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1.
Hematol., Transfus. Cell Ther. (Impr.) ; 44(1): 32-39, Jan.-Mar. 2022. tab, ilus
Article in English | LILACS | ID: biblio-1364905

ABSTRACT

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.


Subject(s)
Humans , Male , Female , Infant , Child, Preschool , Child , Adolescent , Fusariosis , Invasive Fungal Infections , Hematologic Diseases , Sinusitis , Febrile Neutropenia , Fusarium
2.
Prensa méd. argent ; 107(7): 366-373, 20210000. fig, graf, tab
Article in English | LILACS, BINACIS | ID: biblio-1358960

ABSTRACT

Contexto y objetivo: las anomalías hematológicas se encuentran entre las complicaciones más comunes de la infección por el VIH. También se han realizado bastantes estudios sobre las alteraciones en el perfil de lípidos, aunque los resultados en gran medida no han sido concluyentes. El presente estudio se llevó a cabo para evaluar el recuento de células CD4 y el perfil de lípidos en los pacientes infectados por el VIH y el SIDA en la población india y los correlaciona con los controles sero-negativos. Materiales y métodos: El presente estudio fue diseñado como un estudio transversal, con base en un hospital, para evaluar el recuento de células CD4 y el perfil de lípidos en los pacientes infectados por VIH y SIDA en la población india y los correlaciona con los controles sero-negativos. La evaluación del perfil de lípidos se realizó utilizando Erba EM 360, un analizador automático impulsado por un fotómetro de rejilla de difracción, mientras que los recuentos de células CD4 se evaluaron utilizando el Contador de ciclo de Partec. Análisis estadístico utilizado: Los datos se analizaron con SPSS versión 15.0 (SPSS Inc., Chicago, IL, EE. UU.). La comparación de dichos parámetros se realizó mediante el análisis de varianza (ANOVA) y la prueba post-hoc de Games-Howell. Se consideró estadísticamente significativo un valor de p <0,05. Resultados: Los niveles de colesterol total y lipoproteínas de baja densidad (LDL) disminuyeron significativamente, mientras que los triglicéridos y las lipoproteínas de muy baja densidad (VLDL) aumentaron significativamente en los pacientes infectados por VIH y SIDA en comparación con los controles sero-negativos. Conclusión: El colesterol total, las LDL, los triglicéridos y las VLDL se alteraron significativamente en los pacientes infectados por VIH y con SIDA en comparación con los controles sero-negativos


Context and Aim: Hematological abnormalities are amongst the most common complications of infection with HIV.There have been quite a few studies on the alterations in lipid profile, too, though the results have largely been inconclusive. The present study was carried-out to assess CD4 cell counts and lipid profile in the HIV infected and AIDS patients in the Indian population and correlates them with the sero-negative controls. Materials and Methods: The present study was designed as a cross-sectional, hospital-based study to assess CD4 cell counts and lipid profile in the HIV infected and AIDS patients in the Indian population and correlates them with the sero-negative controls. Evaluation of lipid profile was done using Erba EM 360, an automated analyzer powered by a diffraction grating photometer while CD4 cell counts were evaluated using Partec Cyflow Counter. Statistical analysis used: The data was analyzed using SPSS version 15.0 (SPSS Inc., Chicago, IL, USA). Comparison of the said parameters was done using Analysis of Variance (ANOVA) and post-hoc Games-Howell test. p-value of <0.05 was considered statistically significant. Results: The levels of total cholesterol and low-density lipoproteins (LDLs) were significantly decreased while triglycerides and very low density lipoproteins (VLDLs) were significantly increased in the HIV infected and AIDS patients when compared with the sero-negative controls. Conclusion: Total cholesterol, LDLs, triglycerides and VLDLs were significantly altered in the HIV infected and AIDS patients when compared with the sero-negative controls


Subject(s)
Humans , Acquired Immunodeficiency Syndrome/complications , CD4 Lymphocyte Count , Dyslipidemias , Hematologic Diseases/complications
4.
Rev. cuba. invest. bioméd ; 40(2): e1119, 2021. tab
Article in Spanish | LILACS, CUMED | ID: biblio-1347454

ABSTRACT

Introducción: El desempeño competente del personal médico en medicina transfusional implica profundos conocimientos que no recibe durante el proceso formativo. Objetivo: Identificar elementos para la normalización de la competencia en medicina transfusional para la especialidad Anestesiología y Reanimación. Métodos: Estudio educacional de corte transversal. Se realizó un análisis documental sobre normalización de las competencias profesionales en Cuba y sobre los componentes de la competencia en medicina transfusional para la especialidad. Asimismo, se examinaron los contenidos y habilidades de medicina transfusional en el programa de la residencia. Finalmente, fueron entrevistados profesores de la especialidad. Resultados: La competencia abarca conocimientos sobre riesgos de transmisión de infecciones al paciente, prescripción segura de componentes sanguíneos, reconocimiento de las reacciones transfusionales, ejecución y enseñanza al no graduado de los procedimientos para la transfusión de sangre alogénica. Implica conocer y actualizarse en los aspectos éticos, científicos y legales involucrados. Incluye demostrar conocimientos, habilidades y valores para la preparación preoperatoria de los pacientes con anemia, riesgo hemorrágico y otras enfermedades hematológicas, para el empleo de alternativas y para el ahorro de componentes sanguíneos. Los profesores reconocieron que al comenzar la residencia es necesario un entrenamiento, para luego poder realizar una práctica transfusional segura. Conclusiones: Ser competente en medicina transfusional, subdisciplina de la especialidad Anestesiología y Reanimación, demanda movilizar un conjunto de conocimientos, habilidades, actitudes y valores de manera simultánea, interrelacionada y oportuna para transfundir menos y mejor, con más seguridad y menor costo(AU)


