Hasil untuk "Diseases of the blood and blood-forming organs"

G.G. Loscocco, F. Mannelli, M. Piccini et al.

Background: Genomic abnormalities associated with extramedullary (EM) acute myeloid leukemia (eAML) and their impact on clinical outcomes are not well understood since previous studies were limited to small case series with controversial results. Aim and methods: A large monocentric cohort of 600 newly diagnosed AML patients (pts) at the University of Florence (Careggi Hospital), comparing those with and without concurrent EM involvement, was analyzed. Results: 494 (82%) pts were treated with intensive chemotherapy (cht), and 243 (41%) with allogenic hematopoietic stem cell transplantation (AHSCT). Out of 539 evaluable pts, 245 (46%) had abnormal karyotype, 71 (13%) had a complex karyotype. 140 (23%) NPM1, 126 (21%) FLT3-ITD, 27 (5%) inv (16), 26 (4%) CEBPA bZIP, 24 (4%) t(8;21). Of the whole cohort, 103 pts (17%) had eAML, 81% and 19% (n=119) with de novo and sAML, respectively. eAML were younger (56 vs 58 years, p=0.01), with higher white blood cell (p< 0.01) and peripheral blast count (p= 0.02). ELN '22 favorable risk was less common in eAML (18% vs 31%, p=0.01), while the intermediate category was more frequent (42% vs 32%, p=0.03). FLT3-ITD (31% vs 19%, p<0.01) and TP53 mutations (22% vs 10%, p=0.04) were also more common in eAML. Median overall survival (OS) of the cohort was 20.2 months, significantly shortened in eAML (11 vs 23.5 months, p<0.01) (Fig. 1A). Of note, having more than 1 EM localization was associated with furtherly shortened OS (8.5 months, HR 2; p<0.01) (Fig. 1B). In multivariate analysis, eAML (HR 1.4; p=0.02), older age (p<0.01), and ELN '22 risk categorization (p<0.01) were significant for reduced OS. Considering eAML pts only, ELN '22 failed to resolve intermediate and adverse categories (p=0.4). Intensive cht and AHSCT were associated with superior survival in pts with and without eAML. For eAML, SRSF2 (HR 3; p=0.03), SETBP1 (HR 10; p=0.04), and JAK2 (HR 4.9; p=0.04) mutations were associated with reduced OS. When analyzing ELN '22 risk categories separately for pts with and without eAML, a significant different OS for intermediate risk pts only was documented (HR 1.6; p=0.02, Fig. 1C). Conclusions: The study establishes key molecular markers associated with EM, such as FLT3-ITD and TP53 mutations. Of interest, ELN '22 categorization lost the power to discriminate between intermediate and adverse category. Finally, intensive cht and AHSCT improved OS in pts with EM involvement.  

Petar Kalaydzhiev, Tsvetelina Velikova, Yanitsa Davidkova et al.

