

BACKGROUND: Having a haematological condition can adversely affect the quality of life (QoL) of family members/partners of patients. It is important to measure this often ignored burden in order to implement appropriate supportive interventions. OBJECTIVE: To measure current impact of haematological conditions on the QoL of family members/partners of patients, using the Family Reported Outcome Measure-16 (FROM-16). METHODS: A cross-sectional study, recruited online through patient support groups, involved UK family members/partners of people with haematological conditions completing the FROM-16. RESULTS: 183 family members/partners (mean age = 60.5 years, SD = 13.2; females = 62.8%) of patients (mean age = 64.1, SD = 12.8; females = 46.4%) with 12 haematological conditions completed the FROM-16. The FROM-16 mean total score was 14.0 (SD = 7.2), meaning 'a moderate effect on QoL'. The mean FROM-16 scores of family members of people with multiple myeloma (mean = 15.8, SD = 6.3, n = 99) and other haematological malignancies (mean = 13.9, SD = 7.8, n = 29) were higher than of people with pernicious anaemia (mean = 10.7, SD = 7.5, n = 47) and other non-malignant conditions (mean = 11, SD = 7.4, n = 56, p < .01). Over one third (36.1%, n = 183) of family members experienced a 'very large effect' (FROM-16 score>16) on their quality of life. CONCLUSIONS: Haematological conditions, in particular those of malignant type, impact the QoL of family members/partners of patients. Healthcare professionals can now, using FROM-16, identify those most affected and should consider how to provide appropriate holistic support within routine practice.

Health Equity and Implementation Science in Heart, Lung, Blood, and Sleep-Related Research: Emerging Themes From the 2018 Saunders-Watkins Leadership Workshop.

Disparities in health outcomes for heart, lung, blood, and sleep-related health conditions are pervasive in the United States, with an unequal burden experienced among structurally disadvantaged populations. One reason for this disparity is that despite the existence of effective interventions that promote health equity, few have been translated and implemented consistently in the healthcare system. To achieve health equity, there is a dire need to implement and disseminate effective evidence-based interventions that account for the complex and multilayered social determinants of health among marginalized groups across healthcare settings. To that end, the National Heart, Lung, and Blood Institute's Center for Translation Research and Implementation Science invited early stage investigators to participate in the inaugural Saunders-Watkins Leadership Workshop in May of 2018 at the National Institutes of Health. The goals of the workshop were to: (1) present an overview of health equity research, including areas which require ongoing investigation; (2) review how the fields of health equity and implementation science are related; (3) demonstrate how implementation science could be utilized to advance health equity; and (4) foster early stage investigator career success in heart, lung, blood, and sleep-related research. Herein, we highlight key themes from the 2-day workshop and offer recommendations for the future direction of health equity and implementation science research in the context of heart, lung, blood, and sleep-related health conditions.

Clinical decision support for hematology laboratory test utilization.

Clinical decision support (CDS) is the use of information and communication technologies to improve clinical decision making and patient care. CDS applications have been used in many aspects of health care, including medication ordering and diagnostic prediction algorithms. As economic and regulatory pressures place a strain on laboratory resources, the potential of CDS to improve utilization of laboratory testing has also begun to be realized. Hematology and coagulation laboratories stand to gain tremendously from the implementation of CDS interventions, given their mixture of high-volume, low-cost tests (eg CBC, PT, aPTT) and tests that carry a high potential of being misused or misinterpreted (eg lupus anticoagulant, erythrocyte sedimentation rate, heparin-induced thrombocytopenia testing). This brief review will define the key terms in the field of clinical decision support, provide instructive examples of CDS interventions to improve utilization of hematology and coagulation testing, introduce methods to implement these interventions effectively, and discuss metrics by which the success of these interventions can be evaluated.

Assessment of hematological parameters in workers exposed to organophosphorus pesticides, carbamates and pyrethroids in Cundinamarca 2016-2017.

OBJECTIVE: To establish hematotoxic alterations through clinical and paraclinical exploration in workers who are exposed to organophosphorus pesticides, carbamates and pyrethroids (OPCP) due to their work in production, packaging, distribution and fumigation processes in Cundinamarca-Colombia between 2016 and 2017. MATERIALS AND METHODS: A cross-sectional descriptive epidemiological study was carried out on a sample of 92 workers from six companies, mostly aged between 18 and 30 years, of which 61 % were males and 39 % females, and 71 % were workers in the operational area and 29 % in the administrative area. Univariate, bivariate and multivariate analyses were performed. RESULTS: Clinical exploration reported findings in 17 % of the sample group, of which only 2 % presented with erythrocyte cholinesterase outside the reference range. The values of hematological parameters such as peripheral blood smear (PBS) and complete blood count (CBC) were outside the range in 15 % and 47 % of the sample, respectively. DISCUSSION: The results suggest that there are hematological alterations in this group that could possibly be associated with chronic exposure to OPCP.