Introduction: Competent performance by the health care personnel in transfusion medicine implies deep knowledge not received during training. Objective: Identify elements of competence standardization in transfusion medicine for the specialty of anesthesiology and resuscitation. Methods: A cross-sectional educational study was conducted. A document analysis was carried out about standardization of professional competences in Cuba and about the components of competence in transfusion medicine for the specialty. Examination was also performed of transfusion medicine contents and skills in the residency program. Finally, professors from the specialty were interviewed. Results: Competence comprises knowledge about risks for the transmission of infections to patients, safe prescription of blood components, identification of transfusion reactions, and performance of and undergraduate training in allogeneic blood transfusion procedures. It implies knowledge and updated awareness of the ethical, scientific and legal aspects involved. It includes demonstrating knowledge, skills and values related to preoperative preparation of patients with anemia, hemorrhagic risk and other hematologic diseases, to use alternatives and save blood components. The professors recognized that training is required at the start of the residency program to ensure the performance of safe transfusion practices. Conclusions: Being competent in transfusion medicine, a subdiscipline of the specialty of Anesthesiology and Resuscitation, requires mobilization of a set of knowledge, skills, attitudes and values in a simultaneous, interrelated and timely fashion to transfuse less but better, with greater safety and at a lower cost(AU)


Subject(s)
Humans , Male , Female , Reference Standards , Blood Transfusion , Delivery of Health Care , Transfusion Medicine , Hematologic Diseases , Anesthesiology , Transfusion Reaction
6.
Med. interna (Caracas) ; 37(1): 26-30, 2021. ilus, tab
Article in Spanish | LILACS, LIVECS | ID: biblio-1253885

ABSTRACT

La enfermedad indiferenciada del tejido conectivo es una condición de etiología desconocida que comparte características clínicas, patológicas y de laboratorio de varias colagenosis, sin cumplir los criterios del Colegio Americano de Reumatología para el diagnóstico de una enfermedad reumática específica y muchos pacientes evolucionan a condiciones definidas a lo largo del tiempo tales como Lupus, Esclerosis sistémica progresiva, Enfermedad de Sjögren entre otros. Antecedentes: Linfoma Hodgkin diagnosticado desde 2012 para lo cual recibió múltiples esquemas de quimioterapia. Las muestras de ganglio y médula ósea se habían enviado al laboratorio de Inmunopatologia de la Universidad de Stanford y allí no se apreciaron hallazgos compatibles con enfermedad linfoproliferativa. Enfermedad actual: Mujer de 27 años de edad con cuadro clínico de 1 mes de evolución, caracterizado por edema blando en miembros inferiores acompañado de edema palpebral matutino; concomitantemente presenta aumento de temperatura intermitente sin patrón especifico y dolor osteomuscular generalizado con limitación para la deambulación. Se ingresa. Al examen físico, regulares condiciones clínicas. En la piel se aprecia engrosamiento cutáneo importante. Se realizó biopsia cutánea y los hallazgos fueron compatibles con Esclerosis Sistémica(AU)


Undifferentiated connective tissue disease is a condition of unknown etiology that shares clinical, pathological and laboratory characteristics of several collagenopathies that do not meet the criteria of the American College of Rheumatology for the diagnosis of a specific disease; a large number of patients evolve to conditions defined over time such as Lupus, Systemic Sclerosis, Sjogren's Disease, among others. Past history: Hodgkin lymphoma was diagnosed since 2012 for which she received multiple chemotherapy schemes. A gland biopsy was sent to the Stanford University, as well as a bone marrow sample, and lymphoma was discarded. Present history: this 27-year-old female consulted for edema in lower limbs present during one month, accompanied by eyelid edema in the mornings; also fever without a specific pattern, myalgias and arthralgias. On physical examination, the skin was thickened and limb edema was present. A skin biopsy was performed, and the findings were consistent with Systemic Sclerosis. The patient is receiving cyclophosphamide and Azathioprine and leading her normal life(AU)


Subject(s)
Rheumatology , Scleroderma, Systemic/diagnosis , Undifferentiated Connective Tissue Diseases/physiopathology , Hematologic Diseases , Biopsy , Diagnostic Imaging
7.
Article in Chinese | WPRIM | ID: wpr-921956

