Polimorfismos genéticos implicados en el desarrollo de la purpura trombocitopénica inmune / Genetic polymorphisms involved in the development of Immune Thrombocytopenic Purpura

Introducción: los cambios en el ácido desoxirribonucleico se conocen como mutaciones, estas dan lugar a los polimorfismos, los cuales generan variación alélica entre individuos y diversidad de la misma especie. Se ha sugerido que los polimorfismos genéticos en los mediadores inmunitarios desempeñan un papel fundamental en la patogénesis de muchos trastornos autoinmunes, como en la púrpura trombocitopénica inmune, siendo esta el tipo más común de púrpura trombocitopénica y, a menudo, se diagnostica como un tipo de trastorno autoinmune, debido a la destrucción de las plaquetas mediadas por el sistema inmunitario. Objetivo: realizar una revisión bibliográfica sobre el papel de los polimorfismos genéticos y su influencia en el desarrollo de la púrpura trombocitopénica inmune. Métodos: se realizó revisión literaria en inglés y español en PubMed y Elsevier, desde marzo hasta mayo del 2021, con el uso de combinación de palabras clave y términos MeSH, como púrpura trombocitopénica y polimorfismos genéticos. Se realizó análisis y resumen de la literatura encontrada. Conclusión: la púrpura trombocitopénica inmune es considerada como una patología multifactorial, causada por factores ambientales y genéticos, dentro de los cuales se encuentran los polimorfismos para los mediadores inmunitarios que pueden llevar a una exacerbación de la enfermedad o no intervenir en la misma.

Introduction: Changes in deoxyribonucleic acid are known as mutations, these give place to polymorphisms, which generate allelic variation between individuals and provide diversity among same species. Genetic polymorphisms in immune mediators have been suggested to play a key role in the pathogenesis of many autoimmune disorders, such as immune thrombocytopenic purpura, this being the most common type of thrombocytopenic purpura and is often diagnosed as a type of autoimmune disorder, due to the destruction of platelets mediated by the immune system. Objective: To execute a bibliographic review on the role of genetic polymorphisms and their influence on the development of immune thrombocytopenic purpura. Methods: A literary review in English and Spanish was performed in PubMed and Elsevier from March to May 2021, with the use of a combination of keywords and MeSH terms such as Thrombocytopenic Purpura and genetic polymorphisms. Analysis and summary of the literature found was executed. Conclusion: Immune thrombocytopenic purpura is considered a multifactorial pathology, caused by environmental and genetic factors, among which are polymorphisms for immune mediators that can lead to an exacerbation of the disease or not intervene in the same.

Poor Oral Health Increases the Physical Pain, Psychological Discomfort and Social Incapacity of Individuals with Oncohaematological Diseases

ABSTRACT: The objective of this study was to evaluate the relationship between oral health status and its impact on quality of life, and to suggest dental management strategies in patients undergoing antineoplastic therapy for onco-hematological diseases. A retrospective study including 33 individuals (age 9-79 yr) was conducted. It was observed that the dimensions related to physical pain, psychological discomfort, and social incapacity had statistically significant values. The most frequently performed dental treatments were periodontal treatment (45.45 %), dental restoration (36.36 %), tooth extractio n (33.33 %), and endodontic treatment (24.24 %). Thus, poor oral health directly affects the quality of life. Dental management should consider the aspects of the disease and antineoplastic treatment while aiming for safe and effective dental care.

RESUMEN: El objetivo de este estudio fue evaluar la relación entre el estado de salud oral y su impacto en la calidad de vida, y sugerir estrategias de manejo odontológico en pacientes sometidos a terapia anti-neoplásica por enfermedades onco-hematológicas. Se realizó un estudio retrospectivo que incluyó a 33 personas (de 9 a 79 años de edad). Se observó que las dimensiones relacionadas con dolor físico, malestar psicológico e incapacidad social tuvieron valores estadísticamente significativos. Los tratamientos dentales realizados con mayor frecuencia fueron el tratamiento periodontal (45,45 %), la restauración dental (36,36 %), la extracción dentaria (33,33 %) y el tratamiento endodóntico (24,24 %). Así, la mala salud oral afecta directamente la calidad de vida. El tratamiento dental debe tener en cuenta los aspectos de la enfermedad y el tratamiento antineoplásico mientras se busca una atención dental segura y eficaz.