<b>Background</b>. Type 2 diabetes mellitus (T2D) and moderate-to-severe obstructive sleep apnea (OSA) commonly coexist and exacerbate poor glycemic control, systemic inflammation, and diminished quality of life (QoL). Although continuous positive airway pressure (CPAP) therapy has demonstrated metabolic and anti-inflammatory benefits, its real-world impact in Bulgarian outpatient settings—where CPAP costs are borne entirely by patients—has not been characterized. <b>Objectives</b>. To evaluate the effects of six months of CPAP therapy on glycemic control (hemoglobin A₁c [HbA₁c]), systemic inflammation (high-sensitivity C-reactive protein [hsCRP]), body mass index (BMI), lipid profile (low-density lipoprotein [LDL]), QoL (Short Form 36 Physical Component Summary [SF-36 PCS] and Mental Component Summary [SF-36 MCS]), and survival among Bulgarian outpatients with T2D and moderate-to-severe OSA. <b>Methods</b>. In this prospective, multicenter cohort study conducted from January 2022 to July 2023, 142 adults with established T2D and OSA (apnea–hypopnea index [AHI] ≥ 15) were enrolled at three outpatient centers in Bulgaria. Fifty-five patients elected to purchase and use home-based CPAP (intervention group), while 87 declined CPAP—either because of cost or personal preference—and continued standard medical care without CPAP (control group). All participants underwent thorough outpatient evaluations at baseline (month 0) and at six months, including measurement of HbA₁c, hsCRP, BMI, fasting lipid profile (LDL), and patient-reported QoL, via the SF-36 Health Survey. Survival was tracked throughout follow-up. <b>Results</b>. After six months, the CPAP group experienced a significant reduction in HbA₁c from a median of 8.2% (IQR 7.5–9.5%) to 7.7% (6.7–8.7%), <i>p</i> < 0.001, whereas the control group’s HbA₁c decreased modestly from a median of 8.6% (IQR 7.9–9.4%) to 8.3% (7.6–9.1%); <i>p</i> < 0.001), with a significant between-group difference at follow-up (<i>p</i> = 0.005). High-sensitivity CRP in the CPAP arm fell from a median of 2.34 mg/L (IQR 1.81–3.41) to 1.45 mg/L (IQR 1.25–2.20), <i>p</i> < 0.001, while remaining unchanged in controls (<i>p</i> = 0.847). BMI in the CPAP group declined significantly from 28.6 kg/m², IQR 26.6–30.6 to 28 kg/m², IQR 25.6–29.2 (<i>p</i> < 0.001), compared to no significant change in controls (median 28.9 kg/m²), <i>p</i> = 0.599. LDL decreased in the CPAP group from a median of 3.60 mmol/L (IQR 3.03–3.89) to 3.22 mmol/L (IQR 2.68–3.48), <i>p</i> < 0.001, with no significant reduction in controls (<i>p</i> = 0.843). Within the CPAP arm, both SF-36 PCS and SF-36 MCS scores improved significantly from baseline (<i>p</i> < 0.001 for each), although between-group differences at six months did not reach statistical significance (PCS: 48 ± 10 vs. 46 ± 9, <i>p</i> = 0.098; MCS: 46, IQR 40–54 vs. 46, IQR 39–53, <i>p</i> = 0.291). All-cause mortality during follow-up included 2 events in the CPAP group and 11 events in the control group (log-rank <i>p</i> = 0.071). <b>Conclusions</b>. In Bulgarian outpatients with T2D and moderate-to-severe OSA, six months of CPAP therapy significantly improved glycemic control, reduced systemic inflammation, lowered BMI and LDL, and enhanced QoL, with a non-significant trend toward reduced mortality. These findings underscore the importance of integrating CPAP into multidisciplinary management despite financial barriers.

DL Queiroz, BR Campos, GE Dresch et al.

Introdução/Objetivo: A anemia hemolítica autoimune (AHAI) ocorre quando autoanticorpos se ligam à superfície dos eritrócitos, ocasionando sua destruição. Os anticorpos envolvidos podem ser da classe IgM, ocorrendo a hemólise via ativação do sistema complemento, ou podem ser da classe IgG, os quais são incapazes de aglutinar as hemácias e consequentemente a hemólise ocorre no sistema reticuloendotelial. Essa condição se classifica de acordo com sua etiologia, ou seja, primária (idiopática) ou secundária (associada a imunodeficiências, infecções virais, uso de medicamentos ou neoplasias). Dessa forma, o presente estudo teve por objetivo relatar um caso de um paciente que desenvolveu AHAI após ser internado e receber tratamento para pneumonia. Relato de caso: Homem, 74 anos, antecedente de Insuficiência Cardíaca (IC), refere que há cerca de dois meses iniciou com tosse seca, astenia e edema generalizado, recebendo tratamento para pneumonia e foi submetido a procedimento de cateterismo. Recebeu alta hospitalar em uso de medicamento para IC e Claritromicina. Após três semanas, evoluiu com febre, hipotensão, tosse e astenia, sendo tratado com Cefepime. Ao ser internado novamente, foi realizado o hemograma, no qual evidenciou-se anemia, com diminuição progressiva de hemoglobina, policromasia, macrocitose e CHCM elevado, outros exames evidenciaram reticulocitose, hiperbilirrubinemia às custas de bilirrubina indireta, hemoglobinúria, aumento da enzima lactato desidrogenase (LDH) e Coombs Direto positivo para IgG. Após suspeita de anemia hemolítica autoimune, foi iniciado corticoterapia com Metilprednisolona, ácido fólico e realizado transferência para hospital especializado, para tratamento adequado. Discussão: Diversos medicamentos utilizados na prática clínica podem causar efeitos colaterais graves, como a AHAI, sendo mais frequente por antibióticos, os quais destacam-se as beta-lactamases (principalmente cefalosporinas e penicilinas), de tal forma que induzem à produção de Imunoglobulinas G (IgG), que reagem com o complexo fármaco-membrana eritrocitária. Esse mecanismo resulta na remoção acelerada dos glóbulos vermelhos através de fagocitose por macrófagos, ocorrendo principalmente no baço. Conclusão: Sendo assim, o reconhecimento precoce da AHAI e sua etiologia é fundamental para a instituição de suporte intensivo necessário e um prognóstico favorável. Por fim, percebe-se o quão importante é o uso racional e controlado dos antibióticos, tendo em vista o efeito que tais fármacos podem ter sobre o sistema sanguíneo.

