Oral Mucositis in Children with Leukemia Undergoing Chemotherapy: A Case Series

ABSTRACT Objective: To report nine cases of pediatric patients with Acute Lymphoid Leukemia (ALL) or Acute Myeloid Leukemia who developed severe oral mucositis (SOM) at the first week of chemotherapy. Material and Methods: The cases were selected from a sample of 105 children followed for 10 consecutive weeks. Hematological and personal data were obtained from the patient's medical records. The oral cavity was examined weekly using the modified Oral Assessment Guide. Results: More of the patients were male (55.6%), had black/brown skin (55.6%), with ALL (66.7%), and the mean age was 5.55. Two patients had values below normal for leukocytes, platelets, and creatinine over the follow-up. However, all patients showed changes in the normality of hematological data in most weeks. The most used chemotherapeutic agents were aracytin, etoposide, and methotrexate, known for their high stomatotoxic potential. Patients had 2 to 6 (mean of 4) episodes of SOM and 4 to 7 (mean of 5.5) episodes of OM. One patient at week 7, one patient at week 5, and one patient at weeks 2 and 10 did not have OM. Saliva (84 times) and lips (44 times) were the most affected items. Conclusion: The patients showed oscillations in the severity of oral mucositis and hematological parameters over the follow-up. All patients were exposed to stomatotoxic drugs during the initial phase of cancer treatment.

Haematological diseases associated with Hepatitis E virus infection / 临床肝胆病杂志

‍Hepatitis E virus （HEV） infection can cause acute， fulminant， and chronic hepatitis and is thus a worldwide health problem. The general population has no obvious symptoms or only mild symptoms after HEV infection， and most symptoms are self-limited and are easily ignored in clinical diagnosis. Previous reports have shown that HEV infection can cause dysfunction in multiple systems， especially the blood system， in which it often leads to a series of adverse outcomes. Comprehensive evaluation， early diagnosis， and early intervention of these HEV-related diseases will greatly reduce the medical burden of individuals and society. With reference to the previous reports， this article reviews the blood system diseases associated with HEV infection.

Influencing factors of blood transfusion adverse reactions in patients with hematologic diseases / 白血病·淋巴瘤

Objective:To explore the influencing factors and prevention and control measures of blood transfusion adverse reactions in patients with hematologic diseases.Methods:The clinical data of 988 patients with hematologic diseases requiring blood transfusion from January 2019 to December 2021 in Yongchuan Hospital Affiliated to Chongqing Medical University were retrospectively analyzed. The occurrence of blood transfusion adverse reactions in patients transfused with different blood preparations and different types of hematologic diseases was counted. Binary logistic regression was used for multivariate analysis to analyze the effects of gender, age, history of blood transfusion, history of allergy, primary blood disease, and type of blood transfusion on blood transfusion adverse reactions.Results:The 988 patients were transfused 4 712 times, and there were 62 times of blood transfusion adverse reactions, of which the incidence of allergic reactions was 53.2% (33/62), and that of non-hemolytic febrile transfusion reactions was 45.2% (28/62). A patient who presented with chest tightness and shortness of breath did not have a clear diagnosis of blood transfusion adverse reactions. Univariate analysis showed that blood transfusion history ( χ2 = 4.64, P = 0.031), allergic history ( χ2 = 700.07, P < 0.01) and type of blood transfusion ( χ2 = 19.88, P < 0.01) were all associated with blood transfusion adverse reactions. Multivariate logistic regression analysis showed that allergy history ( OR = 0.013, 95% CI 0.007-0.024, P < 0.001) and type of blood transfusion ( OR = 0.192, 95% CI 0.077-0.479, P < 0.001) were independent factors influencing the occurrence of blood transfusion adverse reactions. Conclusions:For patients with hematologic diseases requiring blood transfusion, strictly controlling transfusion indications, being alert to high-risk groups with a history of allergies requiring plasma and platelet transfusions, and taking countermeasures in advance can help reduce the occurrence of blood transfusion adverse reactions and improve transfusion safety.

