Iron deficiency and child psychomotor development in a rural region of Chota, Peru, 2022 / Deficiencia de hierro y desarrollo psicomotor infantil en una zona rural de Chota, Perú 2022 / Deficiência de ferro e desenvolvimento psicomotor infantil em uma área rural de Chota, Peru 2022

Introduction: Iron deficiency and psychomotor developmental delay are two public health problems that cause high childhood morbidity and mortality worldwide, which can be related to social, economic, cultural and health factors that affect the environment where children and their family live. Objective: To determine the relationship between iron deficiency anemia and psychomotor development in children aged 2 to 4 years treated at the Cuyumalca Clinic, Chota. Materials and methods: Relational, cross-sectional study conducted on 48 children, who underwent hemoglobin testing through a portable hemoglobinometer and were subjected to the Psychomotor Development Test. Results: 31.2% of the children displayed some type of anemia, with the most common being moderate anemia (17.7%). On average, 10.9% showed some type of psychomotor developmental delay, including coordination (6.3%), language (8.4%), motor skills (16.7%), and overall development (12.5%). 4.2% of the children who had minor to moderate anemia showed developmental delay risks in the three assessed areas as well as in their overall development. Conclusion: There is no statistically significant relationship between iron deficiency anemia and several domains of psychomotor development, including coordination, language, motor skills as well as overall development.

Introducción: La deficiencia de hierro y las alteraciones en el desarrollo psicomotor son dos problemas de salud pública que causan una alta morbimortalidad infantil alrededor del mundo. Los estudios apuntan a que esto se relaciona con los factores sociales, económicos, culturales y sanitarios en los que el niño y su familia vive. Objetivo: Determinar la relación entre anemia ferropénica y desarrollo psicomotor en niños de 2 a 4 años atendidos en el Puesto de Salud de Cuyumalca, Chota. Materiales y métodos: Estudio relacional, transversal, desarrollado con 48 niños a quienes se les realizó un dosaje de hemoglobina con hemoglobinómetro portátil y se les aplicó el Test de Desarrollo Psicomotor. Resultados: El 31,2% de niños presentaron algún tipo de anemia, siendo la anemia moderada la más frecuente (16,7%); en promedio 10,9% evidenciaron alguna alteración en el desarrollo psicomotor en coordinación (6,3%), lenguaje (8,4%), motricidad (16,7%) y desarrollo global (12,5%). El 4,2% de niños con riesgo para el desarrollo presentaron anemia leve o moderada en las tres áreas evaluadas, al igual que en el desarrollo global. Conclusión: No existe relación estadística significativa entre anemia ferropénica y desarrollo psicomotor para las áreas de coordinación, lenguaje y motricidad; además del desarrollo global.

Introdução: A deficiência de ferro e as alterações no desenvolvimento psicomotor são dois problemas de saúde pública que causam elevada morbidade e mortalidade infantil em todo o mundo. Estudos sugerem que isso está relacionado aos fatores sociais, econômicos, culturais e de saúde em que vivem a criança e sua família. Objetivo: Determinar a relação entre anemia ferropriva e desenvolvimento psicomotor em crianças de 2 a 4 anos atendidas no Posto de Saúde Cuyumalca, Chota. Materiais e métodos: Estudo relacional, transversal, desenvolvido com 48 crianças que realizaram dosagem de hemoglobina com hemoglobinômetro portátil e foi aplicado o Teste de Desenvolvimento Psicomotor. Resultados: 31,2% das crianças apresentaram algum tipo de anemia, sendo a anemia moderada a mais frequente (16,7%); em média, 10,9% apresentaram alguma alteração no desenvolvimento psicomotor na coordenação (6,3%), linguagem (8,4%), motricidade (16,7%) e desenvolvimento global (12,5%). 4,2% das crianças em risco de desenvolvimento apresentaram anemia leve ou moderada nas três áreas avaliadas, bem como no desenvolvimento global. Conclusão: Não há relação estatística significativa entre anemia ferropriva e desenvolvimento psicomotor para as áreas de coordenação, linguagem e motricidade; bem como o desenvolvimento global.

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.

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.