Esplenectomía Laparoscópica en el Hospital Pediátrico Baca Ortiz, estudio retrospectivo de los últimos 10 años / Laparoscopic splenectomy at Baca Ortiz Pediatric Hospital, a retrospective study of the last 10 years. Observational Study

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

Establishment of the review criteria of blood cell analysis in children and its application in clinical diagnosis / 中华检验医学杂志

Objective@#To establish the review criteria for children′s blood cell analysis, ensure the accuracy of blood cell analysis results, and ensure that pathological cells are not missed.@*Methods@#A total of 1 420 samples of blood cell analysis were collected from outpatients and inpatients in Shanxi Children′s Hospital from May to June 2018, which were detected by SYSMEXXN-350 and XN-A1 automated blood cell analyzer. Blood smears weredouble-blindly examinedunder microscope. Among them, 463 were used for the establishment of review criteria, 586 were used for the verification and evaluation of review criteria, and 371 were used for the application effect study of review criteria. According tothe 41 rules recommended by ISLH, combining the characteristics of children′s physiology, pathology, disease and abnormal alarming information of hematology analyzer, the review criteria suitable for children′s blood cells were established.@*Results@#Through the evaluation and optimization of 41 rules recommended by ISLH, 23 rules for reexamination of children′s blood cell analysis were formulated, with a reexamination rate of 25.09%, and a false positive rate was 14.16%. A total of 371 samples of patients with hematological diseases were selected for the application of the review criteria. The false negative rate was 2.96%, and no pathological cells were missed.@*Conclusion@#The children′s blood cell review criteria established in this study has been verified and evaluated, which not only ensures the quality of the report, but also improves the work efficiency, and provides an important basis for the diagnosis and differential diagnosis of childhood leukemia, infectious mononucleosis and other hematological diseases.

Research progress on regulation and construction of bone marrow hematopoietic niche by traditional Chinese medicine / 中草药

The bone marrow hematopoietic niche is the place where hematopoietic stem cells and progenitors survive. It is not only related to the hematopoiesis function, but also its steady-state disorders and abnormalities can induce a variety of hematologic diseases, which will even be adapted by malignant cells such as leukemia stem cells, so as to cause seriously poor prognosis. Therefore, it is of great significance to the regulation and construction of the bone marrow niche. In this review, we outline the composition, physiological and pathological basis of bone marrow hematopoietic niche, from the regulation of Chinese materia medica components and TCM formula to graphically analyze the target and effect of drug efficacy. Then, combined with the clinical practice for integrating and summarizing decentralized research, aiming to provide new directions and perspectives for the TCM research and treatment of hematologic diseases.

Retrospective study on the prevention of infection in patients with hematological malignancies after chemotherapy in a class 100 laminar flow ward / 中国感染控制杂志

Objective To explore the value of class 100 laminar flow ward in the prevention of infection in patients with hematological malignancies(HM) after chemotherapy.Methods Patients with HM and received chemotherapy in the department of hematology in a hospital from March 2016 to February 2017 were surveyed retrospectively,according to patients' wishes,those who were admitted to the class 100 laminar flow ward and received chemotherapy were as trial group,and those who were admitted to the common ward and received chemotherapy were as control group.The incidence of infection,duration of fever,antimicrobial use time,length of hospital stay,and index of infection were compared and analyzed between two groups.Results A total of 267 patients with HM received chemotherapy,74 cases in trial group and 193 in control group.During the chemotherapy period,incidence of infection in trial group was lower than that of control group (47.3％ vs 72.0％,P＜0.001).Respiratory tract,digestive tract,and urinary tract were main infection sites in both groups.A total of 45 strains of pathogens were isolated from two groups of patients,7 strains were isolated from trial group and 38 from control group.The isolated pathogens were Escherichia coli,Klebsiella pneumoniae,Stenotrophomonas maltophilia,Pseudomonas aeruginosa,and yeast.Duration of fever,antimicrobial use time,and length of hospital stay in trial group were all lower than control group (all P＜0.05);serum procalcitonin (PCT) and C-reactive protein (CRP) levels in trial group were both lower than control group(both P＜0.01),the time for PCT and CRP to return to normal in trial group were both lower than control group(both P＜0.05).Conclusion Patients with MH and receive chemotherapy in class 100 laminar flow ward can reduce the incidence of infection,shorten the length of stay,and reduce the economic burden,it is worthy of further clinical promotion.