Clinical characteristics and CT findings of Pneumocystis Jirovecii pneumonia in 46 cases with hematological diseases / 中华血液学杂志

Objective: To summarize the original CT features of Pneumocystis Jirovecii pneumonia in patients with hematological diseases. Methods: A retrospective analysis was carried out in 46 patients with proven pneumocystis pneumonia (PJP) in the Hospital of Hematology, Chinese Academy of Medical Sciences between January 2014 and December 2021. All patients had multiple chests CT and related laboratory examinations, imaging typing were conducted based on the initial CT presentation, and the distinct imaging types were analyzed against the clinical data. Results: In the analysis, there were 46 patients with proven pathogenesis, 33 males, and 13 females, with a median age of 37.5 (2-65) years. The diagnosis was validated by bronchoalveolar lavage fluid (BALF) hexamine silver staining in 11 patients and clinically diagnosed in 35 cases. Of the 35 clinically diagnosed patients, 16 were diagnosed by alveolar lavage fluid macrogenomic sequencing (BALF-mNGS) and 19 by peripheral blood macrogenomic sequencing (PB-mNGS) . The initial chest CT presentation was categorized into 4 types, including ground glass (GGO) type in 25 cases (56.5%) , nodular type in 10 cases (21.7%) , fibrosis type in 4 cases (8.7%) , and mixed type in 5 cases (13.0%) . There was no substantial discrepancy in CT types among confirmed patients, BALF-mNGS diagnosed patients and PB-mNGS diagnosed patients (χ(2)=11.039, P=0.087) . The CT manifestations of confirmed patients and PB-mNGS diagnosed patients were primarily GGO type (67.6%, 73.7%) , while that of BALF-mNGS diagnosed patients were nodular type (37.5%) . Of the 46 patients, 63.0% (29/46) had lymphocytopenia in the peripheral blood, 25.6% (10/39) with positive serum G test, and 77.1% (27/35) with elevated serum lactate dehydrogenase (LDH) . There were no great discrepancies in the rates of lymphopenia in peripheral blood, positive G-test, and increased LDH among different CT types (all P>0.05) . Conclusion: The initial chest CT findings of PJP in patients with hematological diseases were relatively prevalent with multiple GGO in both lungs. Nodular and fibrosis types were also the initial imaging findings for PJP.

Plasma Calcium Level and C-Reactive Protein Albumin Ratio Affect Severe Bleeding After Coronary Artery Bypass Grafting

ABSTRACT Objective: In this study, we aimed to determine whether plasma calcium level and C-reactive protein albumin ratio (CAR) as well as other demographic and hematological markers are related in predicting severe bleeding after coronary artery bypass grafting (CABG). Methods: A total of 227 adult patients who underwent CABG at our hospital between December 2021 and June 2022 were prospectively studied. Total amount of chest tube drainage was evaluated within the first 24 hours postoperatively or until the patient was re-explored for bleeding. The patients were divided into two groups - Group 1, patients with low amount of bleeding (n=174), and Group 2, patients with severe bleeding (n=53). Univariate and multivariate regression analyzes were performed to determine independent parameters related to severe bleeding within the first 24 hours after surgery. Results: When the groups were compared in terms of demographic, clinical, and preoperative blood parameters; cardiopulmonary bypass time and serum C-reactive protein (CRP) levels were found to be significantly higher in Group 2 compared to the low bleeding group. In addition, lymphocytes, hemoglobin, calcium, albumin, and CAR were found to be significantly lower in Group 2. In multivariate analysis, calcium, albumin, CRP, and CAR were found to be independent predictors of significant association with excessive bleeding. A cut-off value of 8.7 (94.3% sensitivity and 94.8% specificity) for calcium and 0.155 (75.4% sensitivity and 80.4% specificity) for CAR predicted excessive bleeding. Conclusion: Plasma calcium level, CRP, albumin, and CAR can be used to predict severe bleeding after CABG.

Acute invasive fungal rhinosinusitis in pediatric patients with oncohematological diseases

Abstract Introduction Invasive fungal diseases represent important causes of morbidity and mortality among pediatric oncohematological patients. Acute invasive fungal rhinosinusitis is a rare and aggressive disease that occurs mainly in immunocompromised patients. The mortality rate is high and therefore, accurate and early diagnosis is essential. Objectives The aim of this study was to describe the frequency of acute invasive fungal rhinosinusitis among pediatric oncohematological patients and characterize them with confirmed diagnoses. Methods This was a retrospective study that analyzed the medical records of pediatric patients diagnosed with oncohematological diseases and suspected fungal infections, who were included after obtaining informed consent, from January to December 2017, in the pediatric unit of a tertiary university hospital. Data collected from medical record analysis included the following: underlying diagnosis, absolute neutrophil count, clinical presentation, culture and biopsy results, surgical procedures performed, survival and mortality. Results A total of 27 patients were evaluated, with three suspected cases of acute invasive fungal rhinosinusitis. Histopathological and microbiological analyses confirmed two cases. In both cases, the pathogen isolated in the culture was Fusarium sp. The two confirmed cases were female, aged 12 and 14 years, both with an absolute neutrophil count of 10 cells/μL. The underlying disease of the first patient was acute myeloid leukemia (subtype M5), whereas the second patient presented idiopathic bone marrow aplasia. Conclusion Both confirmed cases of acute invasive fungal rhinosinusitis presented with constitutional symptoms and signs of nasal and sinusital inflammation. This demonstrates the importance of fever as a symptom in immunocompromised patients and it should prompt otorhinolaryngological investigation.