Hematology in Africa.

This review of hematology in Africa highlights areas of current practice and the immediate needs for development and clinical research. Acute hematological practice is dominated by anemia, sickle cell disease, and the need to provide a safe and rapidly available supply of blood. There is a growing need for specialist services for bleeding and coagulation, hematological malignancy, and palliative care. There are many areas of practice where straightforward measures could yield large gains in patient care. There is an urgent need for good clinical research to describe the epidemiology, natural history, and management of hematological diseases in Africa.

Hematological Practice in Hong Kong and China.

Thalassemias and hemophilias are the most important inherited hematological diseases in Hong Kong and China. Prenatal diagnosis has significantly decreased the burden of these diseases. For thalassemia major, adequate transfusion and iron chelation therapy have dramatically improved patient outlook. Hematopoietic stem cell transplantation is curative for thalassemia major and is increasingly adopted. The efficacy of arsenic trioxide in acute promyelocytic leukemia (APL) was discovered in China. An oral formulation of arsenic trioxide was developed in Hong Kong for newly diagnosed and relapsed APL patients. With combination chemotherapy containing non-P-glycoprotein-dependent drugs and L-asparaginase, durable remission can be achieved in the most patients.

The European Hematology Association Roadmap for European Hematology Research: a consensus document.

The European Hematology Association (EHA) Roadmap for European Hematology Research highlights major achievements in diagnosis and treatment of blood disorders and identifies the greatest unmet clinical and scientific needs in those areas to enable better funded, more focused European hematology research. Initiated by the EHA, around 300 experts contributed to the consensus document, which will help European policy makers, research funders, research organizations, researchers, and patient groups make better informed decisions on hematology research. It also aims to raise public awareness of the burden of blood disorders on European society, which purely in economic terms is estimated at €23 billion per year, a level of cost that is not matched in current European hematology research funding. In recent decades, hematology research has improved our fundamental understanding of the biology of blood disorders, and has improved diagnostics and treatments, sometimes in revolutionary ways. This progress highlights the potential of focused basic research programs such as this EHA Roadmap.The EHA Roadmap identifies nine 'sections' in hematology: normal hematopoiesis, malignant lymphoid and myeloid diseases, anemias and related diseases, platelet disorders, blood coagulation and hemostatic disorders, transfusion medicine, infections in hematology, and hematopoietic stem cell transplantation. These sections span 60 smaller groups of diseases or disorders.The EHA Roadmap identifies priorities and needs across the field of hematology, including those to develop targeted therapies based on genomic profiling and chemical biology, to eradicate minimal residual malignant disease, and to develop cellular immunotherapies, combination treatments, gene therapies, hematopoietic stem cell treatments, and treatments that are better tolerated by elderly patients.

An assessment of methods used in the investigation of iron status: findings in a population of young British South Asian children.

AIMS: To assess the value of laboratory tests available for the investigation of iron status in a population of young British South Asian children. METHODS: Blood count, red cell distribution width (RDW), percentage hypochromic red cells (%hypo), concentrations of C-reactive protein (CRP), zinc protoporphyrin (ZPP), ferritin, soluble transferrin receptor, plasma iron measurements and incidence of deletional forms of α-thalassaemia were determined. RESULTS: Haemoglobin, mean cell haemoglobin (MCH), ferritin and CRP values classified iron status in 151/205 (73.6%) consecutive children aged 4-43 months. Fifty-four could not be classified: 12 were anaemic with findings, other than normal CRP values, indistinguishable from those with anaemia of inflammation and 42 were non-anaemic with reduced MCH values. All 42 had normal ferritin concentration and 8 of 36 successfully tested had deletional α-thalassaemia trait. Despite apparent iron sufficiency the RDW, %hypo and ZPP values of these 42 were not significantly different from the 32 children classified with iron-deficient erythropoiesis. The gene frequency of deletional α-thalassaemia trait in the entire group was 8.6%. CONCLUSIONS: Among 205 British South Asian children aged 4-43 months with high incidences of anaemia, iron deficiency, infection and α-thalassaemia, 151 (73.6%) were classified using haemoglobin, MCH, ferritin and CRP values. In 42 non-anaemic, iron-sufficient children with subnormal MCH values, that is with a phenotype of α-thalassaemia trait, RDW, %hypo and ZPP values did not differ significantly from those with iron-deficient erythropoiesis. Raised RDW, %hypo and ZPP values should be interpreted with caution in non-anaemic young British South Asian children with microcytosis.

[The ambivalence of progress - do we need a less-is-more strategy?**. **Extract from the programmtic opening speech delivered at the 59th Annual GTH Congress 2015]. / Die Ambivalenz des Fortschritts - ist weniger mehr?*. *Auszug aus der programmatischen Eröffnungsansprache zum 59. Jahreskongress der GTH 2015.