Birol Güvenç

In alignment with this vision, we are excited to introduce a new initiative: Z-Ematology. This concept is designed to bridge the gap between Generation Z and the evolving field of hematology. The new generation brings fresh perspectives and skills, especially in areas such as artificial intelligence, big data, telemedicine, and digital health technologies. Z-Ematology reflects our belief that adapting to these trends is essential for the future of hematology, and we are committed to integrating innovation into every aspect of the profession.

LCM Faial, CSG Faial, AN Norberg et al.

Objetivo: Descrever dois casos de anemia ferropriva como manifestação de doença celíaca. Material de método: Trata-se de um estudo descritivo baseado na análise de anamnese do paciente, exames complementares e revisão da literatura. Resultados: Primeiro caso EMP 71 anos, branca, casada, bancária aposentada, procedente de bom Jesus do Itabapoana, portadora fibrilação atrial, doença do refluxo gastroesofágico e síndrome mielodisplásica sem anemia, evolui com fraqueza, emagrecimento, esteatorreia, perda de gordura glútea, distensão abdominal e anemia normocítica normocrômica. Exame físico: palidez cutâneo mucosa ++/4+, queilite angular. Na endoscopia digestiva alta: hérnia de hiato por deslizamento, gastrite enantematosa moderada de corpo gástrico, discreta duodenite crônica inespecífica com redução vilositária e linfocitose intraepitelial. Solicitado cinética do ferro e anticorpos de rastreio de doença celíaca: anticorpo anti-transglutaminase tecidual IgA 67, IgG 1,5, anti-endomísio IgA 1:160, IgG não reagente, anti-gliadina IgA > 142 e IgG 25, ferritina 43,5 ng/mL e índice de saturação de transferrina 21%. Realizado diagnóstico de doença celíaca e ferropenia sintomática. Iniciado dieta sem o glúten e ferroterapia. Quatro meses após paciente ganhou 8 kg informou disposição para as atividades diárias, sem queixas gastrointestinais. Na nova cinética do ferro: índice de saturação de transferrina ascendeu para 27% e ferritina 45,3. Secundo caso SOB 23 anos, branca, solteira, nutricionista, natural e procedente de Bom Jesus do Itabapoana, portadora de hipotireoidismo por tireoidite de Hashimoto desde os 7 anos em uso de levotiroxina 162,5 mcg/dia, hipercolesterolemia. Informa queda de cabelo, desânimo e sonolência mesmo em suplementação de ferro há 4 meses. Uso de dispositivo intra-útero hormonal, fluxo menstrual normal, refere constipação e irritabilidade. História familiar: avó materna portadora de hipotireoidismo por tireoidite de Hashimoto e cirrose auto-imune. Exame físico: palidez cutâneo-mucosa +/+4 Hemograma: Hb 13,9 g/dL; Hct 40,7%; VCM 92,3 mm3 HCM 31,5 pg; CHCM 34,2g/dL; RDW 12,7%; Leucometria (0,9/3,9/0/0/0,5/43,1/43,9/7,7) plaquetas 256 400 mm3, ferritina 24 ng/mL. No rastreio de doença celíaca: anti-gliadina IgA 15, IgG 40; anti-transglutaminase tecidual IgA > 128, igG1,4; anti-endomísio IgA reagente 1:320, IgG não reagente. Endoscopia digestiva alta com biopsia de mucosa de duodeno em andamento, ajustado a reposição de ferro. Discussão: a doença celíaca é enteropatia imunomediada, crônica, do intestino delgado, precipitada pela ingestão de glúten na dieta. Apresenta fenótipo variado de assintomático a gravemente sintomática, a anemia ferropriva pode ser o único sinal de doença tanto em crianças quanto em adultos. Há o risco aumentado de desenvolver linfoma não hodking. O diagnóstico perpassa pela dosagem de anticorpos da classe IgA e IgG: anti-endomísio, anti-gliadina desaminase, anti-transglutaminase tecidual e biopsia da mucosa duodenal por endoscopia ou cápsula de sucção. A dieta restritiva ao glúten, melhora os sintomas intestinais, previne complicações autoimunes e garante sucesso da ferroterapia. Conclusão: A doença celíaca é um distúrbio intestinal crônico, imunomediado, com potencial morbimortalidade, cujo hematologista deve estar atento na busca da etiologia da anemia ferropriva.