Relação entre a infecção por Helicobacter pylori e doenças hematológicas: uma revisão integrativa / The relationship between Helicobacter pylori infection and hematologic diseases: an integrative review

Helicobacter pylori, uma bactéria gram-negativa, desde 1984 vem sendo associada às doenças gástricas. A partir da década de 1990, surgiram crescentes relatos indicando a relação da infecção com manifestações extragástricas. Nesse sentido, o objetivo do estudo foi investigar, através de uma revisão integrativa, as evidências relacionadas à H. pylori e a presença de doenças hematológicas, focando na anemia por deficiência de ferro (ADF) e na púrpura trombocitopênica idiopática (PTI). Bases de dados foram consultadas com as palavras-chave e descritores Helicobacter pylori, Doenças extragástricas, Doenças hematológicas, Anemia ferropriva e Púrpura Trombocitopênica Idiopática, nos idiomas inglês, português e espanhol, combinados com o operador booleano "AND". Após a leitura de 1.964 títulos, 85 artigos atendiam aos critérios de inclusão. Com a exclusão dos artigos duplicados e pela análise dos resumos, 62 trabalhos foram selecionados e lidos na íntegra. Por fim, 27 estudos foram incluídos: 13 relacionados à ADF e 77% deles encontraram associação com H. pylori, e 14 relacionados à PTI, nos quais a relação com a bactéria foi encontrada em 93%. As evidências que associam essas doenças hematológicas com H. pylori são expressivas, portanto, mais estudos são necessários para elucidar os mecanismos relacionados e contribuir para prevenção, diagnóstico e tratamento mais eficazes.

Helicobacter pylori is a gram-negative bacterium that has been associated with gastric diseases since 1984. Since the 1990s, there have been increasing reports indicating that the infection may also be associated with extragastric manifestations. This integrative review aimed to investigate the evidence on the relationship between H. pylori and hematological diseases, specifically iron deficiency anemia (IDA) and idiopathic thrombocytopenic purpura (ITP). Databases were searched for the keywords "Helicobacter pylori," "extragastric diseases," "hematologic diseases," "iron deficiency anemia," and "idiopathic thrombocytopenic purpura" in English, Portuguese, and Spanish, combined with the boolean operator "AND." The search yielded 1,964 studies. After reading the titles, only 85 met the inclusion criteria. Sixty-two studies were selected for full-text reading after exclusion of duplicates and abstract analysis. Finally, 27 studies were included in this review. Thirteen studies addressed IDA, among which 77% found an association with H. pylori; whereas 14 studies addressed ITP, among which 93% found a relationship with H. pylori. There is strong evidence supporting the association between hematologic diseases and H. pylori. Further studies are needed to elucidate the mechanisms involved in this relationship, contributing to more effective prevention, diagnosis, and treatment.

Avaliação do perfil bioquímico, hematológico e imunológico em pacientes com diagnóstico recente para HIV de um Centro de Referência em Santarém, Pará, Brasil / Evaluation of the biochemical, hematological and immunological profile in patients with a recent diagnosis of HIV in Santarém Reference Center, Pará, Brazil

Introdução: Alterações hematológicas, bioquímicas e imunológicas podem estar presentes no paciente infectado pelo HIV, no momento do diagnóstico, antes ou depois de iniciar com os antirretrovirais. Objetivo: Analisar o perfil bioquímico, hematológico e imunológico de pacientes com diagnóstico recente para HIV. Método: O estudo avaliou 321 prontuários de pacientes recém diagnosticados com a infecção pelo HIV. A coleta de dados envolveu informações sociodemográficas (data de nascimento, idade, sexo, escolaridade, estado civil, vínculo empregatício e procedência), clínicas (data do diagnóstico para a infecção pelo HIV, situação de imunodeficiência e tipo de exposição), bioquímicas (glicose, triglicerídeos, colesterol total e frações), hematológicas (hemoglobina e plaqueta) e imunológicas (linfócitos T CD4+ e carga viral). Os dados foram analisados por estatística descritiva e inferencial, adotando-se p<0,05. Resultados: Notou-se predominância do sexo masculino (67%), faixa etária de 18-27 anos (39,9%), solteiros (58,6%) e com 32% dos pacientes apresentando Aids. Das variáveis analisadas, o sexo masculino apresentou, em relação às mulheres, maior quantidade de hemoglobina e menores valores para contagem de linfócitos T CD4+, glicose e colesterol total (p<0,05). Além disso, ressalta-se que 69% da amostra apresentou alguma alteração lipídica, 96% tinha carga viral detectável e 29% apresentou linfócitos T CD4+ <200 cel/mm3. Conclusão: Pessoas vivendo com o HIV, no momento do diagnóstico, podem apresentar alterações imunológicas, hematológicas e bioquímicas, tornando imprescindível a avaliação, acompanhamento e orientação multiprofissional, tanto antes como posterior introdução dos antirretrovirais, a fim de evitar futuros agravos a saúde. [au]