Economizing health care systems has led to industrialized hospitals. The consequences and side effects are substantial and rather promote costs explosion due to the dictated increase in cases and efficiency. Hence, position sensing and change in direction are required. Effective ways to find out of the current crisis are discussed.

Hematological Assessment in Pet Rabbits: Blood Sample Collection and Blood Cell Identification.

Pet rabbits are presented to veterinary clinics for routine care and treatment of clinical diseases. In addition to obtaining clinical history, additional diagnostic testing may be required, including hematological assessments. This article describes common blood collection methods, including venipuncture sites, volume of blood that can be safely collected, and handling of the blood. Hematological parameters for normal rabbits are provided for comparison with in-house or commercial test results. A description of the morphology of rabbit leukocytes is provided to assist in performing a differential count. Differential diagnoses are provided for abnormal values identified in the hemogram.

Hematologic Assessment in Pet Rats, Mice, Hamsters, and Gerbils: Blood Sample Collection and Blood Cell Identification.

Hamsters, gerbils, rats, and mice are presented to veterinary clinics and hospitals for prophylactic care and treatment of clinical signs of disease. Physical examination, history, and husbandry practice information can be supplemented greatly by assessment of hematologic parameters. As a resource for veterinarians and their technicians, this article describes the methods for collection of blood, identification of blood cells, and interpretation of the hemogram in mice, rats, gerbils, and hamsters.

Hematological Assessment in Pet Guinea Pigs (Cavia porcellus): Blood Sample Collection and Blood Cell Identification.

Pet guinea pigs are presented to veterinary clinics for routine care and treatment of clinical diseases. In addition to obtaining clinical history and exam findings, diagnostic testing may be required, including hematological assessments. This article describes common blood collection methods, including venipuncture sites, the volume of blood that can be safely collected, and handling of the blood. Hematological parameters for normal guinea pigs are provided for comparison with in-house or commercial test results. A description of the morphology of guinea pig leukocytes is provided to assist in performing a differential count.

Bridging the Distance in the Caribbean: Telemedicine as a means to build capacity for care in paediatric cancer and blood disorders.

Over the past 50 years, survival for children in high-income countries has increased from 30% to over 80%, compared to 10-30% in low and middle income countries (LMIC). Given this gap in survival, established paediatric cancer treatment centres, such as The Hospital for Sick Children (SickKids) are well positioned to share clinical expertise. Through the SickKids Centre for Global Child Health, the SickKids-Caribbean Initiative (SCI) was launched in March 2013 to improve the outcomes and quality of life for children with cancer and blood disorders in the Caribbean. The six participating Caribbean countries are among those defined by the United Nations as Small Island Developing States, due to their small size, remote location and limited accessibility. Telemedicine presents an opportunity to increase their accessibility to health care services and has been used by SCI to facilitate two series of interprofessional rounds. Case Consultation Review Rounds are a forum for learning about diagnostic work-up, management challenges and treatment recommendations for these diseases. To date, 54 cases have been reviewed by SickKids staff, of which 35 have been presented in monthly rounds. Patient Care Education Rounds provide nurses and other staff with the knowledge base needed to safely care for children and adolescents receiving treatment. Five of these rounds have taken place to date, with over 200 attendees. Utilized by SCI for both clinical and non-clinical meetings, telemedicine has enhanced opportunities for collaboration within the Caribbean region. By building capacity and nurturing expert knowledge through education, SCI hopes to contribute to closing the gap in childhood survival between high and low-resource settings.

Immunologic assessment and KMT2D mutation detection in Kabuki syndrome.

Kabuki or Niikawa-Kuroki syndrome (KS) is a rare disorder with multiple malformations and recurrent infections, especially otitis media. This study aimed to investigate the genetic defects in Kabuki syndrome and determine if immune status is related to recurrent otitis media. Fourteen patients from 12 unrelated families were enrolled in the 9-year study period (2005-2013). All had Kabuki faces, cleft palate, developmental delay, mental retardation, and the short fifth finger. Recurrent otitis media (12/14) and hearing impairment (8/14) were also more common features. Immunologic analysis revealed lower memory CD19+ cells (11/13), lower memory CD4+ cells (8/13), undetectable anti-HBs antibodies (7/13), and antibody deficiency (7/13), including lower IgA (4), IgG (2), and IgG2 (1). Naïve emigrant lymphocytes, lymphocyte proliferation function, complement activity, and superoxide production in polymorphonuclear cells were all normal. All the patients had KMT2D mutations and 10 novel mutations of R1252X, R1757X,Y1998C, P2550R fs2604X, Q4013X, G5379X, E5425K, R5432X, R5432W, and R5500W. Resembling the phenotype of common variable immunodeficiency, KS patients with antibody deficiency, decreased memory cells, and poor vaccine response increased susceptibility to recurrent otitis media. Large-scale prospective studies are warranted to determine if regular immunoglobulin supplementation decreases the frequency of otitis media and severity of hearing impairment.