Prise en charge des hémopathies malignes au centre national d'oncologie médicale et de radiothérapie Alassane Ouattara d'Abidjan / Managem ent of hem atologic neoplasm sat the Centre National d'Oncologie Médicale et de Radiothérapie Alassane Ouattara ofAbidjan

Introduction. Les hémopathies malignes sont des proliférations anormales et anarchiques de cellules hématopoïétiques à point de départ médullaire ou périphérique. Notre étude qui avait pour objectif de faire le bilan de la prise en charge des hémopathies malignes au Centre national d'oncologie médical et de radiothérapie Alassane Ouattara. Méthodes. Il s'agissait d'une étude rétrospective descriptive d'une durée de 2 ans 3 mois allant du 1er janvier 2018 au 31 Mars 2020 portant sur 80 dossiers de malades porteurs d'hémopathies malignes et prise en charge dans le centre. Résultats. Notre étude a permis de recenser 2,2% d'hémopathies malignes sur les 3650 cas de pathologies cancéreuses recensées au CNRAO, soit une incidence 26,66 cas/an avec un sex-ratio 1,2. Les syndromes lymphoprolifératifs (SLP) sont les plus fréquents (96,25 %), sous trois principales formes: lymphomes malins non hodgkiniens (LMNH) non Burkitt (51,25%), leucémie myéloïde chronique (20%) et le myélome multiple (16,25%). Les LMNH représentent 51,25% des HM recensées avec 41,46 % de lymphomes de haut grade de malignité. La chimiothérapie était de mise chez tous nos patients. Ainsi sur 41 cas de LMNH, seulement 12 (29,27%) ont bénéficié du protocole R-CHOP. On notait 46,34% de réponse complète. Dans notre étude, le LH représentait 8,75% il était traité à 71,43% avec le protocole ABVD avec une réponse complète chez 6 patients. 37,5% des patients porteurs de leucémie myéloïde chronique ont reçu le Rituximab; ils ont été traités par les protocoles COP (31,25%), CHOP (31,25%), RCVP (12,5%) et R-CHOP (25%). La réponse thérapeutique était complète à 68,75%. Le protocole utilisé dans le traitement du myélome multiple a été le VMCD-REV à 76,92% avec pour réponse thérapeutique complète chez 6 patients, 3 réponses partielles et 4 en cours de traitement. Conclusion. Les SLP qui sont les plus fréquents des HM avec trois principales formes: LMNH non Burkitt, leucémie myéloïde chronique et myélome multiple. Nous avons cependant des difficultés quant à la mise en route de la chimiothérapie.

Introduction. Hematologic neoplasms are abnormal and anarchic proliferations of hematopoietic cells with a medullary or peripheral starting point. Our study aimed to report the management of hematological malignancies at the Centre National d'Oncologie Médicale et de Radiothérapie Alassane Ouattara (CNRAO). Methods. This was a descriptive retrospective study lasting 2 years 3 months from January 1st, 2018 to March 31st, 2020 concerning 80 patients with hematologic neoplasms who were managed in the CNRAO. Results. Hematologic neoplasms represented 2.2% of cancers (80/3650) at CNRAO, giving an annual incidence of 26.66 cases. The sex ratio was 1.2. Lymphoproliferative syndromes were the most common subgroups (96.25%). These were mainly non-Burkitt non Hodgkin lymphoma (51.25%), high grade lymphomas (41.46%), chronic lymphocytic lymphoma (20%) and multiple myeloma (16.25%). Chemotherapy was administered to all patients. Among 41 cases of non-Hodgkin lymphoma, 12 (29.27%) benefited from the R-CHOP protocol and full response was observed in 46.34% of them. We found 7 patients with Hodgkin lymphoma (8.75%) and the ABVD protocol was used for 6 cases (71.43%). Six out of these seven patients were in complete response. Among the 16 patients with chronic lymphocytic leukemia, 6 (37.5%) received Rituximab. The distribution of the patients with chronic lymphocytic leukemia was as follows: COP 31.25%, CHOP 31.25%, RCVP 12.5% and R-CHOP 25% and 68.75% had full response. The most common treatment protocol for multiple myeloma was VMCD-REV (76.92%). Six patients had complete response, 3 had partial response and 4 were in the course of treatment. Conclusion. In our practice, hematologic neoplasms are mainly lymphoproliferative syndromes and the most common varieties are non-Burkitt non Hodgkin lymphoma, high grade lymphomas, chronic lymphocytic lymphoma and multiple myeloma. We have difficulties in getting chemotherapy started.