Introduction: Hematological, biochemical, and immunological alterations may already be present in HIV-infected patients at the time of diagnosis or before, or after starting antiretroviral therapy. Objective: Analyze the biochemical, hematological, and immunological profile of patients with a recent diagnosis of HIV. Method: The study evaluated 321 medical records of patients newly diagnosed with HIV infection. Data collection involved sociodemographic (date of birth, age, gender, education, marital status, employment relationship, and origin), clinical (date of diagnosis for HIV infection, immunodeficiency status, and type of exposure), biochemical (glucose, triglycerides, total cholesterol, and fractions), hematological (hemoglobin and platelet) and immunological (CD4+ T lymphocytes and viral load) information. Data were analyzed by descriptive and inferential statistics, adopting p<0.05. Results: There was a predominance of males (67%), aged 18-27 years (39.9%), single (58.6%), and 32% of patients had AIDS. Of the variables analyzed, males presented higher amounts of hemoglobin and lower values for CD4+ T lymphocyte count, glucose, and total cholesterol in relation to females (p<0.05). In addition, it is noteworthy that 69% of the sample presented a lipid alteration, 96% had a detectable viral load, and 71% had CD4+ T lymphocytes <200 cells/mm3. Conclusion: People living with HIV, at the time of diagnosis, may present immunological, hematological, and biochemical alterations, making multidisciplinary evaluation, follow up, and guidance essential, both before and after the introduction of antiretroviral therapy, in order to avoid future health problems. [au]

Aeromonas hydrophila bloodstream infection in patients with hematologic diseases: a report of 4 cases / 中国热带医学

@#Abstract: To report the diagnosis, treatment and outcome of 4 patients with hematological diseases complicated with Aeromonas hydrophila bloodstream infection in the Second Affiliated Hospital of Kunming Medical University, further clarify the importance of blood culture and deepen the clinical understanding of the disease. Four patients with hematological diseases complicated with Aeromonas hydrophila bloodstream infection treated in the Second Affiliated Hospital of Kunming Medical University from 2017 to 2021 were recruited as the study objects. The clinical manifestations, blood culture collection, detection time of Aeromonas hydrophila, laboratory examination, treatment and prognosis of the patients were retrospectively analyzed. In this study, 4 cases were male patients with hematological diseases, who were in myelosuppression after chemotherapy. After fever, blood culture was collected and Aeromonas hydrophila was detected. The positive time of blood culture in 4 cases ranged from 4 to 11 hours. The results of antibiotic sensitivity showed that it was highly sensitive to the second, third and fourth generation cephalosporins, quinolones and carbapenems. Four patients were treated with imipenem cilastatin sodium in the early stage, and one patient recovered after active anti infection and leukocyte raising treatment. One patient did not complete chemotherapy due to a request for discharged, and the follow-up was unknown. Two patients developed rapidly into necrotizing fasciitis and died later. Hematological diseases complicated with Aeromonas hydrophila bloodstream infection are rare, but the mortality rate is high. For patients with repeated fever and considering infection, blood culture should be carried out as soon as possible to confirm the pathogen and drug sensitivity test. During clinical treatment, the treatment should be adjusted in time in combination with the patient's situation. In addition to anti-infection treatment, the patient's immunity should be improved and the development of necrotizing fasciitis should be vigilant. Keywords: Aeromonas hydrophila; hematologic diseases; leukemia; bloodstream infection; blood culture; necrotizing fasciitis

Effect of donor clonal hematopoiesis on allogeneic hematopoietic stem cell transplantation / 白血病·淋巴瘤

Clonal hematopoiesis (CH) refers to the clonal expansion of hematopoietic stem/progenitor cells in some individuals with normal blood indexes. The incidence of CH increases with age, reflecting the decline of the hematopoietic and potential clonal evolution to a certain extent. In recent years, an increasing number of studies have shown that donor CH is an unfavorable factor affecting transplantation, graft-versus-host disease and donor cell leukemia after allogeneic hematopoietic stem cell transplantation. Emphasis on and identification of donor CH can optimize donor selection and help transplant patients benefit more. This article introduces the relevant research progress in combination with the content of the 63rd American Society of Hematology Annual Meeting.