ABSTRACT

OBJECTIVE@#To explore the genotype-phenotype correlation in a child with Kabuki syndrome type 1 (KS1) caused by a mosaic frameshift variant of KMT2D gene.@*METHODS@#Trio-based whole exome sequencing (WES) was carried for the patient and her parents. Candidate variant was verified by Sanger sequencing.@*RESULTS@#The proband, a 3-year-and-2-month-old Chinese girl, presented with distinctive facial features, cognitive impairment, mild developmental delay, dermatoglyphic abnormalities, minor skeletal anomalies, ventricular septal defect, and autistic behavior. Trio-based WES revealed that the proband has carried a de novo mosaic frameshit variant of the KMT2D gene, namely NM_003482.3:c.13058delG (p.Pro4353Argfs*31) (GRCh37/hg19), for which the mosaicism rate was close to 21%. The variant was unreported previously and was confirmed by Sanger sequencing. Chromosomal microarray analysis (CMA) has revealed no pathogenic or likely pathogenic copy number variations. Compared with previously reported cases, our patient has presented obvious behavior anomalies including autism, anxiety and sleep problems, which were rarely reported.@*CONCLUSION@#This study has expanded the spectrum of KMT2D gene variants, enriched the clinical phenotypes of KS1, and facilitated genetic counseling for the family.


Subject(s)
Abnormalities, Multiple , China , DNA Copy Number Variations , DNA-Binding Proteins/genetics , Face/abnormalities , Female , Hematologic Diseases , Humans , Infant , Neoplasm Proteins/genetics , Phenotype , Vestibular Diseases
8.
Journal of Experimental Hematology ; (6): 1340-1345, 2021.
Article in Chinese | WPRIM | ID: wpr-888563

ABSTRACT

OBJECTIVE@#To investigate the clinical features, treatment and prognosis of patients with hematological diseases complicated with mucor infection.@*METHODS@#The risk factors, clinical features, treatment regimen and prognosis of 18 hematological disease patients with mucor infection diagnosed by histopathology in our center from April 2014 to June 2020 were retrospectively analyzed.@*RESULTS@#Thirteen males and five females, with an average age of 30 (13-54) years old, were diagnosed as mucor infection by histopathological examination at the site of infection, including 16 cases of mucor infection alone and 2 cases of mucor + aspergillus mixed infection. There were 12 cases with malignant hematological disease and 6 cases with severe aplastic anemia, all of whom with long-term agranulocytosis, and their clinical manifestations and imaging findings were not specific. The common sites of infection were sinuses and lungs, and some patients showed multiple systemic manifestations. The remission status of hematological diseases and recovery of immune function showed an impact on the prognosis. All the patients were treated with amphotericin B liposome combined with posaconazole, and 15 patients were treated with surgery combined with antifungal drugs, 9 of whom were effective and 6 were ineffective, while intravenous administration in 3 cases was ineffective.@*CONCLUSION@#It is difficult to diagnose hematological disease complicated with mucor infection. After early diagnosis, prognosis can be improved by amelioration of primary state and combination of drugs and surgery.


Subject(s)
Adolescent , Adult , Antifungal Agents/therapeutic use , Female , Hematologic Diseases/complications , Humans , Male , Middle Aged , Mucormycosis/drug therapy , Prognosis , Retrospective Studies , Young Adult
9.
Article in Chinese | WPRIM | ID: wpr-888387

ABSTRACT

OBJECTIVE@#Clinical examination and molecular genetic analysis were carried out for one case with special facial features with developmental retardation, hearing impairment and cleft lip and palate.@*METHODS@#The intelligence test, hearing test, and MRI test were performed. At the same time, the blood were collected to detect the copy number variation of the whole genome with the chromosomal karyotype analysis and the chromosomal microarray analysis (CMA). And the whole exome sequencing (WES) was used to analyze the pathogenic variant.@*RESULTS@#The children had mild mental retardation and the IQ was 61. There was moderate hearing loss in both ears(left ear 60 dB, right ear 65 dB). And bilateral horizontal hypoplasia of semicircular canal was found by cranial MRI test. No copy number abnormality was found by chromosome karyotype analysis and chromosome microarray analysis in peripheral blood. And whole exome sequencing suggested that there was heterozygous pathogenic variants in KMT2D gene (p.Leu545Argfs*385).@*CONCLUSION@#The patient has a peculiar face and multiple system defects, and was diagnosed as Niikawa-Kuroki syndrome type I by KMT2D gene variant. The whole exome sequencing is helpful for the diagnosis of complex genetic diseases.