Invasive fungal infection in patients with hematologic disorders in a Brazilian tertiary care hospital

ABSTRACT INTRODUCTION: Invasive fungal infections (IFIs) are an important complication in immunocompromised individuals, particularly neutropenic patients with hematological malignancies. In this study, we aimed to verify the epidemiology and diagnosis of IFIs in patients with hematologic problems at a tertiary hospital in Goiânia-GO, Brazil. METHODS: Data from 117 patients, involving 19 cases of IFIs, were collected. The collected data included diagnosis methods, demographics, clinical characteristics, and in vitro susceptibility to different antifungal agents. Among the 19 cases, 12 were classified as proven IFI and 7 as probable invasive aspergillosis with detection of galactomannan in blood and presence of lung infiltrates in radiographic images. Logistic regression analysis showed that the proven and probable IFIs were associated with increased risk of death. Statistical analysis demonstrated that age, sex, and underlying disease were not independently associated with risk of death in IFI patients. RESULTS: Most bloodstream isolates of Candida spp. exhibited low minimum inhibitory concentrations (MICs) to all antifungal agents tested. Voriconazole and amphotericin had the lowest MICs for Aspergillus spp. and Fusarium spp., but Fusarium spp. showed the least susceptibility to all antifungals tested. Amphotericin B, fluconazole, and itraconazole were found to be inactive in vitro against Acremonium kiliense; but this fungus was sensitive to voriconazole. CONCLUSIONS: Considering the high number of IFI cases, with crude mortality rate of 6%, we could conclude that IFIs remain a common infection in patients with hematological malignancies and underdiagnosed ante mortem. Thus, IFIs should be monitored closely.

Distribution and drug resistance of pathogens at hematology department of Jiangsu Province from 2014 to 2015: results from a multicenter, retrospective study / 中华血液学杂志

Objective@#To describe the distribution and drug resistance of pathogens at hematology department of Jiangsu Province from 2014 to 2015 to provide reference for empirical anti-infection treatment.@*Methods@#Pathogens were from hematology department of 26 tertiary hospitals in Jiangsu Province from 2014 to 2015. Antimicrobial susceptibility testing was carried out according to a unified protocol using Kirby-Bauer method or agar dilution method. Collection of drug susceptibility results and corresponding patient data were analyzed.@*Results@#The separated pathogens amounted to 4 306. Gram-negative bacteria accounted for 64.26%, while the proportions of gram-positive bacteria and funguses were 26.99% and 8.75% respectively. Common gram-negative bacteria were Escherichia coli (20.48%) , Klebsiella pneumonia (15.40%) , Pseudomonas aeruginosa (8.50%) , Acinetobacter baumannii (5.04%) and Stenotropho-monas maltophilia (3.41%) respectively. CRE amounted to 123 (6.68%) . Common gram-positive bacteria were Staphylococcus aureus (4.92%) , Staphylococcus hominis (4.88%) and Staphylococcus epidermidis (4.71%) respectively. Candida albicans were the main fungus which accounted for 5.43%. The rates of Escherichia coli and Klebsiella pneumonia resistant to carbapenems were 3.5%-6.1% and 5.0%-6.3% respectively. The rates of Pseudomonas aeruginosa resistant to tobramycin and amikacin were 3.2% and 3.3% respectively. The resistant rates of Acinetobacter baumannii towards tobramycin and cefoperazone/sulbactam were both 19.2%. The rates of Stenotrophomonas maltophilia resistant to minocycline and sulfamethoxazole were 3.5% and 9.3% respectively. The rates of Staphylococcus aureus, Enterococcus faecium and Enterococcus faecalis resistant wards vancomycin were 0, 6.4% and 1.4% respectively; also, the rates of them resistant to linezolid were 1.2%, 0 and 1.6% respectively; in addition, the rates of them resistant to teicoplanin were 2.8%, 14.3% and 8.0% respectively. Furthermore, MRSA accounted for 39.15% (83/212) .@*Conclusions@#Pathogens were mainly gram-negative bacteria. CRE accounted for 6.68%. The rates of Escherichia coli and Klebsiella pneumonia resistant to carbapenems were lower compared with other antibacterial agents. The rates of gram-positive bacteria resistant to vancomycin, linezolid and teicoplanin were still low. MRSA accounted for 39.15%.