Analysis of the distribution of infectious bacteria and the status of drug resistance in hospitalized patients of hematology department / 白血病·淋巴瘤

Objective:To investigate the bacterial distribution of secondary infection and the status of drug resistance in hospitalized patients of hematology department.Methods:The clinical data of 1 125 inpatients in the Hematology Department of the Affiliated Hospital of Xuzhou Medical University from January 2015 to December 2019 were retrospectively analyzed, and the distribution of infectious pathogens and the status of drug resistance of these inpatients were analyzed.Results:A total of 9 335 microbial samples from 1 125 inpatients were submitted for examination, among which 1 349 were positive samples. Among 1 349 positive samples, the gram-negative bacteria-positive samples accounted for 66.4% (895/1 349) and the gram-positive bacteria-positive samples accounted for 33.7% (454/1 349); the blood samples accounted for 44.7%(603/1 349), the sputum samples accounted for 33.9% (457/1 349), and the urine samples accounted for 9.4%(127/1 349). The isolated bacteria whose proportion ranked as the top 3 were Escherichia coli (31.0%), Staphylococcus aureus (21.0%) and Klebsiella pneumoniae (18.0%). The drug resistance rate of Escherichia coli to ceftriaxone was as high as 77.2%, and that of Staphylococcus aureus and coagulase-negative Staphylococcus to benzoxicillin was 58.2% and 66.7%, but both had no resistance to vancomycin.Conclusions:There are a wide variety of infectious pathogens in hospitalized patients of hematology department, and the Escherichia coli and Klebsiella pneumonia are predominant. More attention should be paid to antibiotic prescribing training for clinicians to optimize and standardize the use of antibiotics.

Skeletal involvement in Gaucher disease: extent of bone disease, splenic volume, and quality of life / Envolvimento ósseo na doença de Gaucher: extensão do acometimento ósseo, volume esplênico e qualidade de vida

Abstract Objective: To investigate the correlations among the extent of bone involvement, splenic volume, and quality of life in patients with Gaucher disease. Materials and Methods: This was a descriptive, prospective cross-sectional study of 18 patients with Gaucher disease who underwent 3-T magnetic resonance imaging of both femurs and the lumbar spine. Semiquantitative analyses were performed on the basis of the bone marrow burden (BMB) score. We looked for linear relationships among the variables splenic volume, quality of life score, and BMB score. Results: We identified a linear relationship between the BMB scores and splenic volume. The quality of life score showed no statistically significant relationship with splenic volume or the BMB score. Conclusion: The linear relationship between the BMB score and the splenic volume indicates that the extent of bone disease is greater in individuals with splenomegaly. No correlation was found between the BMB and quality of life scores, illustrating the insidious and silent progression of Gaucher disease.

Resumo Objetivo: Investigar a correlação entre a extensão do envolvimento ósseo, o volume esplênico e a qualidade de vida em pacientes com doença de Gaucher. Materiais e Métodos: Estudo descritivo, prospectivo e transversal de 18 pacientes com doença de Gaucher submetidos a ressonância magnética de 3-T de ambos os fêmures e da coluna lombar. Análise semiquantitativa foi feita utilizando o escore bone marrow burden (BMB). Correlação linear foi estudada para as variáveis volume esplênico, qualidade de vida e escore BMB. Resultados: Uma correlação linear entre os escores BMB e volume esplênico foi demonstrada. Em relação ao índice de qualidade de vida, não foi observada correlação estatisticamente significante nem com o volume esplênico e nem com o escore BMB. Conclusão: Nosso estudo demonstrou uma correlação linear entre o escore BMB e o volume esplênico, correspondendo a maior extensão de doença óssea em indivíduos com maior esplenomegalia. Nenhuma correlação foi encontrada entre o escore BMB e a qualidade de vida, indicando a natureza insidiosa e a progressão silenciosa da doença de Gaucher.

Effect of leukocyte-depleted suspended red blood cells storaged for different time on patients with hematologic diseases and malignant tumors / 中国输血杂志

