Blood Product Transfusion in Adults: Indications, Adverse Reactions, and Modifications.

Millions of units of blood products are transfused annually to patients in the United States. Red blood cells are transfused to improve oxygen-carrying capacity in patients with or at high risk of developing symptomatic anemia. Restrictive transfusion thresholds with lower hemoglobin levels are typically clinically equivalent to more liberal thresholds. Transfusion of plasma corrects clinically significant coagulopathy in patients with or at high risk of bleeding. Mildly abnormal laboratory coagulation values are not predictive of clinical bleeding and should not be corrected with plasma. Transfused platelets prevent or treat bleeding in patients with thrombocytopenia or platelet dysfunction. Cryoprecipitate is transfused to treat hypofibrinogenemia. Many adverse reactions can occur during or after blood product transfusion. Transfusion-associated circulatory overload (i.e., volume overload) is the most common cause of mortality associated with blood products. Modifications to blood products can prevent or decrease the risks of transfusion-related adverse reactions. It is critical to quickly recognize when a reaction is occurring, stop the transfusion, assess, and support the patient. Reporting a reaction to the blood bank is part of ensuring patient safety and supporting hemovigilance efforts.

Emerging single-cell tools are primed to reveal functional and molecular heterogeneity in malignant hematopoietic stem cells.

PURPOSE OF REVIEW: The recent emergence of single-cell technologies has permitted unprecedented insight into the molecular drivers of fate choice in blood stem and progenitor cells. This review gives a broad overview of current efforts to understand the molecular regulators of malignant hematopoietic stem cells (HSCs) at the single-cell level. RECENT FINDINGS: The large-scale adoption of single-cell approaches has allowed extensive description of the transcriptional profiles and functional properties of single HSCs. These techniques are now beginning to be applied to malignant HSCs isolated directly from patients or from mouse models of malignancy. However, these studies have generally struggled to pinpoint the functional regulators of malignant characteristics, since malignant HSCs often differ in more than one property when compared with normal HSCs. Moreover, both normal and malignant populations are complicated by HSC heterogeneity. SUMMARY: Despite the existence of single-cell gene expression profiling tools, relatively few publications have emerged. Here, we review these studies from recent years with a specific focus on those undertaking single-cell measurements in malignant stem and progenitor cells. We anticipate this to be the tip of the iceberg, expecting the next 2-3 years to produce datasets that will facilitate a much broader understanding of malignant HSCs.

[Hemolytic disorders and venous thrombosis: An update]. / Maladies hémolytiques et thrombose veineuse : mise au point.

Many factors can contribute to the risk of venous thrombosis observed in hemolytic diseases. Some mechanisms are related to hemolysis by itself, while others seem more specific to each disease. Despite recent advances in the quantification of this risk and in understanding its physiopathology, the association of hemolysis with venous thrombosis is often unknown. The purpose of this general review is to clarify the main pro-thrombotic mechanisms during hemolysis and to synthesize the clinical data currently available. We will focus on the main types of hemolytic pathologies encountered in current practice, namely paroxysmal nocturnal hemoglobinuria, hemoglobinopathies, auto-immune hemolytic anemia and thrombotic microangiopathies.

Human exposure to mercury and its hematological effects: a systematic review.

Mercury is a metal found in the environment from natural and anthropogenic sources. It is highly toxic to ecosystems and living beings. Most human exposures come from ingestion of contaminated seafood, outgassing from dental amalgam or occupational exposure (e.g. gold mining), among other cases. Large populations are exposed to mercury, making it a very important issue from the public health perspective. Adverse health effects are commonly seen in the nervous system, but every organ is a potential target, such as the bone marrow. The main goal of this study was to assess the available evidence on human exposure to mercury and its hematological effects. A search strategy was constructed, including key terms (MeSH, text word and equivalents) for querying 2 repositories of master dissertation and PhD thesis (Fiocruz/ARCA and University of São Paulo) and 4 different electronic databases: BVS/LILACS, MEDLINE/PubMed, Scopus and TOXLINE/NIH, for articles published from 1950 to February 2018. There was no language restriction and a tool (EPHPP) was used to assess the quality of included studies. According to pre-established criteria, 80 studies were retrieved, all of them observational (48 case reports, 24 cross-sectional, 6 case series and 2 cohorts), comprising 9,284 people. Despite the fact that most exposed ones (6,012) had normal blood cell count and mercury hematological effects did not seem very usual (1,914 cases: 14 severe and 29 deaths), three studies reported association (ß) for anemia, lymphopenia, neutrophilia and basophilia. We concluded that the gathered information pointed to mercury hematotoxic effects, some of them may be serious and even fatal.