Subject(s)
Abnormalities, Multiple , Child , Cleft Lip , Cleft Palate , DNA Copy Number Variations , Face/abnormalities , Hematologic Diseases , Humans , Vestibular Diseases
10.
Article in Chinese | WPRIM | ID: wpr-888374

ABSTRACT

OBJECTIVE@#To explore the genetic basis of a child with recurrent infection, multiple malformation and dysmorphism.@*METHODS@#The child and his parents were subjected to trio whole exome sequencing.@*RESULTS@#The child had a complaint of fever and cough, with long and thin eye fissures and long eyelashes. Genetic testing revealed that the child has carried a non-triplet deletion of the KDM6A gene, which was unreported previously. The variant resulted in frameshift and premature termination of the translation. His parents were both of the wild type for the locus. After antibiotic and immunoglobulin treatment, the severe secondary pneumonia caused by immunodeficiency has improved.@*CONCLUSION@#With combined laboratory test, imaging examination and genetic testing, the child was ultimately diagnosed with Kabuki syndrome type 2. The characteristics of immunodeficiency of Kabuki syndrome may render conventional antibiotic treatment ineffective, which deserves clinical attention.


Subject(s)
Abnormalities, Multiple , Child , DNA-Binding Proteins/genetics , Face/abnormalities , Genetic Testing , Hematologic Diseases , Histone Demethylases/genetics , Humans , Neoplasm Proteins/genetics , Nuclear Proteins/genetics , Phenotype , Pneumonia , Vestibular Diseases
11.
Article in Chinese | WPRIM | ID: wpr-880066

ABSTRACT

OBJECTIVE@#To analyze the distribution and drug resistance of pathogens sampled from the patients with bloodstream infection in the department of hematology of PLA General Hospital, so as to provide evidences for clinical prevention and control infection.@*METHODS@#From January 2014 to December 2017, A total of 286 cases-time positive blood culture samples from 212 patients in the department of hematology of the General Hospital of Chinese PLA were collected. The clinical characteristics of patients and the distribution and drug resistance of pathogens were analyzed retrospectively.@*RESULTS@#182(63.64%) bacterial strains were Gram-negative, and the other 104(36.36%) were Gram-positive. There were 88 strains of Escherichia coli(30.77%), 34 strains of Pseudomonas aeruginosa(11.89%), 26 strains of Klebsiella pneumoniae(9.09%), 25 strains of Staphylococcus epidermidis(8.74%), 20 strains of Gram-positive rods(6.99%), 16 strains of Staphylococcus hominis(5.59%), 11 strains of Etaphylococcus haemolyticus(3.85%), 10 strains of Staphylococcus aureus(3.50%), 6 strains of Staphylococcus capitis(2.10%), 5 strains of Acinetobacter baumannii(1.75%) and so on. Escherichia coli, Pseudomonas aeruginosa and Klebsiella pneumoniae as Gram-negative bacteria were sensitive to amikacin. Staphylococcus epidermidis and Staphylococcus aureus as Gram-positive bacteria were sensitive to vancomycin and nitrofurantoin.@*CONCLUSION@#The blood culture patients with bloodstream infection in department of hematology of our hospital confirmed that more infections are Gram-negative. The clinicians should choose suitable antibiotics according to the results of bacterial culture and drug sensitive test.


Subject(s)
Drug Resistance, Bacterial , Hematologic Diseases , Humans , Methicillin-Resistant Staphylococcus aureus , Microbial Sensitivity Tests , Retrospective Studies , Sepsis
12.
Rev. cuba. hematol. inmunol. hemoter ; 36(4): e1285, oct.-dic. 2020. tab
Article in Spanish | LILACS, CUMED | ID: biblio-1289413

ABSTRACT

Introducción: La neutropenia febril incide frecuentemente en pacientes con hemopatías malignas y representa una urgencia médica; actualmente su manejo terapéutico constituye un verdadero reto debido a la aparición de infecciones por microorganismos multirresistentes y la coexistencia de factores dependientes del paciente y del tratamiento, lo cual contribuye a situaciones de riesgo para el desarrollo de complicaciones graves. Métodos: Se realizó una revisión exhaustiva del tema en las principales bases de datos de la Biblioteca Virtual de Salud, se utilizó como referencia artículos actualizados publicados principalmente en los últimos 5 años. Análisis y síntesis de la información: Se abordó fundamentalmente el enfoque diagnóstico y terapéutico en los pacientes de alto riesgo de complicaciones por infecciones bacterianas, así como su evaluación integral. Se incluyen herramientas recientes de pesquisa de sepsis y daño orgánico relacionado con ella, que constituyen elementos predictivos de mortalidad en estos pacientes. Conclusiones: El abordaje integral de la neutropenia febril incluye además de una evaluación clínica y humoral exquisita, la aplicación de herramientas pronósticas para la estratificación de riesgo de cada paciente. El tratamiento de los pacientes debe comenzar en la primera hora de haberse documentado la fiebre, ya que las infecciones pueden ser rápidamente progresivas con un alto riesgo de desarrollo de sepsis, inestabilidad hemodinámica y disfunción multiorgánica(AU)