Fluoroscopy-guided subclavian vein catheterization in children with hematologic diseases: methodology study / 介入放射学杂志

Objective To investigate the success rate of fluoroscopy-guided subclavian vein catheter implantation (SVCI) in children with hematologic diseases,to improve the visualization of the position of the catheter head,and to reduce the incidence of procedure-related complications.Methods Fluoroscopyguided SVCI was performed in 183 sick children (aged 1-16 years) with confirmed hematologic disease.The success rate of the catheter implantation,the number of needle puncturing,the operation time,the fluoroscopy time and the occurrence of procedure-related complications were recorded.Results Successful fluoroscopy-guided SVCI was accomplished in all 183 sick children,with a success rate being 100％.Successful SVCI was obtained with ＜3 times of puncturing in 151 sick children (82.5％),with 4-6 times of puncturing in 25 sick children,and with 7-10 times of puncturing in 7 sick children.The catheter tip was successfully positioned at the junction of the superior vena cava with the right atrium in all sick children.The operation time ranged from 5 min to 25 min with a mean of (10.38±4.04) min.The fluoroscopy time varied from 16 seconds to 607 seconds with a mean of (65.46±55.86) seconds.During the procedure,artery was wrongly punctured two times in two sick children.The mean follow-up time was 35 days.Cather-related infection occurred in 2 sick children.No local hematoma at puncture point,nor hemopneumothorax or catheter-related thrombosis occurred.Conclusion Fluoroscopy-guided SVCI has high technical success rate in children with hematologic diseases.For a successful procedure of SVCI,less number of needle puncturing is needed by using this technique.The satisfaction rate for the placement of catheter tip is high and the incidence of complications is low.Therefore,fluoroscopy-guided SVCI is a safe and effective method.

Distribution and antimicrobial resistance of clinically isolated pathogens in hematology department from 2011 to 2013 / 中国感染控制杂志

Objective To analyze the distribution and antimicrobial resistance of pathogens isolated from department of hematology during the past three years.Methods Pathogenic strains isolated from patients hospitalized in a hematology de-partment between January 2011 and December 2013 were collected,antimicrobial susceptibility testing was performed by Kirby-Bauer disk diffusion method or automatic system,antimicrobial susceptibility testing results were judged according to American Clinical and Laboratory Standards Institute 2011, data were analyzed by WHONET 5.6 software. Results A total of 462 clinical isolates were collected in 2011—2013,including 161 gram-positive cocci isolates,279 gram-negative bacilli,and 22 fungi.Of Staphylococcus spp ,detection rate of methicillin-resistant coagulase negative Staphylo-coccus(MRCNS)and methicillin-resistant Staphylococcus aureus (MRSA)was 81.37% and 62.50%respectively.The re-sistant rate of Staphylococcus spp .and Enterococcus spp .to linezolid was 1.69% and 3.57% respectively,resistant rate of Staphylococcus spp .to teicoplanin was 3.39%,vancomycin-resistant gram-positive coccus was not found.Enterobacte-riaceae strains Escherichia coli and Klebsiella pneumoniae were highly susceptible to carbapenems,the sensitivity rates were 97.56%—98.88%;while nonfermentative gram-negative bacilli Pseudomonas aeruginosa and Acinetobacter bauman-nii strains were obviously resistant to carbapenems,the resistance rates were 38.71%—64.00%.Conclusion Antimicrobial resistance of major pathogenic strains from hematology department is high,antimicrobial agents should be used according to pathogenic distribution characteristics and antimicrobial susceptibility testing results,healthcare-associated infection control should be strengthened to reduce antimicrobial resistant rate.