Nathan Dubois, David Van Morckhoven, Laurentijn Tilleman et al.

Conrad-Amadeus Voltin, Philipp Gödel, Laura Beckmann et al.

The introduction of chimeric antigen receptor (CAR) T-cell therapy has led to a fundamental shift in the management of relapsed and refractory large B-cell lymphoma. However, our understanding of risk factors associated with non-response is still insufficient and the search for predictive biomarkers continues. Some parameters measurable on 18F-fluorodeoxyglucose positron emission tomography (PET) may be of additional value in this context. A total of 47 individuals from three German university centers who underwent re-staging with PET prior to CAR T-cell therapy were enrolled into the present study. After multivariable analysis considering tumor characteristics and patient factors that might affect progression-free survival (PFS), we investigated whether metabolic tumor volume (MTV) or maximum standardized uptake value (SUVmax) further improve risk stratification. Their most suitable cut-offs were determined by Cox and logistic regression. Forward selection identified extra-nodal disease as the most predictive factor of those routinely available, and we found it to be associated with significantly inferior overall survival after CAR T-cell treatment (P = 0.012). Furthermore, patients with MTV and SUVmax higher than the optimal threshold of 11 mL and 16.7, respectively, experienced shorter PFS (P = 0.016 and 0.002, respectively). Hence, these risk factors might be useful for selection of individuals likely to benefit from CAR T-cell therapy and their management.

PC Gontijo, DDVC Branco, FCS Bicalho et al.

Objetivos: O linfoma plasmablástico (LPB) é um linfoma não-Hodking raro, agressivo e formado por grandes células neoplásicas CD20-negativo e com fenótipo plasmocítico. Apesar de inicialmente ter sido descrito em pacientes portadores do vírus HIV e de outras imunodeficiências, mais recentemente também foi identificado em pacientes imunocompetentes. Este relato de caso e revisão de literatura tem como objetivo apresentar a história de uma paciente diagnosticada com LPB, bem como discutir seu quadro clínico extremamente incomum e os desafios para diagnóstico e tratamento dessa condição. Materiais e métodos: Foram utilizados dados obtidos por meio da anamnese, do exame físico, da propedêutica e do plano terapêutico da paciente, além de literatura publicada nas bases de dados PubMed e Scielo. Resultados: Paciente do sexo feminino, 46 anos, assintomática, com história pregressa de sífilis e Epstein-Barr vírus positivo, observou, em 09/2020, durante autoexame, nódulo na mama esquerda, motivando propedêutica adequada. Em 11/2020 foi submetida a uma quadrantectomia esquerda e biópsia de linfonodos sentinela. Em 12/2020, a análise da biópsia excisional confirmou o diagnóstico de LPB estágio IV-A, de forma que em 02/2021 iniciou-se quimioterapia com protocolo EPOCH. Em 06/2021, foi identificada uma resposta metabólica parcial ao protocolo EPOCH, o que motivou uma troca para o protocolo DHAP, resultando em uma resposta metabólica completa em 12/2021. A paciente foi submetida a um transplante de medula óssea (TMO) autólogo para consolidação do tratamento em 05/2022, e o PET-CT pós-TMO revelou recidiva de lesões em região cervical, coluna e reto. Entretanto, o tamanho das lesões contraindicava a realização de biópsia.A paciente está em seguimento oncológico e permanece assintomática. Discussão: Este relato de caso apresenta um cenário individualizado, já que apesar de a paciente apresentar uma imuno-histoquímica característica para a doença (CD20-, Ki-67+, CD138+), sua evolução clínica foi bastante incomum. Diagnosticada com LBP em estágio IV-A (sobrevida esperada de cerca de 9 meses), a paciente teve resposta completa à quimioterapia. Entretanto, 5 meses depois, evoluiu com recidiva da doença. Nesse contexto, por mais que os parâmetros propedêuticos indiquem presença de LPB na paciente, ela permaneceu assintomática desde o diagnóstico inicial, sofrendo somente com os efeitos adversos da quimioterapia. Além disso, por não apresentar nenhum fator de imunossupressão crônica, a paciente não se enquadra no perfil do grupo que historicamente apresenta a doença. Pela apresentação clínica heterogênea da doença e pela dificuldade em estabelecer um padrão de tratamento, no momento, é questionável o seguimento terapêutico. Os principais protocolos quimioterápicos já foram instituídos e a possibilidade de um novo TMO é limitada, tendo em vista que um TMO autólogo já foi realizado e que os familiares de primeiro grau não são compatíveis. Conclusão: Este relato de caso destaca a dificuldade de se estabelecer um tratamento adequado para o LPB, já que não há consenso na literatura acerca dos protocolos propedêuticos e terapêuticos de tal condição. Demonstra ainda a importância de uma abordagem multidisciplinar e individualizada para cada paciente, principalmente por se tratar de uma neoplasia rara que pode se apresentar clinicamente de forma incomum.