Haematological indices and coagulation profile as predictors of disease severity and associations with clinical outcome among COVID­19 patients in Lagos, Nigeria

Background: This study aims to evaluate the use of haematological indices and coagulation profiles as possible low cost predictors of disease severity and their associations with clinical outcomes in COVID 19 hospitalized patients in Nigeria. Materials and Methods: We carried out a hospital based descriptive 3 month observational longitudinal study of 58 COVID 19 positive adult patients admitted at the Lagos University Teaching Hospital, Lagos, Nigeria. We used a structured questionnaire to obtain the participants' relevant sociodemographic and clinical data, including disease severity. Basic haematologic indices, their derivatives, and coagulation profile were obtained from patients' blood samples. Receiver Operating Characteristic (ROC) analysis was used to compare these laboratory based values with disease severity. A P < 0.05 was considered statistically significant. Results: The mean age of the patients was 54.4 ± 14.8 years. More than half of the participants were males (55.2%, n = 32) and most had at least one comorbidity (79.3%, n = 46). Significantly higher absolute neutrophil count (ANC), neutrophil­lymphocyte ratio (NLR), systemic immune inflammation index (SII), lower absolute lymphocyte count (ALC) and lymphocyte­monocyte ratio (LMR) were associated with severe disease (P< 0.05). Patients' hemoglobin concentration (P= 0.04), packed cell volume (P< 0.001), and mean cell hemoglobin concentration (P= 0.03) were also significantly associated with outcome. Receiver operating characteristic (ROC) analysis of disease severity was significant for the ANC, ALC, NLR, LMR, and SII. The coagulation profile did not show any significant associations with disease severity and outcomes in this study. Conclusion: Our findings identified haematological indices as possible low cost predictors of disease severity in COVID 19 in Nigeria

Incidence and clinical characteristics of engraftment syndrome after syngeneic hematopoietic stem cell transplantation in patients with hematological diseases / 中华血液学杂志

Objective: To explore the incidence and clinical characteristics of engraftment syndrome (ES) after syngeneic hematopoietic stem cell transplantation (syn-HSCT) in patients with hematological diseases. Methods: The clinical data of 21 patients who received syn-HSCT at People's Hospital of Peking University from January 1994 to May 2018 were retrospectively analyzed. Results: Seven (33.3% ) of 21 patients developed ES. The onset of ES symptoms occurred at a median of 8 (range: 5-13) days after HSCT, and the diagnosis of ES occurred at a median of 10 (range: 7-14) days after HSCT. Steroids were administered immediately after the diagnosis of ES, the median time of symptom continuance was 2 (range: 1-5) days, and all patients showed complete resolution of ES symptoms. In the multivariate analysis, patients with acute myeloid leukemia and faster neutrophil reconstitution were the risk factors for ES (HR=15.298, 95% CI 1.486-157.501, P=0.022, and HR=17.459, 95% CI 1.776-171.687, P=0.014) . Meanwhile, there was no significant difference in the overall survival and disease-free survival between patients with ES and those without ES. Conclusion: A high incidence of ES was observed in syn-HSCT recipients. Moreover, the prognosis of ES was excellent.

Retrospective Analysis of Irregular Antibodies Causing Hemolytic Disease of the Fetus and Newborn in Jiangxi Province / 中国实验血液学杂志

OBJECTIVE@#To analyze the characteristics of antibody-specific distribution, laboratory detection results of hemolytic disease of the fetus and neonatal(HDFN) caused by irregular blood group antibodies other than ABO, and its correlation with the clinical situation.@*METHODS@#The non-ABO-HDFN cases in our hospital from October 2012 to December 2021 were selected as the research objects, and the cases diagnosed with ABO-HDFN in the same period were randomly selected as the control group, and the data of antibody specific distribution, total bilirubin, direct antibodies, maternal history, age of the children, the presence or absence of combined ABO-HDFN, and whether to exchange/transfuse blood were retrospectively analyzed. The characteristics of non-ABO-HDFN in Jiangxi province were analyzed.@*RESULTS@#The detection rate of non-ABO-HDFN in Jiangxi province increased. Among 187 non ABO-HDFN cases, the highest percentage of Rh-HDFN was detected (94.6%). Compared with the control group of ABO-HDFN, the non-ABO-HDFN had higher mean integral value of direct antibody, higher peak total bilirubin, and longer duration. Anti-M-HDFN may have severe disease but the direct antibody weak positive/negative, it was easy missed in clinical and delayed the treatment. There is no correlation between the specificity of irregular antibodies, the sex of the child, the mother's previous childbirth history, the presence or absence of combined ABO-HDFN and the need for blood exchange/transfusion(P>0.05).@*CONCLUSION@#The irregular antibodies of causing non ABO-HDFN in Jiangxi area are mainly Rh blood group system, followed by MNS blood group system. Understanding the characteristics of HDFN disease, serological features and the correlation with clinical indexes will help to detect and treat non ABO-HDFN in time and reduce the risk of complications.