Retinopatía vasoproliferativa secundaria a enfermedad de Waldenström / Vasoproliferative retinopathy secondary to Waldenström's disease

Paciente de 66 años en seguimiento por retinopatía diabética refractaria a múltiples modalidades de tratamiento a pesar del buen control metabólico que refiere pérdida de peso progresiva. Por este motivo se decide realizar un estudio sistémico, detectándose anemia, elevación de la velocidad de sedimentación globular e hiperproteinemia a expensas de un pico monoclonal de IgM. Posteriormente, mediante la biopsia de médula ósea y el estudio genético, se llega al diagnóstico de macroglobulinemia de Waldenström. La macroglobulinemia de Waldenström es una patología linfoproliferativa de escasa frecuencia cuya principal manifestación es a través del síndrome de hiperviscosidad. Este puede producir signos oftalmológicos detectables mediante funduscopia y pruebas de imagen. El estudio multimodal es útil en el diagnóstico y seguimiento de la afectación retiniana. La incorporación de la angiografía por tomografía de coherencia óptica permite un estudio más preciso de los trastornos microvasculares que se pueden presentar a nivel del polo posterior

A 66 year-old patient, monitored for diabetic retinopathy refractory to multiple treatment methods despite a good metabolic control, referred to progressive weight loss. For this reason, a systemic study was performed, detecting anaemia, elevation of the erythrocyte sedimentation rate, and hyperproteinaemia due to elevated serum levels of monoclonal IgM. Subsequently, by performing a bone marrow biopsy and genetic study, the diagnosis of Waldenström macroglobulinaemia was made. Waldenström's macroglobulinaemia is a low frequency lymphoproliferative disease, for which the main manifestation is a hyperviscosity syndrome that can produce ophthalmological signs detectable by funduscopy and imaging tests. A multimodal study is useful in the diagnosis and monitoring of retinal involvement. The incorporation of angiography by optical coherence tomography allows a more precise study of the microvascular disorders that may occur at the posterior pole level

Human exposure to mercury and its hematological effects: a systematic review / Exposição humana ao mercúrio e seus efeitos hematológicos: uma revisão sistemática / Exposición humana al mercurio y sus efectos hematológicos: una revisión sistemática

Mercury is a metal found in the environment from natural and anthropogenic sources. It is highly toxic to ecosystems and living beings. Most human exposures come from ingestion of contaminated seafood, outgassing from dental amalgam or occupational exposure (e.g. gold mining), among other cases. Large populations are exposed to mercury, making it a very important issue from the public health perspective. Adverse health effects are commonly seen in the nervous system, but every organ is a potential target, such as the bone marrow. The main goal of this study was to assess the available evidence on human exposure to mercury and its hematological effects. A search strategy was constructed, including key terms (MeSH, text word and equivalents) for querying 2 repositories of master dissertation and PhD thesis (Fiocruz/ARCA and University of São Paulo) and 4 different electronic databases: BVS/LILACS, MEDLINE/PubMed, Scopus and TOXLINE/NIH, for articles published from 1950 to February 2018. There was no language restriction and a tool (EPHPP) was used to assess the quality of included studies. According to pre-established criteria, 80 studies were retrieved, all of them observational (48 case reports, 24 cross-sectional, 6 case series and 2 cohorts), comprising 9,284 people. Despite the fact that most exposed ones (6,012) had normal blood cell count and mercury hematological effects did not seem very usual (1,914 cases: 14 severe and 29 deaths), three studies reported association (β) for anemia, lymphopenia, neutrophilia and basophilia. We concluded that the gathered information pointed to mercury hematotoxic effects, some of them may be serious and even fatal.