M. Simunic, A. Dobricic, M. Vujcic et al.

Merline Augustine, Ankita Mahambare, M V Mallya et al.

Background and Objectives: An increasing prevalence of sexually transmitted infections especially Syphilis in blood donors may lead to increased donor deferrals and lessen the donor pool. Hence it is the need of the hour to device a structured screening methodology for Syphilis reactive donors. The aim of the study is to estimate the prevalence of syphilis in blood donors and to estimate the response rate of notified blood donors. Patients and Methods: This was a retrospective study, conducted in a Blood Centre (BC) on the blood donors visiting for donation from January 2020 to September 2021. The donors screened positive on chemiluminescence immunoassay were included after informed consent was obtained, and donor demographics and follow-up response rate were analyzed. Frequency and percentages were used to express descriptive statistics and Chi-square was used to test the relationship between categories( p<0.05 considered significant). Results: Out of 26,698 donations during the study period, 133 donors were screened positive for syphilis. 127 (96%) donors consented to postdonation. Out of 127, only 61 were notified regarding the infection and were advised about further followup. 52% of the donors who consented to postdonation counseling were not notified due to erroneous contact details. Out of 46 responders, only 17 underwent further testing. Conclusions: The prevalence rate of syphilis was found to be 0.49%. Among the donors notified, 75% responded back to the BC. Due to social stigma and inadequate knowledge about the disease, often, donors are hesitant to give a reliable exposure history. Adequate education and adapting to a structured screening methodology is the need of the hour to reduce the risk of transfusion-transmitted syphilis.

Connie H. Yan, Maryam Naveed, Ali Alobaidi et al.

Abstract Background Anticoagulation with warfarin represents a transportation‐sensitive treatment state. Transportation barrier is a common reason for not using health care services. Objective To assess the association between transportation barriers to anticoagulation clinic and anticoagulation control (AC) among an inner‐city, low‐income population. Patients/Methods Adults expected to be on chronic warfarin therapy were recruited from an ambulatory anticoagulation clinic. Participants completed a validated questionnaire that assessed transportation barriers to clinic, defined as self‐reported trouble getting transportation to a clinic and a composite score of the presence of transportation barriers. Suboptimal AC was defined as time in therapeutic range (TTR) <60% over 6 months. Prevalence ratios with 95% confidence intervals (CIs), adjusted for age, sex, and annual household income, described the association of transportation trouble and barriers with AC. Results Of 133 participants, 42.9% had suboptimal AC. Mean age was 60.4 (SD, 13.6) years, and the majority of participants were women (62.2%). Participants with transportation trouble were more likely to report being disabled/unable to work (63.6%) and annual household income <$15 000 (45.5%). Mean TTR was significantly lower for participants with transportation trouble compared to those without (53.8% [SD, 24.7%] vs 64.7% [SD, 25.0%]; P = .03). Participants reporting transportation trouble or at least one transportation barrier were 1.60 (95% CI, 1.07‐2.39) and 1.68 (95% CI, 1.01‐2.80) times more likely, respectively, to have suboptimal AC compared to those without. Conclusion Inner‐city, low‐income individuals with transportation barriers were more likely to have suboptimal AC. Further research is warranted to evaluate the impact of alleviating patient‐specific transportation barriers on anticoagulation outcomes.