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.

Effect of Hemoglobin on Efficacy of CAR-T Therapy in Patients with Multiple Myeloma / 中国实验血液学杂志

OBJECTIVE@#To investigate the effect of hemoglobin (Hb) on the efficacy of chimeric antigen receptor T cell therapy (CAR-T) in patients with multiple myeloma (MM).@*METHODS@#From June 2017 to December 2020, 76 MM patients who received CAR-T therapy in the Department of Hematology, The Affiliated Hospital of Xuzhou Medical University, with complete clinical data and evaluable efficacy, were selected as the research objects. According to the receiver operating characteristic (ROC) curve, the best cut-off value was obtained. The patients were divided into groups on the basis of Hb 105.5 g/L as the cut-off value. The age, sex, serum calcium, β2-microglobulin, serum creatinine, lactate dehydrogenase (LDH), and the influencing factors of CAR-T treatment efficacy in MM patients were analyzed.@*RESULTS@#Hb was an influencing factor of efficacy. Univariate analysis showed that Hb, LDH, and albumin affected the efficacy of CAR-T therapy. Multivariate analysis showed that Hb ( OR=1.039, 95% CI: 1.002-1.078) and LDH ( OR=1.014, 95% CI: 1.000-1.027) were the influencing factors for the efficacy of CAR-T therapy.@*CONCLUSION@#The efficacy of CAR-T therapy in MM patients with low Hb is poor, and Hb is a factor affecting the efficacy of CAR-T therapy.

Factors Influencing and Adverse Reactions of Voriconazole Clearance in Patients with Hematological Diseases / 中国实验血液学杂志

OBJECTIVE@#To monitor the changes of voriconazole minimum concentration(Cmin) in patients with hematological diseases, and evaluate the factors influencing and adverse reactions of voriconazole clearance in patients with hematological diseases, so as to provide a theoretical basis for reasonable clinical use of voriconazole.@*METHODS@#136 patients with hematological diseases who used voriconazole in Wuhan NO.1 Hospital from May 2018 to December 2019 were selected. The correlation between C-reactive protein, albumin, creatinine and voriconazole Cmin were analyzed, and the changes of voriconazole Cmin after glucocorticoid treatment was also detected. In addition, stratified analysis was used to explore the adverse events of voriconazole.@*RESULTS@#Among 136 patients, 77 were male (56.62%) and 59 were female (43.38%). There were positive correlations between voriconazole Cmin and C-reactive protein and creatinine levels (r=0.277, r=0.208), while voriconazole Cmin was negatively correlated with albumin level (r=-2.673). Voriconazole Cmin in patients treated with glucocorticoid was decreased significantly (P<0.05). In addition, sratified analysis of voriconazole Cmin showed that compared with voriconazole Cmin 1.0-5.0 mg/L group, the incidence of adverse reactions of visual impairment in voriconazole Cmin> 5.0 mg/L group was increased (χ2=4.318, P=0.038).@*CONCLUSION@#The levels of C-reactive protein, albumin and creatinine are closely related to the voriconazole Cmin, which indicate that inflammation and hyponutrition may prevent the clearance of voriconazole in patients with hematological diseases. It is necessary to monitor the voriconazole Cmin of patients with hematological diseases, and adjust the dosage in time to reduce adverse reactions.

Expansion of cord blood stem cells in fibronectin-coated microfluidic bioreactor

ABSTRACT Background: Hematopoietic stem/progenitor cell transplantation is the main treatment option for hematological malignancies and disorders. One strategy to solve the problem of low stem cell doses used in transplantation is pre-transplant expansion. We hypothesized that using fibronectin-coated microfluidic channels would expand HSPCs and keep self-renewal potential in a three-dimensional environment, compared to the conventional method. We also compared stem cell homing factors expression in microfluidic to conventional cultures. Materials and methods: A microfluidic device was created and characterized by scanning electron microscopy. The CD133+ cells were collected from cord blood and purified. They were subsequently cultured in 24-well plates and microfluidic bioreactor systems using the StemSpan serum-free medium. Eventually, we analyzed cell surface expression levels of the CXCR4 molecule and CXCR4 mRNA expression in CD133+ cells cultured in different systems. Results: The expansion results showed significant improvement in CD133+ cell expansion in the microfluidic system than the conventional method. The median expression of the CXCR4 in the expanded cell was lower in the conventional system than in the microfluidic system. The CXCR4 gene expression up-regulated in the microfluidic system. Conclusion: Utilizing microfluidic systems to expand desired cells effectively is the next step in cell culture. Comparative gene expression profiling provides a glimpse of the effects of culture microenvironments on the genetic program of HSCs grown in different systems.