O mercúrio é um metal que pode ser encontrado naturalmente no meio ambiente e através de fontes antropogênicas. É altamente tóxico para ecossistemas e seres vivos. A maior parte da exposição humana provém da ingestão de pescados contaminados, da liberação de gases da amálgama dentária ou da exposição ocupacional (p.ex.: extração de ouro). Vastas populações são expostas ao mercúrio, tornando-se uma questão de saúde pública muito importante. Efeitos adversos à saúde são comumente observados no sistema nervoso, mas todos os órgãos são alvos em potencial, como a medula óssea. O principal objetivo do estudo foi avaliar as evidências disponíveis sobre a exposição humana ao mercúrio e seus efeitos hematológicos. Uma estratégia de busca foi realizada, incluindo termos chave (palavras-chave, palavras do texto e equivalentes), para pesquisar dois repositórios de dissertações de mestrado e teses de doutorado (Fiocruz/ARCA e Universidade de São Paulo) e quatro bases de dados eletrônicas: BVS/LILACS, MEDLINE/PubMed, Scopus e TOXLINE/NIH (artigos publicados de 1950 até fevereiro de 2018). Não houve restrições de linguagem e uma ferramenta (EPHPP) foi utilizada para avaliar a qualidade dos estudos incluídos. De acordo com os critérios pré-estabelecidos, foram encontrados 80 estudos, todos observacionais (48 relatos de caso, 24 estudos transversais, 6 séries de casos e 2 coortes), que compreendiam 9.284 pessoas. Apesar do fato de que as pessoas mais expostas (6.012) tinham contagens de células sanguíneas normais, e os efeitos hematológicos do mercúrio não pareciam muito comuns (1.914 casos, 14 graves e 29 mortes), três estudos relataram a associação de (β) anemia, linfopenia, neutrofilia e basofilia. Concluímos que as informações coletadas indicam efeitos hematotóxicos do mercúrio, alguns dos quais podem ser muito graves e até fatais.

El mercurio es un metal que se puede encontrar de forma natural en el ambiente y mediante fuentes antropogénicas. Es altamente tóxico para los ecosistemas y seres vivos. Entre otras, la mayor parte de la exposición humana, proviene de la ingestión de pescado contaminado, liberación de gases de amalgamas dentales o exposición ocupacional (p.ej. extracción de oro). Vastas poblaciones están expuestas al mercurio, convirtiéndolo en un asunto muy importante desde la perspectiva de la salud pública. Los efectos adversos para la salud se observan comúnmente en el sistema nervioso, pero cada órgano es un objetivo potencial, como la médula ósea. El objetivo principal del estudio fue evaluar las evidencias disponibles sobre la exposición humana al mercurio y sus efectos hematológicos. Se realizó una estrategia de búsqueda, incluyendo términos clave (palabras-clave, palabras del texto y equivalentes), se consultaron 2 registros de trabajos finales de máster y tesis de doctorado (Fiocruz/ARCA y Universidad de São Paulo) y 4 bases de datos electrónicas diferentes: BVS/LILACS, MEDLINE/PubMed, Scopus y TOXLINE/NIH, para artículos publicados desde el año 1950, hasta febrero de 2018. No hubo restricciones de lengua y se usó la herramienta (EPHPP) para evaluar la calidad de los estudios incluidos. De acuerdo con los criterios preestablecidos, se recopilaron 80 estudios, todos observacionales (48 informes de casos, 24 estudios transversales, 6 series de casos, y 2 cohortes), que comprendieron a 9.284 personas. A pesar de que la mayoría de los expuestos (6.012) tenían un recuento normal de células sanguíneas y los efectos hematológicos del mercurio no parecían muy comunes (1.914 casos: 14 severos y 29 muertes), tres estudios informaron de la asociación (β) para anemia, linfopenia, neutrofilia y basofilia. Concluimos que la información recabada indicaba los efectos hematotóxicos del mercurio, algunos de los cuales pueden ser muy serios e incluso fatales.

Adherence to iron chelation therapy in patients who switched from deferasirox dispersible tablets to deferasirox film-coated tablets.

OBJECTIVE: To compare real-world adherence to and persistence with deferasirox film-coated tablets (DFX-FCT) and deferasirox dispersible tablets (DFX-DT) among patients who switched from DFX-DT to DFX-FCT, overall and by disease type (sickle cell disease [SCD], thalassemia, and myelodysplastic syndrome [MDS]). METHODS: Patients were ≥2 years old and had ≥2 DFX-FCT claims over the study period and ≥2 DFX-DT claims before the index date (first DFX-FCT claim). The DFX-DT period was defined from the first DFX-DT claim to the index date; the DFX-FCT period was defined from the index date to the end of the study period. Adherence was measured as medication possession ratio (MPR) and proportion of days covered (PDC). Persistence was defined as continuous medication use without a gap ≥30 or 60 days between refills. Comparisons were conducted using paired-sample Wilcoxon sign-rank and McNemar's tests. RESULTS: In total, 606 patients were selected (SCD: 348; thalassemia: 107; MDS: 106; other: 45). Adherence and persistence in the DFX-FCT vs DFX-DT period was significantly higher across all measures: mean MPR was 0.80 vs 0.76 (p < .001); 60.9% vs 54.3% of patients had MPR ≥ 0.8 (p = .009); mean 3-month PDC was 0.83 vs 0.71 (p < .001); 64.2% vs 45.4% of patients had 3-month PDC ≥ 0.8 (p < .001); 87.2% vs 63.4% of patients had 3-month persistence with no gap ≥30 days and 96.1% vs 79.9% with no gap ≥60 days (p < .001). Adherence and persistence improved after switching across all diseases, particularly MDS. CONCLUSIONS: Adherence and persistence improved significantly after switching from DFX-DT to DFX-FCT for all diseases, but especially MDS.