Valoración de índices plaquetarios en las trombocitopenias / Valuation of platelet índices in thrombocytopenia / Avaliagáo de índices plaquetários nas trombocitopenias

El objetivo de este trabajo fue determinar la utilidad clínica de los índices plaquetarios en la caracterización etiológica de las trombocitopenias. Se trata de un estudio descriptivo, retrospectivo y transversal. En pacientes controles se establecieron valores de referencia para número de plaquetas e índices plaquetarios, y éstos se evaluaron en pacientes donde coexistía trombocitopenia con algún desorden oncohematológico (linfoma no Hodgkin, linfoma Hodgkin, leucemia aguda, leucemia crónica, síndrome mielodisplásico, púrpura trombocitopénica inmune). La evaluación de laboratorio fue realizada al momento del diagnóstico, aún libre de tratamiento. En los casos de Púrpura Inmune (disminución de Volumen Plaquetario - Plaquetocrito, y aumento de Amplitud Plaquetaria); Leucemia Mieloide Crónica (aumento de la Amplitud Plaquetaria) y Linfoma no Hodgkin o Síndrome Mielodisplásico (disminución del Plaquetocrito), los índices plaquetarios podrían ser usados como herramienta diagnóstica orientadora. En cambio, no podrían contribuir al momento de diferenciar entre leucemias agudas, dado que no presentan diferencias significativas. Frente a un diagnóstico presuntivo de síndrome mielodiasplásico o leucemia mieloide aguda, el valor de Volumen Plaquetario Medio (VPM) podría contribuir como herramienta orientadora al diagnóstico, ya que sería más bajo en la leucemia aguda. El análisis de los resultados sugiere que en la práctica clínica los índices plaquetarios podrían contribuir de un modo significativo a la confirmación del diagnóstico.

The aim of this study was to determine the clinical utility of platelet indices in the etiological characterization of thrombocytopenia. It was a descriptive, retrospective and cross-sectional study. In control patients, reference values (platelet count and platelet indices) were established and they were used to assess platelet indices in patients where thrombocytopenia coexisted with some oncohematologic disorders (non-Hodgkin Lymphoma, Hodgkin Lymphoma, acute leukemia, chronic leukemia, myelodysplastic syndrome, immune thrombocytopenic purpura). Laboratory evaluation was performed at still treatment-free diagnosis. In the cases of Immune Purpura, (decreased platelet volume- plateletcrit, and increased platelet distribution width) Chronic Myeloid Leukemia (increased platelet distribution width) and non-Hodgkin lymphoma or myelodysplastic syndrome (decreased plateletcrit), platelet indices could be used as a "guiding diagnostic tool". However, they could not contribute to the differenciation between acute leukemias since they do not present any significant differences. In view of a presumptive diagnosis of mielodysplastic syndrome or acute myeloid leukemia, mean platelet value (MPV) could contribute to the diagnosis, since it would be lower in acute leukemia. The analysis of the results suggests that in clinical practice, platelet indices may contribute significantly to the confirmation of the diagnosis.

O objetivo deste trabalho foi determinar a utilidade clínica dos índices plaquetários na caracterizagáo etiológica das trombocitopenias. Trata-se de um estudo descritivo, retrospectivo e transversal. Em pacientes controle foram estabelecidos valores de referencia para número de plaquetas e índices plaquetários, e eles foram avaliados em pacientes onde coexistia trombocitopenia com alguma desordem onco-hematológica (linfoma náo Hodgkin, linfoma Hodgkin, leucemia aguda, leucemia crónica, síndrome mielodisplásica, púrpura trombocitopenia imune). A avaliagáo de laboratório foi realizada no momento do diagnóstico, ainda livre de tratamento. Nos casos de Púrpura Imune (diminuigáo de Volume Plaquetario - Plaquetócrito, e aumento de Amplitude Plaquetária); Leucemia Mieloide Crónica (aumento da Amplitude Plaquetária) e Linfoma náo Hodgkin ou Síndrome Mielodisplásica (diminuigáo do Plaquetócrito), os índices plaquetários poderiam ser usados como ferramenta diagnóstica orientadora. Entretanto, náo poderiam contribuir no momento de diferenciar entre leucemias agudas, visto que náo apresentam diferengas significativas. Diante de um diagnóstico presuntivo de síndrome mielodiasplásica ou leucemia mieloide aguda, o valor de Volume Plaquetário Médio (VPM) poderia contribuir como ferramenta orientadora para o diagnóstico, devido a que seria mais baixo na leucemia aguda. A análise dos resultados sugere que na prática clínica os índices plaquetários poderiam contribuir de um modo significativo para a confirmagáo do diagnóstico.