【Objective】 To investigate the effect of leukocyte-depleted suspended red blood cells (lds-RBCs) storaged for different time on blood transfusion effect of patients with hematologic diseases and malignant tumors, as well as to evaluate the storage quality of lds-RBCs in blood stations. 【Methods】 Seven hospitals (4 tertiary-A hospitals and 3 secondary-A hospitals), applying for blood from our blood center, were selected. Blood transfusion cases (medical record) and related data (indicators) of patients with blood diseases and malignant tumors in those hospitals from December 2018 to May 2019 were collected, including disease diagnosis (type) before transfusion, demographic characteristics, date of solo transfusion of lds-RBCs, units of lds-RBCs [(1~2)U/bag, 1 U=200 mL whole blood], different storage duration (1~5 weeks) (bar code), and hemoglobin (Hb) 48 h before and after transfusion. The efficacy of lds-RBCs (storaged for different time) transfusion in patients with hematologic diseases and malignant tumors was evaluated by statistical analysis. 【Results】 A total of 3 557 patients with hematologic diseases and malignant tumors were enrolled in this study. No significant changes were noticed in transfusion efficacy by blood transfusion unit, gender and previous transfusion history (P > 0.05). The effective rate of lds-RBCs in patients with blood diseases and malignant tumors, stratified by storage duration, i. e. storaged for >1~2 weeks, >2~3 weeks, >3~4 weeks and more than >4~5 weeks, was 78.77% vs 77.68% vs 75.06% vs 70.37%, and 79.32% vs 76.73% vs 72.79% vs 67.65%, respectively(P<0.05), with lds-RBCs of 4-5 storage weeks presenting the lowest transfusion efficacy in both groups of patients. 【Conclusion】 The storage time of most lds-RBCs supplied by our center is moren than 3 weeks, and the transfusion effect of lds-RBCs stored for 5 weeks needs further observation. In order to ensure and improve the efficacy of blood transfusion, evidence-based medicine and information management are needed to help the clinical gasp the advantageous time of blood products and shorten the storage-to-transfusion time of red blood cells.

Pathogen distribution and antimicrobial resistance among lower respiratory tract infections in patients with hematological malignancies / 中华内科杂志

Objective:To investigate the pathogen distribution and antimicrobial resistance among lower respiratory tract infections in patients with hematological malignancies.Methods:Sputum samples were collected from 967 patients with hematological malignancies and lower respiratory tract infections in Department of Hematology,the Second Hospital of Shanxi Medical University from January 2017 to July 2020. The pathogens and drug sensitivity reports were carried out by automatic bacterial identification instruments. WHONET 5.6 and SPSS 20.0 softwares were used for statistical analysis.Results:A total of 961 strains of pathogens were isolated, 516 (53.7%) pathogens were Gram-negative bacteria, mainly 118 strains of Klebsiella pneumonia (12.3%), 68 strains of Pseudomonas aeruginosa (7.1%), 67 strains of Acinetobacter baumannii (7.0%),52 strains of Stenotrophomonas maltophilia (5.4%), 43 strains of Escherichia coli (4.5%), and 42 strains of Enterbacter cloacae (4.4%). There were 171 (17.8%) strains of Gram-positive bacteria and 274 (28.5%) fungi. The drug resistance rates of Pseudomonas aeruginosa and Acinetobacter baumannii to carbapenem were 22.1%-31.3%. Stenotrophomonas maltophilia was sensitive to levofloxacin, compound sulfamethoxazole and minocycline. The antimicrobial resistance rates of these three enterobacteria to carbapenems, cefoperazone/sulbactam, piperacillin/tazobactam were low (<10%). The resistant Gram-positive bacteria to ticoplanin, vancomycin and linazolamide were not detected.Conclusion:The major pathogens related to lower respiratory tract infections in patients with hematological malignancies are gram-negative bacteria in our centre. Different pathogens appear different characteristics of antimicrobial resistance.

Analysis of adverse reactions of blood transfusion in patients with hematological diseases / 白血病·淋巴瘤

Objective:To explore the causes and preventive strategies of adverse reactions of blood transfusion in patients with hematological diseases.Methods:The clinical data of 150 hematological patients who received 5 184 cases of blood transfusion in Beijing No.6 Hospital from January 2014 to December 2017 were retrospectively analyzed. The blood transfusion adverse reaction reporting forms were collected, and the content of the form included the basic information of the patient, diagnosis, blood transfusion type, blood transfusion time, blood transfusion history, pregnancy history, adverse reaction history of blood transfusion as well as detailed records of clinical symptoms. The blood bank summarized the blood transfusion adverse reaction reporting forms every week.Results:A total of 112 cases of adverse reactions occurred in 5 184 cases of blood transfusion, the incidence rate was 2.16%. Sixty-eight cases (1.32%) had anaphylaxis, mainly caused by platelet transfusion, and 44 cases (0.85%) had fever, mainly caused by transfusion of red blood cells suspension, and no other adverse reactions were found. The incidence rates of adverse reactions of blood transfusion in patients with blood transfusion history and anaphylaxis history were significantly higher than those in patients without blood transfusion history and anaphylaxis history, and the differences were statistically significant [2.34% (102/4 350) vs. 1.20% (10/834), χ2 = 6.899, P = 0.009; 3.06% (98/3 200) vs. 1.42% (14/984), χ2 = 7.767, P = 0.005]; the incidence rate of adverse reactions of blood transfusion in female patients was significantly higher than that in male patients, and the difference was statistically significant [2.82% (60/2 126) vs. 1.70% (52/3 058), χ2 = 8.356, P = 0.004]; there was no significant difference in the incidence rate of adverse reactions of blood transfusion between patients < 18 years old and ≥ 18 years old [1.16% (3/259) vs. 2.21% (109/4 925), χ2 = 1.295, P = 0.255]. Conclusions:The main manifestations of adverse reactions of component blood transfusion are anaphylaxis and non-hemolytic fever. Clinical medical staff must carefully control the blood transfusion indications and select the appropriate blood components to reduce the adverse reactions of blood transfusion and ensure the safety of blood transfusion.