HIV-positive patients presenting with peripheral blood cytopenias: is bone marrow assessment a priority?

ABSTRACT Introduction: Hematologic abnormalities are frequent among persons living with HIV (PLWH). The bone marrow aspirate (BMA) and biopsy (BMB) are commonly performed in the diagnostic approach of patients with unexplained cytopenias. Changes in antiretrovirals, supportive therapy and increased life expectancy have modified the distribution and etiology of cytopenias, questioning their use. Our aim was to analyze the diagnostic yield of BMA, BMB and marrow cultures for the evaluation of cytopenias in PLWH. Methods: This was a retrospective cohort of ≥ 18-year-old PLWH undergoing bone marrow assessment (MA) for the evaluation of cytopenias between January 2002 and December 2015. Results: A total of 236 cytopenic events were analyzed, 47.9% being PLWH who had a longstanding diagnosis (≥ 1 year). Adherence to antiretrovirals was 63.5%. Anemia was seen in 91.9% and pancytopenia in 39%. Common presentations included fever (52.1%), weight loss (42.8%) and adenopathies (28.8%). Median days from detection to MA was 5 (0 - 63 days). Most common etiologies were non-HIV infectious diseases (31.4%) and benign/malignant hematologic diseases (26.3%). The diagnostic yield was 16.1% for BMA, 20.3% for BMB, 30.5% for both and 35.6% when cultures were added. Patients most likely to have conclusive MA were those with moderate/severe thrombocytopenia (p = 0.007). Fever, splenomegaly, and low CD4+ counts were associated with infectious etiologies, while hematologic diagnoses were related to the presence of adenopathies. Conclusion: As a minimally invasive intervention, the MA has a high yield for identifying the etiology of cytopenic events in PLWH, being conclusive in one in three patients. Early performance could lead to prompt diagnosis and timely therapy initiation.

Diferentes formas de presentación de la anemia megaloblástica en el lactante / Different forms of presentation of megaloblastic anemia in the infant

Introducción: La anemia megalobástica es un trastorno madurativo de los precursores eritroides y mieloides causado por déficit de vitamina B12, ácido fólico, o ambos. Es poco común en la infancia y su prevalencia se desconoce por ser una enfermedad poco frecuente. Objetivo: Describir diferentes formas de presentación de la anemia megaloblástica en el lactante. Presentación de casos: Se presentan dos casos de lactantes, en el caso 1 la madre tuvo una alimentación precaria durante el embarazo y la lactancia, prolongó la lactancia materna exclusiva más de 6 meses. La paciente comenzó a perder las habilidades ganadas en el desarrollo psicomotor y presentó trastornos neurológicos graves, por lo que se consideró que se trataba de una enfermedad progresiva del sistema nervioso central. En el caso 2, en el que se prolongó la lactancia materna exclusiva, apareció trombocitopenia, por lo que se sospechó una enfermedad hematológica maligna. Resultados: En ambos casos después de realizar diversas pruebas para descartar enfermedades neurológicas (caso 1) y enfermedades hematológicas (caso 2) se diagnosticó anemia megaloblástica por déficit de vitamina B12 por disminución en la ingesta y una reserva limítrofe en la madre que lacta. En ambos casos los síntomas desaparecieron con el tratamiento vitamínico sustitutivo. Conclusiones: En el lactante la anemia megaloblástica se puede presentar de diferentes formas clínicas a pesar de tener la misma causa, un déficit en la ingesta y una reserva escasa de la madre durante el embarazo y lactancia(AU)

Introduction: Megaloblastic anemia is a maturing disorder of the erythroid and myeloid precursors caused by deficiency of vitamin B12, folic acid, or both. It is uncommon in childhood and its prevalence is unknown because it is a rare disease. Objective: To describe different forms of presentation of megaloblastic anemia in infants. Presentation of cases: Two cases of infants are presented, in case 1 the mother had a precarious diet during pregnancy and lactation, and prolonged exclusive breastfeeding more than 6 months. The patient began to lose the skills gained in psychomotor development and presented severe neurological disorders, so it was considered that it was a progressive disease of the central nervous system. In case 2, in which exclusive breastfeeding was prolonged, thrombocytopenia appeared, so a malignant hematological disease was suspected. Results: In both cases, after performing various tests to rule out neurological diseases (case 1) and hematological diseases (case 2), megaloblastic anemia was diagnosed due to vitamin B12 deficiency due to a decrease in intake and a borderline reserve in the breastfeeding mother. In both cases the symptoms disappeared with vitamin replacement therapy. Conclusions: In the infant, megaloblastic anemia can occur in different clinical ways despite having the same cause, a deficit in intake and a low reserve of the mother during pregnancy and lactation(AU)