Progress in iron chelators: the report of the 53rd annual meeting of American Society of Hematology / 白血病·淋巴瘤

Chelation therapy could remove transfusional iron burden. Three chelators are currently available, including deferoxamine, deferiprone, and deferasirox, which can be used as monotherapy or in combination.Several factors must be considered in the design of optimal and individualized chelation regimens,which include chelator availability and its properties,degree of organ-specific iron loading,ongoing transfusional iron burden, and patient preference. Comparative effectiveness trials may help to determine the ideal strategy. This article reviews latest research presented at the 53rd annual meeting of the American Society of Hematology (ASH) on the use of iron chelators.

CD4+CD25highFoxP3+ Regulatory T-cells in Hematologic Diseases / 대한진단검사의학회지

BACKGROUND: CD4+CD25+ regulatory T-cells (Tregs) play a critical role in immune responses. We explored the status of Tregs in neoplastic and autoimmune hematologic diseases. We also evaluated the technical aspects of Treg measurement in terms of sample type and detection markers. METHODS: A total of 68 subjects were enrolled: 11 with AML, 8 with MDS, 10 with autoimmune diseases, and 39 controls. Tregs were analyzed in peripheral blood (PB) and bone marrow (BM) samples from each subject. Flow cytometry and the Human Regulatory T cell Staining Kit (eBioscience, USA) for CD4, CD25, and FoxP3 (forkhead box P3) were used. RESULTS: The CD4+CD25high/CD4 and CD4+CD25highFoxP3+/CD4 populations were significantly correlated (P<0.0001). The AML and high-risk MDS groups had significantly larger CD4+CD25high/CD4 and CD4+CD25highFoxP3+/CD4 populations in PB than the autoimmune (P=0.007 and 0.012, respectively) and control groups (P=0.004 and 0.006, respectively). Comparable findings were observed in BM. The CD4+CD25highFoxP3+/CD4 population was significantly larger in PB than in BM (P=0.0003). CONCLUSIONS: This study provides comparison data for Tregs in AML, MDS, and autoimmune hematologic diseases, and would be helpful for understanding the different immunologic bases of various hematologic diseases. Treg measurement using CD4, CD25, and/or FoxP3 in PB rather than in BM seems to be practical for routine hematologic purposes. Large-scale analysis of the diagnostic role of Treg measurement is needed.

Urolithiasis in Patients Suffering from Malignant Hematologic Diseases

PURPOSE: We performed this study in order to evaluate the incidence and characteristics of urolithiasis in patients with malignant hematologic diseases. MATERIALS AND METHODS: Nine hundred one patients who underwent medical treatment for malignant hematologic disease and 40,543 patients who visited the emergency room and without malignant hematologic diseases were included in our study. The patients with malignant hematologic diseases were divided into two groups depending on their primary treatment. Group I included patients with acute and chronic leukemia (AML, ALL, CML, CLL) for which chemotherapy and steroid therapy was necessary, and group II included patients with anaplastic anemia and myelodysplastic syndrome and who had undergone repeated transfusion for treatment. Comparisons were made between the two groups in respect to the incidence of urolithiasis and the stones' radiopacity. RESULTS: Twenty nine patients (3.2%) of the 901 malignant hematologic patients were diagnosed with urolithiasis, compared to 575 patients (1.4%) of 40,543 emergency room patients. There was a significant increase of the incidence of urolithiasis in the malignant hematologic group. Compared to the general patients, the patients with malignant hematologic diseases had a higher rate of radiolucent stones (46.6% versus 16.3%, respectively), and the difference was significant. CONCLUSION: The incidence of urolithiasis for malignant hematologic patients was significantly higher than that for the control group.