[MRI for assessment of iron overload in patients with hematologic disease].

OBJECTIVE: Compare the characteristics of magnetic resonance imaging（MRI）liver T2*, cardiac T2* and serum ferritin on the assessment of iron overload. METHODS: A total of sixty-nine patients from November 2011 to June 2014 were enrolled in this study. Their cardiac and liver iron concentration levels were measured through MRI examination, with other clinical data were collected to perform statistical analysis. RESULTS: The correlation between liver T2* and adjusted serum ferritin（ASF） was statistically significant（P=0.003）. However, no significant correlation was found between cardiac T2* and liver T2*, ASF, respectively. According to the statistical analysis of the 69 cases, it is found that the number of iron overload cases diagnosed by liver T2* was 62 and 20 cases were severe iron overload （32.26%）; the number of iron overload cases diagnosed by ASF was 47 and 14 cases were severe iron overload（29.79%）, while the number of iron overload cases diagnosed by cardiac T2* was only 25 and no severe iron overload cases. CONCLUSION: Since SF was affected by other factors, it cannot reflect the level of iron overload in human body objectively. Now, liver T2* has become the gold standard for assessment of iron overload because of its good reliability and repeatability. However, cardiac T2* cannot correctly be used as assessment for iron overload, and it is only a method of evaluating the level of cardiac iron deposition.

Low cost-effectiveness of CD3/CD20 immunostains for initial triage of lymphoid-rich effusions: an evidence-based review of the utility of these stains in selecting cases for full hematopathologic workup.

CD3/CD20 immunostains are often performed in the initial cytological evaluation of lymphoid-rich pleural effusions (LR-PE). Most benign LR-PE are predominantly composed of T(CD3+) cells while most malignant LR-PE are of B(CD20+) cell lineage. As part of the effort to contain laboratory costs and improve diagnostic accuracy, there is increasing interest in applying principles of evidence-based pathology to the use of immunostains. In this retrospective study, we reviewed the effectiveness of CD3/CD20 immunostains as a diagnostic or triage tool during the initial evaluation of 258 consecutive LR-PE. 196 (76%) of the LR-PE were ultimately diagnosed as reactive lymphocytosis and 62 (24%) as lymphoma/leukemia (L/L). There was a previous diagnosis of L/L, concurrent diagnostic tissue, and/or clinical evidence of L/L in 44 (71%) of the L/L effusions. An initial diagnosis of L/L was made in the remaining 18 (29%) cases. Sixteen of these 18 cases showed large cells with high-grade features that mandated L/L workup. In only 2 (0.8%) of the 258 LR-PE, CD3/CD20 stains were helpful to identify small cell lymphocytic lymphoma (SLL) in patients without concurrent peripheral lymphocytosis. CD3/CD20 immunostains do not appear to provide a cost-effective method to diagnose or triage the vast majority of LR-PE submitted to a clinical cytology laboratory. An algorithm that considers history, blood counts, and cytomorphology allows for cost-effective selection of LR-PE that warrant comprehensive hematopathologic workup. Our findings underscore the feasibility of applying evidence-based principles to develop guidelines for the cost-effective utilization of immunostains in cytology.

Approach to peripheral blood film assessment for pathologists.

In today's age of advanced technology used in the diagnosis of many medical problems, the blood film stands out as an inexpensive and quick diagnostic tool that can generally be performed in most laboratories. Understanding when to perform a blood film and how it is made and having an organized sequential approach to reviewing the blood film are critical in the efficient use of this ubiquitous laboratory investigation. This article will review these issues, as well as common morphologic abnormalities found on blood films, and will discuss related clinical diagnoses that they represent. The final step of communicating the blood film findings in a clear and concise method to relay clinical suspicions to the end user will be discussed.

Whole blood reflectance for assessment of hematologic condition and detection of angiographic contrast media.

We present simple whole blood reflectance analyses in the range 500-900 nm, using intact whole blood to simultaneously quantify hematocrit and oxygen saturation from a single spectral reading. We applied these results for the development of an intravascular catheter-based reflectance sensing system to detect and remove contrast media injected during angiography so as to reduce the risk of complications associated with the injected contrast media. We further tested the practicality of the optical detection of angiographic contrast media in a pilot animal study in vivo. We successfully demonstrated the feasibility of real-time in vivo contrast detection and removal during angiography. Our simple method for the detection and removal of angiographic contrast media will facilitate the development of intravascular optical sensing systems.