Resposta hematológica do cascudo ornamental amazônico Peckoltia oligospila ao estresse de transporte / Hematological response of Amazonian ornamental Peckoltia oligospila subjected to stress by transportation

O objetivo deste trabalho foi avaliar as respostas hematológicas do acari-bola Peckoltia oligospila submetido ao estresse de transporte. Variações nos parâmetros de sangue foram analisadas às zero, seis, 24, 48, 72 e 96 horas após o transporte. Respostas ao estresse foram observadas entre zero e seis horas do transporte, mas a maioria dos parâmetros retornou aos valores basais em 24 horas. O tempo de zero hora (momento imediato após transporte) foi o mais crítico, com valores elevados de glicemia, eritrócitos e eritroblastos. Respostas secundárias tardias foram observadas para a proteína plasmática total, o volume corpuscular médio (VCM) e a hemoglobina corpuscular média (HCM) em seis horas após o transporte dos peixes, retornando aos valores basais após esse período. O número de leucócitos não sofreu alterações após o transporte. O estresse de transporte não comprometeu a fisiologia de P. oligospila, o que indica que esse peixe é resistente ao estresse se comparado com outras espécies. Porém, recomenda-se que não se realize qualquer outro procedimento estressante durante pelo menos 24 horas da recuperação dos peixes após transporte, para garantir a saúde e a sobrevivência dos animais transportados.(AU)

The objective of this work was to evaluate the hematological responses of bola-pleco (Peckoltia oligospila) undergoing the stress of transportation. Variations on blood parameters were analyzed at 0, 6, 24, 48, 72 and 96h after transportation. Responses to stress were detected from 0 to 6h after the transportation of fish, however, most parameters returned to baselines values within 24h of transportation. The moment of 0h was the most critical, presenting higher values of glycemia, erythroblasts and erythrocytes. Late secondary responses were observed to total plasmatic protein, mean corpuscular volume (MCV) and mean corpuscular hemoglobin (MCH) at 6h, returning to baselines values after this time. Leukocyte number was not affected by stress of transportation. The stress by transportation was not severe to influence the health of P. oligospila, indicating that fish is resistant to stress if compared to other species. However, we recommended no stressful procedures for at least 24 hours for recovery, in order to ensure health and survival of fish.(AU)

Revised medical criteria for evaluating hematological disorders. Final rules.

We are revising the criteria in the Listing of Impairments (listings) that we use to evaluate cases involving hematological disorders in adults and children under titles II and XVI of the Social Security Act (Act). These revisions reflect our adjudicative experience, advances in medical knowledge, diagnosis, and treatment, and public comments we received in response to a Notice of Proposed Rulemaking (NPRM).

Extramedullary hemopoiesis.

Various chronic hematologic disorders that lead to ineffective hemopoiesis or inadequate bone marrow function (ie, chronic hemolytic anemias, thalassemia, sickle cell anemia, myelofibrosis of many causes, lymphoma, and leukemia) can potentially precipitate extramarrow new blood element creation. Extramarrow soft tissue that produces blood elements is called extramedullary hemopoietic tissue and the process extramedullary hemopoiesis (EMH). Sites commonly involved by EMH include the liver, spleen, lymph nodes, and most commonly, paravertebral regions, although other sites can sometimes be involved. Physicians rarely consider EMH in their differential diagnosis even in cases where it is warranted (diseases of ineffective erythropoiesis). This is likely because of the rarity of the condition and because imaging findings are nonspecific. We present here a systematic review of the imaging findings in EMH.

How we developed and use the American Society for Apheresis guidelines for therapeutic apheresis procedures.

The decision to treat a patient with therapeutic apheresis depends on multiple factors, such as what does the patient most likely have, is the diagnosis amenable to apheresis treatment, what is the harm-versus-benefit ratio of apheresis treatment in this patient, and what are the internal and external issues associated with receiving apheresis treatment? The "Guidelines on the Use of Therapeutic Apheresis in Clinical Practice-Evidence-based Approach from the Writing Committee of the American Society for Apheresis" addresses these issues and helps aid in clinical decision making. The development and application of these Guidelines as well as potential new applications will be discussed in this article.

Impact of illness per ICD-9 classification on progression to physical evaluation board for U.S. marines placed on limited duty status 2006-2010.