Alterações na qualidade de vida de adultos com câncer hematológico em cinco anos após o transplante de células-tronco hematopoéticas / Changes in the quality of life of adults with hematological cancer in five years after hematopoietic stem cell transplantation

Resumo: O objetivo desta tese foi avaliar as alterações da qualidade de vida relacionada à saúde dos pacientes adultos com câncer hematológico, submetidos ao transplante de células-tronco hematopoéticas, nos primeiros cinco anos após o procedimento. Trata-se de um estudo quantitativo, longitudinal, observacional e analítico, realizado em hospital público do sul do Brasil, referência na América Latina para esta modalidade de tratamento. Foram incluídos 55 participantes com idade igual ou superior a 18 anos, que se submeteram a esta terapia. A coleta de dados ocorreu de setembro de 2013 a janeiro de 2021, com avaliações em dez etapas: pré-transplante (antes de iniciar o condicionamento), pancitopenia, pré-alta hospitalar, após 100 dias, após 180 dias, Follow up 1 (após 360 dias), e anualmente até completar cinco anos da realização do procedimento. Foram aplicados um instrumento para coleta de dados sociodemográficos e clínicos e os questionários de Qualidade de vida relacionada à saúde Quality of life Questionnaire Core 30 e Functional Assessment of Cancer Therapy - Bone Marrow Transplant, ambos traduzidos, adaptados e validados para o português - Brasil. Em relação ao diagnóstico, as leucemias estão presentes em 65% dos casos; quanto à modalidade de tratamento, o transplante de células-tronco alogênico foi realizado em 71% dos pacientes. No que diz respeito aos óbitos, a causa de maior incidência foi por recidiva da doença (44%), e o maior número ocorreu no primeiro ano (37%). A qualidade de vida global (56,6/100) e geral (90,7/148) apresentou os menores escores na etapa de pancitopenia, com melhores índices no quinto ano, (80,4/100) e (116,1/148), respectivamente. A análise com o modelo linear generalizado misto evidenciou alterações significativas nos escores dos domínios de qualidade de vida relacionada à saúde entre as etapas ao longo do tempo. Foi comprovada a hipótese de que os pacientes com melhores escores nos domínios de qualidade de vida relacionada à saúde observados no início do tratamento têm maior sobrevida. Os resultados do estudo inferem as correlações entre os domínios mensurados e encontram, assim, sustentação no modelo conceitual teórico utilizado. As contribuições consistem em reafirmar a dimensionalidade do constructo qualidade de vida relacionada à saúde, além de agregar conhecimento acerca das alterações autopercebidas pelos pacientes durante o tratamento.

Abstract: The objective of this thesis was to evaluate the changes in the health-related quality of life of adult patients with hematological cancer undergoing hematopoietic stem cell transplantation in the first five years after the procedure. This is a quantitative, longitudinal, observational and analytical study carried out in a public hospital in southern Brazil, a reference in Latin America for this treatment modality. We included 55 participants aged 18 years and over, who underwent this therapy. Data collection took place from September 2013 to January 2021, with evaluations in ten stages: pre-transplantation (before starting conditioning), pancytopenia, pre-hospital discharge, after 100 days, after 180 days, Follow up 1 ( after 360 days), and annually until completing five years of the procedure. An instrument for collecting sociodemographic and clinical data and the Health-related Quality of life Questionnaire Core 30 and Functional Assessment of Cancer Therapy - Bone Marrow Transplant questionnaires were applied, both translated, adapted and validated for Portuguese - Brazil. Regarding diagnosis, leukemias are present in 65% of cases; regarding the treatment modality, allogeneic stem cell transplantation was performed in 71% of the patients. With regard to deaths, the cause of highest incidence was disease recurrence (44%), and the highest number occurred in the first year (37%). The global (56.6/100) and general (90.7/148) quality of life had the lowest scores in the pancytopenia stage, with better rates in the fifth year (80.4/100) and (116.1/148), respectively. The analysis with the mixed generalized linear model showed significant changes in the scores of the health-related quality of life domains between the stages over time. The hypothesis was confirmed that patients with better scores in the domains of health-related quality of life observed at the beginning of treatment have greater survival. The study results infer the correlations between the measured domains and thus find support in the theoretical conceptual model used. The contributions consist of reaffirming the dimensionality of the health-related quality of life construct, in addition to adding knowledge about the self-perceived changes by patients during treatment.