Shira R. Paul, Preston S. Gable

Invasive mucormycosis is an increasingly common cause of morbidity and mortality in hematologic malignancy patients. Early consideration of the diagnosis is essential in at-risk patients, exhibiting suggestive signs and symptoms. A 56-year-old female with acute myeloid leukemia initially presented with neutropenic fever before subsequently developing dense hemiplegia due to septic emboli to the spine and multifocal abscesses. These findings were later determined to be a result of a disseminated mucor infection and represented a rare manifestation of the disease. Despite the disseminated nature of the infection, identification of the causative organism was initially impeded by limitations in obtaining a tissue sample in a severely thrombocytopenic patient, as is common among hematologic malignancy patients. As a result of this limitation, diagnosis was ultimately made via PCR on bronchiolar lavage fluid. Early consideration of the diagnosis with prompt initiation of treatment is of utmost importance in this invasive infection. Further research is needed to identify and validate rapid, minimally invasive strategies for early diagnosis of mucormycosis.

M. Yamashita

Xavier Waltz, Marc Romana, Marie-Laure Lalanne-Mistrih et al.

The aim of the study was to determine the factors associated with resting and exercise-induced hemoglobin oxygen desaturation. The well-established six-minute walk test was conducted in 107 sickle cell children (50 with sickle hemoglobin C disease and 57 with sickle cell anemia) at steady state. Hemoglobin oxygen saturation was measured before and immediately after the six-minute walk test. Blood samples were obtained on the same day to measure hematologic and hemorheological parameters. Exercise-induced hemoglobin oxygen desaturation was defined as a drop in hemoglobin oxygen saturation of 3% or more at the end of the six-minute walk test compared to resting levels. No children with sickle hemoglobin C disease, but approximately 50% of children with sickle cell anemia showed mild or moderate oxygen desaturation at rest, which was independently associated with the percentage of reticulocytes. Exercise-induced hemoglobin oxygen desaturation was observed in 18% of children with sickle hemoglobin C disease and 34% of children with sickle cell anemia, and was independently associated with the six-minute walk test, acute chest syndrome rate and the strength of red blood cell aggregates in children with sickle cell anemia. No association was found in children with sickle hemoglobin C disease between exercise-induced hemoglobin oxygen desaturation and the measured parameters. Hemoglobin oxygen desaturation at rest was common in children with sickle cell anemia but not in children with sickle hemoglobin C disease, and was mainly associated with greater hemolysis. Physiological strain during exercise and red blood cell aggregation properties may predict the occurrence of exercise-induced hemoglobin oxygen desaturation in children with sickle cell anemia.

Jurg Schwaller

Matilde Cacciatore, Carla Guarnotta, Marco Calvaruso et al.

Aggressive B-cell lymphomas share high proliferative and invasive attitudes and dismal prognosis despite heterogeneous biological features. In the interchained sequence of events leading to cancer progression, neoplastic clone-intrinsic molecular events play a major role. Nevertheless, microenvironment-related cues have progressively come into focus as true determinants for this process. The cancer-associated microenvironment is a complex network of nonneoplastic immune and stromal cells embedded in extracellular components, giving rise to a multifarious crosstalk with neoplastic cells towards the induction of a supportive milieu. The immunological and stromal microenvironments have been classically regarded as essential partners of indolent lymphomas, while considered mainly negligible in the setting of aggressive B-cell lymphomas that, by their nature, are less reliant on external stimuli. By this paper we try to delineate the cardinal microenvironment-centred dynamics exerting an influence over lymphoid clone progression in aggressive B-cell lymphomas.

Ester Mejstrikova, Jana Volejnikova, Eva Fronkova et al.