Risk Factors for Osteonecrosis of the Femoral Head in Patients with Heamatologic diseases

PURPOSE: We aimed to clarify the risk factors associated with the development of ONFH by comparing patients with hematologic diseases and osteonecrosis of the femur head (ONFH) to those patients without ONFH and who have hematologic diseases MATERIALS AND METHODS: The study population was limited to the patients admitted to our Hematology-Oncology department from 1 January 1994 to 31 May 2007. The patients were divided into 2 groups (those with ONFH, 54 patients and those without ONFH, 54 patients) and the risk factors for ONFH were evaluated by a comparative analysis. We analyzed the effect of a history of bone marrow transplantation (BMT), graft-versus-host disease (GVHD), total body radiation (TBI) and the amount of steroid used as the risk factors for ONFH. RESULTS: On the multiple logistic regression analysis, a total steroid use of >g/BMI was statistically identified as a significant risk factor for ONFH. The history of BMT and TBI were not statistically correlated with the development of ONFH. Among the patients with BMT, allogenic BMT and a history of GVHD were not statistically correlated with the development of ONFH on the multiple logistic regression analysis. CONCLUSION: Patients with hematologic diseases and who have used steroid >1.5g/BMI should carefully observed because they are more likely to develop ONFH.

Mesenchymal stem cells for treatment of steroid-resistant chronic graft-versus-host disease / 中华内科杂志

Objective To assess whether treatment with mesenchymal stem cells (MSCs) is an effective adjunct therapy for refractory extensive chronic graft-versus-host disease (GVHD) resistant to conventional therapy. Methods 12 patients with steroid-resistant extensive chronic GVHD were treated with MSCs. One patient received one dose, 10 received two doses, and the remaining three doses. The MSCs were obtained from HI,A-identical sibling donors (n = 14), haploidentical donors (n = 2), unrelated mismatched donor (n = 1) and third-party HLA-mismatched donors (n = 7). Of the 11 patients treated with multiple infusions, 5 received cells derived from two donors. The median first dose of MSCs was 1.0 (0. 4-2. 1) × 106/kg , the median second dose was 1.2(0. 8-1.9) × 106/kg , and the third dose in one patient was 1.1 × 106/kg. Meanwhile the proportion of CD3+ ,CD4+,CD8+ ,CD19+,CD4+ CD25+ ,FOXP3+,FOXP3+CD4+ and FOXP3+ CD25+ was determined with double fluorescent-labeled antibodies and flow cytometry before and 4 weeks after the MSCs infusion. Results No patients had side-effects during or immediately after the infusions of MSCs. After a treatment course of one to three doses, 3 patients had complete response(CR), 6 showed partial response(PR) and 3 did not respond; the total effective rate was 75% (9/12). Complete resolution was seen in the involvement of skin (3/12), lung (1/3), joints (1/5), liver (3/10), oralcavity (4/12) and eye (2/7). Response rate was not related to donor HLA-match. 3 CR patients discontinued all of the immunosuppressive agents without relapse 100 to 292 days after the MSC infusion and 6 PR patients taped all immunosuppressive agents after 60 to 79 days. Mean follow-up period was 1152(795-1914) days, leukemia free survival rate was 91.7% (11/12) and the overall survival rate was 75% (9/12). The ratio of CD4/CD8 and the proportion of regulatory T cells were significantly higher than that before MSCs treatment. Conclusion Third-party MSCs were as effective as HLA-identical or haploidentical cells. This finding has practical implications and suggests that third-party cells can be prepared and stored frozen to be used for steroid-resistant extensive chronic GVHD therapy. It is concluded that MSCs may prevent the lethal cGVHD after allogeneic hematopoietic stem cell transplantation and raise the survival rate by increasing the ratio of CD4/CD8 and proportion of regulatory T cells in vivo.