Introduction: Febrile neutropenia frequently affects patients with hematological malignancies and constitutes a medical emergency. Its therapeutic management is a real challenge at present, due to the appearance of infections caused by multiresistant microorganisms and the coexistence of patient- and treatment-dependent factors leading to risk for serious complications. Methods: An exhaustive review was conducted about the topic in the main databases of the Virtual Health Library, for which papers mainly published in the last five years were used as reference. Data analysis and synthesis: A diagnostic and therapeutic approach was applied to the study of patients at high risk for complications due to bacterial infections and their comprehensive evaluation. Recent tools to screen sepsis and sepsis-related organ damage are included which constitute mortality prediction elements in these patients. Conclusions: Comprehensive management of febrile neutropenia includes not only a detailed clinical and humoral evaluation, but also the application of prognostic tools for risk stratification in each patient. Patient treatment should be started within the first hour after fever documentation, since infections may be rapidly progressive with a high risk for the development of sepsis, hemodynamic instability and multiple organ dysfunction(AU)


Subject(s)
Humans , Febrile Neutropenia/diagnosis , Neutropenia/drug therapy , Hematologic Diseases/diagnosis
13.
Int. j. morphol ; 38(6): 1618-1622, Dec. 2020. tab, graf
Article in English | LILACS | ID: biblio-1134488

ABSTRACT

SUMMARY: The use of hematological counts for the prevention, diagnosis and follow-up of hematological diseases has increased. Indeed, the correct operation of a clinical laboratory is essential to producing comparable results. However, there is a paucity of validation and reproducibility studies among the different existing methods for clinical analysis. Therefore, our aim was to assess the commutability of the results provided by analyzers with different measuring systems. Sixty venous blood samples were obtained from patients, without discriminating for age or sex. Then, an automated hematological analysis was performed using the Cell-Dyn Ruby and HumaCount 5L instruments. The variables measured were: RBC, Hb, HCT, MCV, MCH and MCHC. The data were compared by a one-way ANOVA and Pearson's correlation coefficient. Statistical significance was fixed at p < 0.05. There were no statistically significant differences for RBC, HCT, MCH or MCHC. In addition, with the exception of MCHC, all the analytes showed a good correlation coefficient between the two instruments. There is a variety of automated systems for the clinical laboratory and it is essential for the clinician to know the different methodologies used in hematological analyzers as well as their sensitivity and specificity. Therefore, our results are useful for demonstrating the importance of practical knowledge of the analyzers mentioned.


RESUMEN: El uso de recuentos de células sanguíneas para la prevención, diagnóstico y monitoreo de enfermedades hematológicas ha ido en aumento. Por ello, el funcionamiento correcto de un laboratorio clínico es indispensable para producir resultados comparables. Sin embargo, existen pocos estudios de validación y reproducibilidad de los diferentes métodos de análisis clínico existentes. Por lo tanto, nuestro objetivo fue evaluar la intercambiabilidad de los resultados entregados por los analizadores que utilizan diferentes sistemas de medición. Se obtuvieron sesenta muestras de sangre venosa de pacientes, sin discriminar por edad o sexo. Los eritrogramas fueron obtenidos utilizando los analizadores automatizados Cell-Dyn Ruby y HumaCount 5L. Las variables medidas fueron: RBC, Hb, HCT, MCV, MCH y MCHC. Los datos fueron comparados por ANOVA a una vía y la correlación de Pearson. La significación estadística se estableció en el nivel estándar p<0,05. No hubo diferencias estadísticamente sig- nificativas para RBC, HCT, MCH y MCHC. Con la excepción de la MCHC, todos los analitos presentaron un buen coeficiente de correlación entre los dos analizadores comparados. Existen varios sistemas de automatización para su uso en laboratorios clínicos. Por lo tanto, es primordial para el clínico estar familiarizado con las diferentes metodologías utilizadas en los analizadores de sangre, así como su sensibilidad y especificidad. Nuestros resultados son útiles para mostrar la importancia del conocimiento práctico de los diferentes sistemas de medidas comparados.


Subject(s)
Humans , Hematologic Diseases/diagnosis , Hematologic Tests/methods , Blood Cell Count/methods , Blood Cells , Hemoglobins , Reproducibility of Results , Analysis of Variance , Sensitivity and Specificity , Erythrocyte Indices , Flow Cytometry , Hematocrit
15.
Rev. cuba. hematol. inmunol. hemoter ; 36(3): e1277, jul.-set. 2020. tab, graf
Article in Spanish | LILACS, CUMED | ID: biblio-1156444