Background Mixed phenotype acute leukemia (MPAL) represents a diagnostic and therapeutic dilemma. The European Group for the Immunological Classification of Leukemias (EGIL) scoring system unambiguously defines MPAL expressing aberrant lineage markers. Discussions surrounding it have focused on scoring details, and information is limited regarding its biological, clinical and prognostic significance. The recent World Health Organization classification is simpler and could replace the EGIL scoring system after transformation into unambiguous guidelines.Design and Methods Simple immunophenotypic criteria were used to classify all cases of childhood acute leukemia in order to provide therapy directed against acute lymphoblastic leukemia or acute myeloid leukemia. Prognosis, genotype and immunoglobulin/T-cell receptor gene rearrangement status were analyzed.Results The incidences of MPAL were 28/582 and 4/107 for children treated with acute lymphoblastic leukemia and acute myeloid leukemia regimens, respectively. In immunophenotypic principal component analysis, MPAL treated as T-cell acute lymphoblastic leukemia clustered between cases of non-mixed T-cell acute lymphoblastic leukemia and acute myeloid leukemia, while other MPAL cases were included in the respective non-mixed B-cell progenitor acute lymphoblastic leukemia or acute myeloid leukemia clusters. Analogously, immunoglobulin/T-cell receptor gene rearrangements followed the expected pattern in patients treated as having acute myeloid leukemia (non-rearranged, 4/4) or as having B-cell progenitor acute lymphoblastic leukemia (rearranged, 20/20), but were missing in 3/5 analyzed cases of MPAL treated as having T-cell acute lymphobastic leukemia. In patients who received acute lymphoblastic leukemia treatment, the 5-year event-free survival of the MPAL cases was worse than that of the non-mixed cases (53±10% and 76±2% at 5 years, respectively, P=0.0075), with a more pronounced difference among B lineage cases. The small numbers of MPAL cases treated as T-cell acute lymphoblastic leukemia or as acute myeloid leukemia hampered separate statistics. We compared prognosis of all subsets with the prognosis of previously published cohorts.Conclusions Simple immunophenotypic criteria are useful for therapy decisions in MPAL. In B lineage leukemia, MPAL confers poorer prognosis. However, our data do not justify a preferential use of current acute myeloid leukemia-based therapy in MPAL.

Thomas Kink, Helmut Reul

Abstract: The most crucial element of a long‐term implantable rotary blood pump is the rotor bearing. Because of heat generation and power loss resulting from friction, seals within the devices have to be avoided. Actively controlled magnetic bearings, although maintenance‐free, increase the degree of complexity. Hydrodynamic bearings for magnetically coupled rotors may offer an alternative solution to this problem. Additionally, for miniature pumps, the load capacity of hydrodynamic bearings scales slower than that of, for example, magnetic bearings because of the cube‐square‐law. A special kind of hydrodynamic bearing is a spiral groove bearing (SGB), which features an excellent load capacity. Mock‐loop tests showed that SGBs do not influence the hydraulic performance of the tested pumps. Although, as of now, the power consumption of the SBG is higher than for a mechanical pivot bearing, it is absolutely contact‐free and has an unlimited lifetime. The liftoff of the rotor occurs already at 10% of design speed. Further tests and flow visualization studies on scaled‐up models must demonstrate its overall blood compatibility.

María E Alfonso Valdés, Rosa M Lam Díaz, José M Ballester Santovenia et al.

Se desarrolló una investigación para estudiar la estructura y funcionamiento de 4 bancos de sangre provinciales del país, su influencia en la calidad de esta actividad y el grado de satisfacción de los donantes, con el empleo de la guía metodológica para investigación de aspectos socio-culturales relacionados con la donación voluntaria de sangre de la Organización Panamericana de la Salud. El estudio arrojó que, en sentido general, los bancos cuentan con la estructura y equipamiento mínimo indispensable para desarrollar su actividad, pero en la mayoría de los casos, necesitan labores de mantenimiento, remodelación de algunas áreas, así como la adquisición y modernización del equipamiento. El funcionamiento general de estos centros es satisfactorio, pero deben mejorarse algunos aspectos como la calidad de la atención a los donantes, la información que se les brinda, las tareas de promoción de la donación y la retención de donantes<br>A research was developed to study the structure and functioning of 4 provincial blood banks in the country, their influence on the quality of this activity and the satisfaction degree of the donors. To this end, it was used the PHO&#8217;s methodological guide for investigating the sociocultural aspects related to voluntary blood donations. The study showed that, in general, the banks have the structure and indispensable minimum equipment to develop their activity, but in most of the cases they need maintenance, remodelation of some areas, as well as the purchasing and modernization of equipment. The general functioning of these centers is satisfactory, but some aspects as the quality of the attention to donors, the information given, the activities carried out to promote donations and the retention of donors should be improved