Advance of growth factor-independent 1B(GFI1B) in common hematologic diseases / 白血病·淋巴瘤

Recent studies have demonstrated that GFI1B is most prominently expressed in cells of the fetal and adult erythroid and megakaryocytic lineages. In 2006,it is first reported that GFI1B is highly expressed in acute myeloid leukemia, especially in erythroleukaemia and megakaryocytic leukaemia.This report provides theoretical basis for diagnosis and differential diagnosis of subtypes of acute myeloid leukaemia.In this review,we covered the expression of GFI1B in leukemia,T-cell lymphoma,myelodysplastic syndrome and so on.

Pulmonary Resection for Invasive Pulmonary Aspergillosis in Hematological Malignancy Patients / 대한흉부외과학회지

BACKGROUND: Invasive pulmonary aspergillosis, a frequent fungal infection in immunocompromised patients, is known to have a poor prognosis despite the use of antifungal therapy in leukemic patients. We studied the outcome of surgical resection of invasive pulmonary aspergillosis where bleeding tendency, localized recurrence of infection, and incidence could be reduced. MATERIAL AND METHOD: We retrospectively reviewed 14 patients with a hematological malignancy where invasive pulmonary aspergillosis was diagnosed during the 10 years between 1998 and 2007. From the medical records, we reviewed the type and treatment of the hematological malignancy, including the diagnostic methods of invasive pulmonary aspergillosis, the preoperative hematological conditions and their management, and the surgical methods and records. We also analyzed the development of postoperative complications and patient mortality, the recurrence of postoperative invasive pulmonary aspergillosis, and if the patients had a bone marrow transplant. RESULT: Fourteen patients with invasive pulmonary aspergillosis and a hematological malignancy underwent a pulmonary lobectomy. One patient had a complication of bronchopleural fistula, but there were no other serious complications such as bleeding or wound infection, and none of the patients died postoperatively. CONCLUSION: We have shown that pulmonary lobectomy is a safe and effective therapy for invasive pulmonary aspergillosis in patients with hematological malignancies that allow further treatment of the hematological malignancy.

Stomach Cancer Secondary to Hematologic Diseases

PURPOSE: Patients with hematologic diseases such as chronic myeloid leukemia (CML) or chronic lymphoid leukemia (CLL) are known to have an increased chance of acquiring a secondary neoplasm. Stomach cancer is one of the most common malignant diseases in Korea, and we investigated whether the incidence of secondary stomach cancer in patients with a hematologic disease increases, in order to determine if a more intensive screening program for detecting secondary gastric cancer was required. We also investigated the safety of performing a gastrectomy in hematologic disease patients. MATERIALS AND METHODS: From 1992 to 2006, the medical records of 8376 patients diagnosed with one of the six common hematologic diseases were reviewed. RESULTS: Nine secondary stomach cancers were found among the 8376 patients during the 15-year observation period. No surgical-related complications occurred, and there was no recurrence of stomach cancer if detected early. CONCLUSION: It seems that a more intensive screening program for detecting secondary gastric cancer in hematologic disease patients is not required, and surgery is not risky in these patients.

Two Pediatric Patients with Neutropenia and Thrombocytopenia Associated with Parvovirus B19 Infection / 소아과

Parvovirus B19, a member of the Erythrovirus genus, is the only member of the Parvoviridae family known to be pathogenic in humans. Haematological consequences of B19 infection arise due to a direct cytotoxic effect on erythroid progenitors in bone marrow, resulting in interruption of erythrocyte production. The physiology of host haematopoiesis and competence of the immune response each determines clinical manifestations of B19 infection. A search of the literature revealed that neutropenia and thrombocytopenia due to B19 have been rarely reported in healthy individuals. In Korea, B19 infection has been reported among individuals with underlying disorders such as leukemia or congenital spherocytosis. Among otherwise healthy persons, there have been few reported cases of B19 infection which resulted in anemia, leukopenia, and thrombocytopenia. We herein report, for the first time in Korea, of two children who experienced leukopenia and thrombocytopenia, but not anemia, after B19 infection and recovered uneventfully.