ABSTRACT

Introducción: La aplasia medular adquirida grave es una enfermedad hematológica infrecuente caracterizada por una disminución o ausencia de precursores hematopoyéticos en la médula ósea, lo cual se expresa con distintos grados de citopenias. Varios factores, infecciosos o no, pueden incidir en su origen. Su manejo es complejo y puede incluir tratamiento inmunosupresor y trasplante de progenitores hematopoyéticos alogénico. Objetivo: Demostrar la utilidad de la realización del trasplante de progenitores hematopoyéticos alogénico haploidéntico en pacientes con aplasia medular grave. Caso clínico: Paciente masculino de 21 años de edad, con antecedentes de salud, que en octubre del 2018 debutó con íctero, pancitopenia, lesiones purpúrico hemorrágicas en piel y mucosas, en el curso de una hepatitis aguda seronegativa. La biopsia de médula ósea mostró aplasia medular severa. Se inició tratamiento inmunosupresor con globulina antitimocίtica, ciclosporina A y metilprednisolona. Al cabo de los 6 meses mantenía trombocitopenia severa con necesidades transfusionales y en octubre de 2019 se decide realizar trasplante de progenitores hematopoyéticos alogénico con donante haploidéntico y empleando como tratamiento acondicionante globulina antitimocίtica, fludarabina, ciclofosfamida y bajas dosis de irradiación corporal total. En evaluación clίnica de julio de 2020 (dίa + 280 del trasplante) el paciente estaba asintomático y con parámetros hematológicos normales. Conclusiones: Se demostró que el trasplante de progenitores hematopoyéticos alogénico haploidéntico es un proceder realizable y útil en pacientes con aplasia medular grave, lo cual corrobora el beneficio clínico que puede aportar su ejecución en pacientes con esta enfermedad(AU)


Introduction: Acquired severe marrow aplasia is a rare hematological disease characterized by decrease or absence of hematopoietic precursors in bone marrow, which is expressed with different degrees of cytopenias. Several factors, infectious or not, can influence its origin. Its management is complex and may include immunosuppressive treatment and allogeneic hematopoietic stem-cell transplantation. Objective: To demonstrate the usefulness of performing haploidentical allogeneic hematopoietic stem-cell transplantation in patients with severe medullary aplasia. Clinical case: A 21-year-old male patient, with medical history, who first presented, in October 2018, with icterus, pancytopenia, as well as purpuric hemorrhagic lesions on the skin and mucosa, in the course of acute seronegative hepatitis. The bone marrow biopsy showed severe marrow aplasia. Immunosuppressive treatment was started with antithymocytic globulin, cyclosporine A, and methylprednisolone. After six months, he maintained severe thrombocytopenia under transfusion requirements and, in October 2019, the decision was to perform allogeneic hematopoietic stem-cell transplantation with a haploidentical donor and using antithymocyte globulin, fludarabine, cyclophosphamide, and low doses of total body irradiation as conditioning treatment. In the clinical assessment carried out in July 2020 (day +280 after transplantation), the patient was asymptomatic and with normal hematological parameters. Conclusions: Transplantation of haploidentic allogeneic hematopoietic progenitors was shown to be a feasible and useful procedure in patients with severe marrow aplasia, which corroborates the clinical benefit that its execution can bring in patients with this disease(AU)


Subject(s)
Humans , Male , Young Adult , Tissue Donors/ethics , Methylprednisolone/therapeutic use , Whole-Body Irradiation/methods , Microscopy, Electron, Scanning Transmission/methods , Hematologic Diseases , Hematopoietic Stem Cell Transplantation/methods , Cuba , Transplantation, Haploidentical/methods , Anemia, Aplastic/therapy , Antilymphocyte Serum
16.
Rev. ecuat. pediatr ; 21(2): 1-7, 31 de agosto del 2020.
Article in Spanish | LILACS | ID: biblio-1140935

ABSTRACT

Introducción: La esplenectomía es un tratamiento estandarizado en niños con trombocitopenia. El método de laparoscopía, en este tratamiento, minimiza los procesos post-operatorios y se ha difundido su aplicación en la comunidad científica. El objetivo del presente estudio es realizar una descripción de la casuística y utilidad de la esplenectomía laparoscópica en los niños con patología hematológica. Métodos: El presente estudio observacional, retrospectivo se realizó en el Hospital Pediátrico Baca Ortiz. Se revisaron expedientes clínicos de los últimos 10 años de pacientes con indicación de esplenectomía quirúrgica. Se analizan variables demográficas, clínicas y de resultados. Se utiliza estadística descriptiva. Resultados: Ingresaron al estudio 14 pacientes que tuvieron una esplenectomía quirúrgica vía laparoscópica. La mayoría de estos pacientes son del sexo femenino, con patologías hematológicas como esferocitosis y púrpura trombocitopénica idiopática (PTI). En el 50% se realizó colecistectomía además de esplenectomía. El tiempo quirúrgico varió de 60 a 120 minutos. Conclusiones: La esplenectomía laparoscópica es considerada una técnica compleja dentro de los procedimientos de laparoscopia, pero es ideal para los pacientes con patología hematológica, por lo que es la técnica de elección. Una ventaja de la esplenectomía laparoscópica es el menor tiempo de recuperación y hospitalización, con heridas quirúrgicas más pequeñas.