Manejo quirúrgico del paciente con hemofilia sometido a cirugía bucal: Reporte de un caso clínico / Surgical management of the patient with hemophilia undergoing oral surgery: A case report

Resumen Introducción: La hemofilia es una deficiencia congénita de un factor de la coagulación, la cual consta en un trastorno recesivo asociado al cromosoma X, generando disminución o ausencia de actividad funcional del factor. Objetivo: Presentar una revisión narrativa de la literatura sobre pacientes hemofílicos, junto con un caso de manejo de un paciente con la condición. Metodología: Paciente de sexo femenino, 18 años, acude al postgrado de Cirugía y Traumatología bucomaxilofacial de la Universidad Andrés Bello de Santiago de Chile, derivada para realizar exodoncia de terceros molares debido al término de su mecánica ortodóntica. Al realizar la anamnesis próxima, la paciente relata padecer hemofilia A leve, y hace 6 meses presentó un 38% de factor VIII. Previo al tratamiento quirúrgico se solicitó un hemograma completo con examen de coagulación para medir el TTPA. Además, se realizó una interconsulta con el hematólogo tratante para evaluación de su patología y recomendaciones para efectuar la misma con la menor cantidad de riesgos intraquirúrgicos y postquirúrgicos, el cual sugirió la administración de factor VIII previo, y posterior al acto quirúrgico. Así mismo, se aplicaron medidas de hemostasia locales para mejor control y un correcto manejo analgésico postquirúrgico. Conclusión: La hemofilia, es un trastorno que requiere un minucioso manejo tanto pre, intra y postoperatorio de parte del odontólogo, donde los exámenes complementarios, comunicación con el hematólogo, procedimiento atraumático y un correcto manejo de la hemostasia, son fundamentales para el éxito del tratamiento.

Abstract Introduction: Hemophilia is a congenital deficiency of a coagulation factor, associated to a recessive pattern located in the X chromosome, which induces a lower or even absent functional activity of that factor. Objective: To provide a narrative review of the literature about haemophiliac patients, as well as a case report of a patient. Methods: Female patient, 18 years old, attended in the postgraduate of Maxillofacial Surgery of the Andrés Bello University to Santiago, Chile, derived to perform extractions of wisdom teeth due to the end of its orthodontic mechanics. At the anamnesis, the patient reports to suffer from mild hemophilia A, and 6 months ago she had 38% VIII factor. Prior to surgical treatment, a complete blood count with a coagulation test was requested to measure TTPA. In addition, an interconsultation was made with the treating hematologist to perform a correct management to assess of her pathology and recommendations to carry out it with the least amount of intrasurgical and post-surgical risks. Suggested the administration of factor of freeze-dried VIII factor before and after surgery. Local hemostasis measures were also applied for better control and proper post-surgical pain management. Conclusion: Hemophilia, requires the dentist to perform a thorough management pre, intra and postoperatory, in which complementary tests, communication with the hematologist, atraumatic procedure and a precise management of hemostasis, are key for the treatment's success.

Idiopathic thrombocytopenic purpura in a patient with situs inversus totalis: case report and literature review / Púrpura trombocitopênica idiopática em paciente com situs inversus totalis: relato de caso e revisão da literatura

ABSTRACT Situs inversus totalis is a rare recessive autosomal congenital abnormality in which the mediastinal and abdominal organs are in a mirrored position when compared to the usual topography. The literature reports some cases of situs inversus totalis and concomitant conditions: spinal abnormalities, cardiac malformations and hematological diseases, such as idiopathic thrombocytopenic purpura, which is an autoimmune disease that causes thrombocytopenia due to platelet destruction or suppression of its production. This article aimed to report the coexistence of situs inversus totalis and idiopathic thrombocytopenic purpura.