Mission readiness is of paramount importance to Marine Corps commanders. Personnel medically unable to perform at full capacity negatively affect a unit's readiness and ability to accomplish mission objectives. A retrospective cohort study was designed to evaluate the impact of diseases and conditions per International Statistical Classification of Diseases and Related Health Problems, Revision 9 (ICD-9) classification of primary diagnosis on likelihood of progressing to Physical Evaluation Board (PEB) for U.S. Marines placed on Limited Duty. A total of 30,937 records belonging to 19,042 unique individuals were identified in the Medical Board Online Tri-Service Tracking System database over the study surveillance period. Approximately half (9,133) of all Marines placed on Limited Duty were eventually referred to PEB. After multivariate adjustment Marines with a primary ICD-9 diagnosis indicating a blood disorder (OR = 4.1, 95% CI 2.1-8.1) or nervous system disorder (OR = 3.3, 95% CI 2.8- 3.8) were at greater risk of progressing to PEB as compared to the lowest risk group, Marines with an orthopedic diagnosis. Occupational category, rank grouping, race/ethnicity, and ICD-9 category had statistically significant impacts of varying magnitude on the risk of progressing to PEB; no significant difference was found for sex.

Classification of and risk factors for hematologic complications in a French national cohort of 102 patients with Shwachman-Diamond syndrome.

BACKGROUND: Patients with the Shwachman-Diamond syndrome often develop hematologic complications. No risk factors for these complications have so far been identified. The aim of this study was to classify the hematologic complications occurring in patients with Shwachman-Diamond syndrome and to investigate the risk factors for these complications. DESIGN AND METHODS: One hundred and two patients with Shwachman-Diamond syndrome, with a median follow-up of 11.6 years, were studied. Major hematologic complications were considered in the case of definitive severe cytopenia (i.e. anemia <7 g/dL or thrombocytopenia <20 × 10(9)/L), classified as malignant (myelodysplasia/leukemia) according to the 2008 World Health Organization classification or as non-malignant. RESULTS: Severe cytopenia was observed in 21 patients and classified as malignant severe cytopenia (n=9), non-malignant severe cytopenia (n=9) and malignant severe cytopenia preceded by non-malignant severe cytopenia (n=3). The 20-year cumulative risk of severe cytopenia was 24.3% (95% confidence interval: 15.3%-38.5%). Young age at first symptoms (<3 months) and low hematologic parameters both at diagnosis of the disease and during the follow-up were associated with severe hematologic complications (P<0.001). Fifteen novel SBDS mutations were identified. Genotype analysis showed no discernible prognostic value. CONCLUSIONS Patients with Shwachman-Diamond syndrome with very early symptoms or cytopenia at diagnosis (even mild anemia or thrombocytopenia) should be considered at a high risk of severe hematologic complications, malignant or non-malignant. Transient severe cytopenia or an indolent cytogenetic clone had no deleterious value.

Cutaneous hematologic disorders in children.

BACKGROUND: To determine and list the clinical and pathological features of cutaneous hematologic diseases in childhood. PROCEDURE: We retrospectively analyzed the data for 51 patients up to 15 years of age, who presented with primary cutaneous hematologic disorders according to the WHO-EORTC classification, at Necker-Enfants Malades Hospital, Paris, France, over a 17-year period. The cases were classified into the following diagnostic categories: CD30+ T-cell lymphoproliferative disorders (24) all consisting of lymphomatoid papulosis (LyP, 24), lymphoblastic lymphoma (LL, 7), acute leukemias (AL, 7), mycosis fungoides (MF, 5), Epstein-Barr virus-related lymphoproliferative disorders (EBV-related LPD, 5), T/NK-cell lymphoma, nasal type (1), γ/δ T-cell lymphoma (1), and panniculitis-like T-cell lymphoma (1). RESULTS: We encountered a majority of LyP. No secondary lymphoma was found in LyP patients with a median follow-up of 8 years. 29% and 80% of LyP and MF patients, respectively, presented with pityriasis lichenoides chronica (PLC) before onset of disease. Owing to a frequently misleading clinicopathological presentation, MF patients were first underdiagnosed. Clinicopathological features of LL and AL were highly stereotypical, leading to the diagnosis being suspected and confirmed more promptly. In the latter patients and in EBV-related LPD patients, skin lesions usually led to the discovery of systemic disease. CONCLUSION: Distribution of cutaneous hematologic diseases seems to be different in adults and in children aged <15-year old. PLC was strongly correlated with MF and LyP. Physicians must be made aware of the stereotypical clinical presentations of LL and AL to allow prompt diagnosis and treatment.