Introduction: Splenectomy is a standardized treatment in children with thrombocytopenia. The laparoscopic method, in this treatment, minimizes post-operative processes and its application has become widespread in the scientific community. The objective of this study is to describe the casuistry and usefulness of laparoscopic splenectomy in children with hematological pathology. Methods: This retrospective, observational study was conducted at Baca Ortiz Pediatric Hospital. Medical records of the last 10 years of patients with an indication for surgical splenectomy were reviewed. Demographic, clinical and outcome variables are analyzed. Descriptive statistics are used. Results: Fourteen patients who had a laparoscopic surgical splenectomy entered the study. Most of these patients are female, with hematological pathologies such as spherocytosis and idiopathic thrombocytopenic purpura (ITP). In 50% a cholecystectomy was performed in addition to splenectomy. The surgical time ranged from 60 to 120 minutes. Conclusions: Laparoscopic splenectomy is considered a complex technique within laparoscopic procedures, but it is ideal for patients with hematological pathology, so it is the technique of choice. An advantage of laparoscopic splenectomy is the shorter recovery time and hospitalization, with smaller surgical wounds


Subject(s)
Humans , Splenectomy , Hematologic Diseases
17.
Rev. chil. pediatr ; 91(4): 597-604, ago. 2020. tab, graf
Article in Spanish | LILACS | ID: biblio-1138677

ABSTRACT

Resumen: Cada vez es más frecuente la atención médica en la Unidad de Cuidados Intensivos (UCI) de niños o adolescentes inmigrantes como también de aquellos nacidos en nuestro país con padres en tal condición. Esto ha ocasionado, en la actualidad, que el equipo de salud se deba enfrentar con problemas diagnósticos derivados del escaso conocimiento de condiciones genéticas propias de esta población y/o el desarrollo de diversas patologías infrecuentes en nuestro país, algunas resultantes de su condi ción sanitaria. En esta revisión se abordan diversos aspectos de la patología hematológica, infecciosa, parasitaria, respiratoria y cardiovascular, todos tópicos relevantes de conocer durante su estadía en la UCI. Es un deber del equipo de salud actualizarse sobre patologías de baja prevalencia en nuestro país, algunas de ellas muy poco conocidas hasta hace una década, pero que, actualmente, están cada vez más presentes en las UCI del sistema de salud público chileno.


Abstract: It is increasingly common to provide medical care in the Intensive Care Unit (ICU) for immigrant children and adolescents as well as those born in Chile with parents in such condition. Currently, this has caused that the health team has to face diverse infrequent pathologies in our country and/ or diagnostic problems derive from the poor knowledge of genetic conditions of this population, some resulting from their health conditions. This review addresses several aspects of hematological, infectious, parasitic, respiratory, and cardiovascular pathologies, all relevant topics to know during their stay in the ICU. It is a duty of the health team to be updated on pathologies of low prevalence in our country, some of them very little known until a decade ago, but which are currently increasingly present in the ICUs of the Chilean public health system.


Subject(s)
Humans , Infant, Newborn , Infant , Child, Preschool , Child , Adolescent , Respiratory Tract Diseases/diagnosis , Respiratory Tract Diseases/ethnology , Respiratory Tract Diseases/therapy , Cardiovascular Diseases/diagnosis , Cardiovascular Diseases/ethnology , Cardiovascular Diseases/therapy , Critical Care/methods , Emigrants and Immigrants , Hematologic Diseases/diagnosis , Hematologic Diseases/ethnology , Hematologic Diseases/therapy , Infections/diagnosis , Infections/ethnology , Infections/therapy , Intensive Care Units , Chile/epidemiology , Prevalence
18.
Rev. méd. panacea ; 9(2): 82-89, mayo-ago. 2020. tab, graf
Article in Spanish | LIPECS, LILACS, LIPECS | ID: biblio-1121154

ABSTRACT

Objetivo: Describir los hallazgos hematológicos en donantes preseleccionados a plaquetoferesis sanguínea en el Hospital Nacional Edgardo Rebagliati Martins (HNERM) de Lima, Perú. Materiales y métodos: Estudio de tipo observacional, descriptivo y transversal, realizado entre Mayo-Julio 2019. Se incluyeron hemogramas de donantes que resultaron ̈Aptos ̈ a la entrevista médica, con resultado ̈Negativo ̈ a marcadores serológicos y que fueron diferidos en donar por presentar alteración hematológica. Los datos fueron recolectados del sistema informático del servicio de medicina transfusional del HNERM. Se utilizó estadística descriptiva y analítica (U de Mann Whitney y Chi-cuadrado de Pearson) considerándose un valor p<0,05 como significativo. Resultados: Se analizaron los hemogramas de 330 donantes preseleccionados diferidos, entre los cuales el recuento absoluto de eosinófilos (14,2%), el hematocrito (14,2%) y el recuento de plaquetas (33,9%) fueron las de mayor presencia. Asimismo, en las observaciones la fórmula leucocitaria invertida (20,6%) fue la más frecuente. Además, se observó diferencia estadística significativa (p<0,05) entre la hemoglobina, el hematocrito, la hemoglobina corpuscular media (HCM) y la fórmula leucocitaria invertida de acuerdo al sexo del donante. Conclusiones: La alteración hematológica observada más frecuentemente en esta población fue la plaquetopenia. Además, algunos parámetros de la serie roja y la distribución leucocitaria en el hemograma estuvieron asociados con el género del donante. (AU)