RESUMO Situs inversus totalis é uma anormalidade congênita autossômica recessiva rara em que os órgãos mediastinais e abdominais encontram-se em posição espelhada em relação à topografia habitual. A literatura relata alguns casos de concomitância do situs inversus totalis com outras condições: anomalias espinhais, malformações cardíacas e doenças hematológicas, como púrpura trombocitopênica idiopática, que é uma doença autoimune com plaquetopenia, devido à destruição dos trombócitos ou supressão da sua produção. Esse artigo teve o objetivo de relatar coexistência de situs inversus totalis e púrpura trombocitopênica idiopática.

Clinical features and prognosis of children with hematological malignancies after chemotherapy with Stenotrophomonas maltophilia blood stream infection / 白血病·淋巴瘤

Objective To investigate the clinical features and prognosis of children with hematological malignancies after chemotherapy accompanied with Stenotrophomonas maltophilia blood stream infection (BSI). Methods The clinical data and antimicrobial susceptibility test of 25 hospitalized children with hematological malignancies who were diagnosed as Stenotrophomonas maltophilia BSI in the Department Pediatric Hematology of Fujian Medical University Union Hospital from January 2013 to May 2018 were analyzed retrospectively. Results A total of 25 children, including 18 males and 7 females with the median age 4 (1-11) years old were diagnosed as hematological malignancies and all received chemotherapy. The frequent risk factors of Stenotrophomonas maltophilia BSI in children with hematological malignancies included prior carbapenem antibiotic for more than 1 week (80％, 20/25), and neutropenia for more than 1 week (68％,17/25) and indwelling central venous catheter (48％, 12/25). Clinically, the main manifestations included neutropenia with fever after chemotherapy; however, anti-pseudomonas cephalosporin/carbicillin antibiotic combined with vancomycin and anti-fungal therapies were ineffective. Drug susceptibility test showed that all strains were sensitive to compound sulfamethoxazole, levofloxacin and minocycline. Before blood culture report, 3 patients died of septic shock, pulmonary hemorrhage and respiratory failure; after blood culture report, the treatment regimens were adjusted to compound sulfamethoxazole combined with cefoperazone sodium and sulbactam sodium (200-260 mg·kg-1·d-1) or levofloxacin anti-infective. Finally, 18 patient was cured, 2 patients died of the bad efficacy of underlying diseases, and 2 patients died of pulmonary hemorrhage. The overall fatality rate was 28％ (7/25) and the related mortality rate caused by Stenotrophomonas maltophilia BSI was 20％ (5/25). Conclusion Stenotrophomonas maltophilia BSI in children with hematological malignancies after chemotherapy has a high fatality rate, and better basic disease control and appropriate antibacterial therapy are the key to improve the prognosis.

Neonatal Kabuki syndrome: a report of two cases / 中华围产医学杂志

This paper reported the diagnosis and treatment of two neonates with Kabuki syndrome (KS).Neither of them had typical facial features of KS during the neonatal period,but poor response,abnormal appearance and multiple organ dysplasia were observed in both.Case 1 was lost to follow up after discharge,while typical KS facial features were gradually appeared in Case 2 including eversion of lower lateral eyelids,arched eyebrows,sparse eyebrow arch,flattened nasal tip,prominent ears,during a three-month follow-up after birth.Next-generation sequencing revealed that both neonates were KS caused by lysine methyltransferase 2D (KMT2D) gene mutation,of which case 1 had a heterozygous deletion mutation ofc.13895delC (p.P4632HfsTer8) in KMT2D gene,while case 2 had a heterozygous repeat mutation of c.12809dupA (p.T4271Dfs*63) in KMT2D gene.Both cases were defined as de novo mutations and the one carried by case 2 was a newly discovered pathogenic mutation.

Determining the risk factors associated with the development of Clostridium difficile infection in patients with hematological diseases

BACKGROUND: Clostridium difficile infection (CDI) is a nosocomial condition prevalent in patients with hematological disorders. We aimed to identify the risk factors associated with the development of CDI and assess the mortality rate at 15 and 30 days among hematologic patients admitted to a tertiary care center. METHODS: We conducted a retrospective case-control study from January 2010 to December 2015. Forty-two patients with hematologic malignancy and CDI, and 84 with hematologic disease and without history of CDI were included in the case and control groups, respectively. RESULTS: Univariate analysis revealed that episodes of febrile eutropenia [odds ratio (OR), 5.5; 95% confidence interval (CI), 2.3–12.9; P1 hospitalization (OR, 5.6; 95% CI, 2.5–12.6; P1 hospitalization (OR, 4.3; 95% CI, 1.7–11.0; P=0.002) were independent risk factors. Three (7.1%) and 6 (14.2%) case patients died at 15 and 30 days, respectively. CONCLUSION: The risk factors for developing CDI were exposure to therapeutic antibiotics and previous hospitalization. Hematological patients who developed CDI had higher early mortality rates, suggesting that new approaches for prevention and treatment are needed.