Resumen: El objetivo de esta tesis fue evaluar los cambios en la calidad de vida relacionada con la salud de pacientes adultos con cáncer hematológico, sometidos a trasplante de células madre hematopoyéticas, en los primeros cinco años después del procedimiento. Se trata de un estudio cuantitativo, longitudinal, observacional y analítico realizado en un hospital público del sur de Brasil, referencia en América Latina para esta modalidad de tratamiento. Se incluyeron 55 participantes mayores de 18 años que se sometieron a esta terapia. La recolección de datos ocurrió de septiembre de 2013 a enero de 2021, con evaluaciones en diez etapas: pretrasplante (antes de iniciar el acondicionamiento), pancitopenia, alta prehospitalaria, después de 100 días, después de 180 días, Seguimiento 1 (después de 360 días), y anualmente hasta completar cinco años del procedimiento. Se aplicó un instrumento de recolección de datos sociodemográficos y clínicos y los cuestionarios Health-related Quality of life Questionnaire Core 30 y Functional Assessment of Cancer Therapy - Bone Marrow Transplant, ambos traducidos, adaptados y validados para portugués - Brasil. En cuanto al diagnóstico, las leucemias están presentes en el 65% de los casos; en cuanto a la modalidad de tratamiento, se realizó trasplante alogénico de células madre en el 71% de los pacientes. En cuanto a las defunciones, la causa de mayor incidencia fue la recidiva de la enfermedad (44%) y el mayor número se produjo en el primer año (37%). La calidad de vida global (56,6/100) y general (90,7/148) tuvieron las puntuaciones más bajas en la etapa de pancitopenia, con mejores tasas en el quinto año (80,4/100) y (116,1/148), respectivamente. El análisis con el modelo lineal generalizado mixto mostró cambios significativos en las puntuaciones de los dominios de calidad de vida relacionada con la salud entre las etapas a lo largo del tiempo. Se confirmó la hipótesis de que los pacientes con mejores puntajes en los dominios de calidad de vida relacionada con la salud observados al inicio del tratamiento tienen mayor sobrevida. Los resultados del estudio infieren las correlaciones entre los dominios medidos y así encuentran apoyo en el modelo teórico conceptual utilizado. Los aportes consisten en reafirmar la dimensionalidad del constructo calidad de vida relacionada con la salud, además de sumar conocimientos sobre los cambios autopercibidos por los pacientes durante el tratamiento.

Hematological changes in SARS-COV-2 positive patients

Abstract Introduction The novel SARS-CoV-2 infection has been spreading around the world since January 2020 causing the Corona Virus Disease 2019. Leukopenia, lymphopenia and hypercoagulability with elevated D- Dimers have been described in COVID-19 patients to date. This study aimed to clarify if some blood parameters can be used as biomarkers to facilitate diagnosis and establish prognosis. Methods: We selected patients who had tested positive for SARS-CoV-2 and had had a hemogram performed between the March 15 and April 15, 2020. Socio-demographic and analytical data were obtained from 274 patients at admission in two Portuguese public hospitals. We then analyzed the hemogram parameters at admission in the intensive care and collected data on patient survival during the SARS-CoV-2 disease follow-up. The data were analyzed using appropriate statistical tests. Results: Patients requiring the intensive care unit (ICU) present an increase in leukocytes and neutrophils (+3.1 × 109/L and +6.4 × 109/L, respectively), a lymphocyte decrease and a platelet rise (-1.6 × 109/L and +60.8 × 109/L, respectively). The erythrocytes, hemoglobin and median globular volume tend to decrease (-0.5 × 1012, - 1.2 g/dL; -3 fL, respectively). The lactic acid dehydrogenase (LDH) at admission was significantly higher (+58.1 U/L). The age, sex, platelets, lymphocyte count neutrophil counts, neutrophil/lymphocyte ratio, erythrocytes and cell hemoglobin concentration mean (CHCM) are independently associated with mortality (odds ratio (OR) = 0.046, p < 0.001; OR = 0.2364, p= 0.045; OR = 9.106, p= 0.001; OR = 0.194, p= 0.033; OR = 0.062, p= 0.003; OR = 0.098, p= 0.002; OR = 9.021, p < 0.001; OR = 7.016, p= 0.007, respectively). Conclusion The hematological data at admission in the health care system can predict the mortality of the SARS-CoV-2 infection and we recommend its use in the clinical decisions and patient prognosis evaluation.