Objective: To describe the hematological findings in preselected donors to blood plateletpheresis at the Edgardo Rebagliati Martins National Hospital (HNERM) in Lima, Peru. Materials and methods: Observational, descriptive and cross-sectional study, carried out between May-July 2019. Blood counts were included from donors that were ̈Approved ̈ to the medical interview, with a ̈Negative ̈ result for serological markers and that were deferred in donating for present hematological alteration. The data was collected from the computer system of the HNERM transfusion medicine service. Descriptive and analytical statistics (Mann Whitney's U and Pearsonʼs Chi-square) were used, considering a p<0.05 as significant. Results: The blood counts of 330 delayed preselected donors were analyzed, among which the absolute eosinophil count (14.2%), the hematocrit (14.2%) and the platelet count (33.9%) were those with the highest presence. Likewise, in observations, the inverted white blood cell formula (20.6%) was the most frequent. In addition, a statistically significant difference (p<0.05) was observed between hemoglobin, hematocrit, mean corpuscular hemoglobin (MCH) and the inverted leukocyte formula according to the sex of the donor. Conclusions: The hematological alteration most frequently observed in this population was plateletpenia. In addition, some parameters of the red series and the leukocyte distribution on the blood count were associated with the gender of the donor. (AU)


Subject(s)
Humans , Male , Female , Blood Cell Count , Blood Donors , Plateletpheresis , Hematologic Diseases , Epidemiology, Descriptive , Cross-Sectional Studies , Observational Studies as Topic
20.
Rev. cuba. hematol. inmunol. hemoter ; 36(2): e1042, abr.-jun. 2020.
Article in Spanish | LILACS, CUMED | ID: biblio-1149893

ABSTRACT

Introducción: El dolor como experiencia subjetiva desagradable es un síntoma frecuente en los pacientes atendidos en el servicio de Hematología. Este afecta al individuo a nivel psicológico y provoca la aparición de dificultades en las áreas laboral, educacional, familiar y en las relaciones interpersonales. La percepción del dolor puede verse distorsionada como resultado de esta alteración provocada en el paciente. Objetivo: Analizar los factores psicológicos que median la percepción del dolor de los pacientes. Métodos: Se realizó una revisión de la literatura, a través del sitio web PubMed y el motor de búsqueda Google Académico. Se emplearon las palabras clave: dolor, manejo del dolor, evaluación del dolor, psicología del dolor, dolor en hematología, dolor en drepanocitosis, dolor en hemofilia, dolor en leucemia. Análisis y síntesis de la información: Se evidencia el carácter multifacético de esta experiencia subjetiva. Los determinantes y mecanismos del dolor son diversos, lo cual implica que su enfrentamiento requiera un enfoque integral que ayude a los pacientes a desarrollar conciencia acerca de los efectos de la enfermedad que padece y los síntomas del dolor. También es necesario guiarlos en la comprensión de los factores que contribuyen a su magnificación, crear estrategias que les ayuden a minimizar la exposición a estos, y orientarlos para que desarrollen métodos personales que les permitan lidiar con el dolor con un mínimo estrés psicológico. Conclusiones: El dolor es un fenómeno complejo que involucra diversas variables y factores en su funcionamiento. Su manejo requiere un enfoque integral para la atención de los pacientes en su enfrentamiento al dolor(AU)


Introduction: Pain as an unpleasant subjective experience is a frequent symptom in patients treated by the Hematology service. This affects the individual on a psychological level and causes the onset of difficulties in the work, educational, family and interpersonal relationships. The perception of pain can be distorted as a result of this alteration caused in the patient. Objective: To analyze the psychological factors that mediate patients' perception of pain. Methods: A literature review was carried out through the PubMed website and the search engine of Google Scholar. The following keywords were used: dolor [pain], manejo del dolor [pain management], evaluación del dolor [pain assessment], psicología del dolor [pain psychology], dolor en hematología [pain in hematology], dolor en drepanocitosis [pain in sickle cell disease], dolor en hemofilia [pain in hemophilia], dolor en leucemia [pain in leukemia]. Information analysis and synthesis: The multifaceted nature of this subjective experience is evidenced. Pain determinants and mechanisms are diverse, which implies that their coping requires a comprehensive approach that helps patients develop awareness about the disease effects and the pain symptoms. It is also necessary to guide them in understanding the factors that contribute to pain magnification, create strategies that help them minimize pain exposure, and guide them to develop personal methods that allow them to deal with pain with minimal psychological stress. Conclusions: Pain is a complex phenomenon involving several variables and factors in its operation. Its management requires a comprehensive approach to patient care regarding coping with pain(AU)


Subject(s)
Humans , Pain Measurement/methods , Pain Measurement/psychology , Pain Perception/physiology , Pain Management/psychology , Hematologic Diseases/psychology , Hematology
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