Dysregulation in erythrocyte dynamics caused by SARS-CoV-2 infection: possible role in shuffling the homeostatic puzzle during COVID-19

Abstract Introduction The evolving COVID-19 pandemic became a hallmark in human history, not only by changing lifestyles, but also by enriching scientific knowledge on viral infection and its consequences. Objective Although the management of cardiorespiratory changes is pivotal to a favorable prognosis during severe clinical findings, dysregulation of other systems caused by SARS-CoV-2 infection may imbalance erythrocyte dynamics, such as a bidirectional positive feedback loop pathophysiology. Method and Results Recent evidence shows that SARS-CoV-2 is capable of affecting the genetics and dynamics of erythrocytes and this coexists with a non-homeostatic function of cardiovascular, respiratory and renal systems during COVID-19. In hypothesis, SARS-CoV-2-induced systematical alterations of erythrocytes dynamics would constitute a setpoint for COVID-19-related multiple organ failure syndrome and death. Conclusion The present review covers the most frequent erythrocyte-related non-homeostatic findings during COVID-19 capable of providing mechanistic clues of SARS-CoV-2-induced infection and inspiring therapeutic-oriented scientific evidence.

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.

Hepatic manifestations of hematological diseases / 中华肝脏病杂志

Liver involvement is often observed in hematological disorders, resulting in liver abnormality, including unconjugated hyperbilirubinemia, monoclonal hyperglobulinemia, portal vein, or hepatic vein thrombosis or portal hypertension, hepatosplenomegaly, or iron accumulation in the liver. Here we summarize the major hematological diseases that often affect the liver: hemolytic anemia, defect in coagulation or anti-coagulation factors, myeloproliferative neoplasm, hemophagocytic lymphohistiocytosis, multiple myeloma, leukemia, and lymphoma. We hope this review will help clinicians diagnose and manage the patients with liver involvement by hematological disorders.

The Correlation Analysis between the Titer of IgG Anti-A/B Erythrocyte Antibody In Vivo of the Neonate and the Severity of Hemolytic Disease of Newborn / 中国实验血液学杂志

OBJECTIVE@#To investigate the titer of IgG anti-A/B erythrocyte antibody in vivo of the neonate with hemolytic disease of newborn(HDN), and explore its clinical valua in evaluating the severity of HDN.@*METHODS@#300 neonates with HDN, 50 neonates with neonatal hyperbilirubinemiain and 50 healthy neonates were selected as research object and Microtubes Gel Test was used to detect the titer of IgG anti-A/B erythrocyte antibody in vivo. Their clinical data and their mothers' prenatal examination data were retrospectively analyzed. Three hemolysis tests (direct antiglobulin test, free antibody test and release test), irregular antibody screening, and the titer of IgG anti-A/B blood group antibody was determined by serological method. Red blood cells(RBC), hemoglobin(Hb), reticulocytes(Ret) and nucleated red cells were detected by hematology analyzer. Indirect bilirubin and albumin(Alb) were detected by biochemical analyzer. The relationship between the titer of IgG anti-A/B erythrocyte antibody in vivo and the severity of HDN was analyzed.@*RESULTS@#There were six serological diagnosis modes in the HDN group,the difference between modes was statistically significant (P<0.05). The antibody titer relationship between HDN neonates and pregnant women was positive correlation(r=0.8302). The highest antibody titer of release test and free antibody test were 1∶32 and 1∶2, and the difference was statistically significant（P<0.05）. RBC, Hb and Alb in HDN patients were lower than those in neonatal hyperbilirubinemia patients and healthy neonates (P<0.05), and were negatively relevant with antibody titer in vivo (r=-0.8016). Bilirubin content in HDN patients were higher than those in neonatal hyperbiliru binemia patients and healthy neonates group(P<0.05), and was positively relevant with antibody titer in vivo (r=0.8731). The hospital day in HDN patients was significantly relevant with the antibody titer in vivo (r=0.8547), but not with the age, sex, weight and ABO blood types (P>0.05).@*CONCLUSION@#The detection of antibody titer in HDN patients can be used to evaluate the antibody concentration in vivo, predict the ability of antibody to induce erythrocyte hemolysis, and help to judge the serenrity and prognosis of HDN.