A retrospective study of ovarian tissue cryopreservation in female patients with hematological diseases for fertility preservation.

PURPOSES: To investigate the effect and safety of ovarian tissue cryopreservation (OTC) for fertility preservation in female patients with hematological diseases. METHODS: We designed a retrospective study. The clinical data of patients with hematological diseases undergoing OTC admitted to Peking University People's Hospital from April 2017 to January 2023 were analyzed and summarized. RESULTS: A total of 24 patients were included in the study, including 19 patients with malignant hematological diseases and 5 patients with non-malignant hematological diseases. The former included 14 patients with acute leukemia, 1 patient with chronic leukemia, and 4 patients with myelodysplastic syndrome, while the latter 5 patients were aplastic anemia (AA). 16 patients had received chemotherapy before OTC. The average age of 24 patients was 22.80 ± 6.81 years. The average anti-Mullerian hormone (AMH) was 1.97 ± 2.12 ng/mL, and the average follicle-stimulating hormone (FSH) was 7.01 ± 4.24 IU/L in examination before OTC. FSH was greater than 10.0 IU/L in 4 cases. The pre-OTC laboratory tests showed that the average white blood cell (WBC) count was (3.33 ± 1.35) × 109/L, the average hemoglobin was 91.42 ± 22.84 g/L, and the average platelet was (147.38 ± 114.46) × 109/L. After injection of recombinant human granulocyte colony-stimulating factor (rhG-CSF), blood transfusion, and iron supplementation in pre-OTC treatment, the average WBC count was (4.91 ± 3.07) × 109/L, the average hemoglobin was 98.67 ± 15.43 g/L, and the average platelet was (156.38 ± 103.22) × 109/L. Of the 24 patients, 22 underwent laparoscopic bilateral partial oophorectomy and oophoroplasty, and 2 underwent laparoscopic unilateral oophorectomy. The average duration of OTC was 59.54 ± 17.58 min, and the average blood loss was 32.1 ± 41.6 mL. The maximum blood loss was 200 mL. There was no significant difference in WBC count and hemoglobin concentration after OTC compared to pre-OTC period. Only the platelet count after OTC surgery was significantly different from that before surgery ([134.54 ± 80.84 vs. 156.38 ± 103.22] × 109/L, p < 0.05). None of the 24 patients had serious complications after OTC. 2 patients had mild infection symptoms, but both recovered well. 23 patients underwent hematopoietic stem cell transplantation (HSCT) after OTC. The median and interquartile range from OTC to the pretreatment of HSCT was 33 (57) days, and the median and interquartile range from OTC to HSCT was 41 (57) days. Seven of them began pretreatment of HSCT within 20 days and began HSCT within 30 days after OTC. All patients were followed up. Of the 23 patients who underwent HSCT after surgery, 22 presented with amenorrhea and 1 with scanty menstrual episodes. Seven patients underwent hormone replacement therapy (HRT) after HSCT. A patient with AA underwent ovarian tissue transplantation (OTT) 3 years after HSCT and resumed regular menstruation 6 months after OTT. CONCLUSIONS: Ovarian tissue cryopreservation has a promising future in fertility protection in patients with hematological diseases. However, patients with hematological malignancies often have received gonadotoxic therapy before OTC, which may be accompanied by myelosuppression while patients with non-malignant hematological diseases often present with severe hemocytopenia. So perioperative complete blood count of patients should be paid attention to. There was no significant difference in the WBC count and hemoglobin concentration in patients with hematological diseases before and after OTC surgery, and the platelet count decreased slightly within the normal range. Infection is the most common post-OTC complication, and HSCT pretreatment can be accepted as early as the 10th day after OTC. OTC has no adverse effects on patients with hematological diseases and does not delay HSCT treatment. For young patients with hematological diseases, OTC is an effective method of fertility preservation.

Αlpha-thalassemia: A practical overview.

α-Thalassemia is an inherited blood disorder characterized by decreased synthesis of α-globin chains that results in an imbalance of α and ß globin and thus varying degrees of ineffective erythropoiesis, decreased red blood cell (RBC) survival, chronic hemolytic anemia, and subsequent comorbidities. Clinical presentation varies depending on the genotype, ranging from a silent or mild carrier state to severe, transfusion-dependent or lethal disease. Management of patients with α-thalassemia is primarily supportive, addressing either symptoms (eg, RBC transfusions for anemia), complications of the disease, or its transfusion-dependence (eg, chelation therapy for iron overload). Several novel therapies are also in development, including curative gene manipulation techniques and disease modifying agents that target ineffective erythropoiesis and chronic hemolytic anemia. This review of α-thalassemia and its various manifestations provides practical information for clinicians who practice beyond those regions where it is found with high frequency.

First Clinical Nutrition Outpatient Consultation: A Review of Basic Principles in Nutritional Care of Adults with Hematologic Disease.

Methods: A bibliographic survey was carried out between 2020 and 2022 using two databases: PubMed/MEDLINE and Scientific Electronic Library Online (SciELO) and the information source Academic Google, irrespective of language or geography. Results: In the first nutrition consultation (FNC), there should be an investigative direction focused on nutritional interventions in the short, medium, and long term. The record in the patient's medical record is relevant for carrying out the consultation, according to the recommendations of the normative councils of medicine and nutrition. The main steps to be followed are the investigation of the presence of food allergies and intolerances; the drugs/nutritional supplements in use; changes in the digestive tract; the presence or absence of picamalacia; and socioeconomic and lifestyle data. In addition, it is necessary to carry out laboratory evaluations, semiological assessment, anthropometric assessment, and assessment of food consumption. In the end, the nutritional approach should be composed of calculation of energy and macronutrient and micronutrient needs, intervention in nutritional status deviations, nutritional guidelines, and nutritional therapeutic planning of return, focusing on adherence to treatment. Conclusion: The first nutrition consultation may represent investigative steps that help the clinical nutritionist in the management, allowing a longitudinal and specific nutritional therapeutic planning for patients assisted in large reference centers for hematological disease.

Assessment of mortality-related risk factors and effective antimicrobial regimens for treatment of bloodstream infections caused by carbapenem-resistant Pseudomonas aeruginosa in patients with hematological diseases.

Background: Infections caused by carbapenem-resistant Pseudomonas aeruginosa (CRPA) are related to higher mortality. The objective of this study was to explore clinical outcomes of CRPA bacteremia, identify risk factors and also, compare the efficacy of traditional and novel antibiotic regimens. Methods: This retrospective study was conducted at a blood diseases hospital in China. The study included hematological patients who were diagnosed with CRPA bacteremia between January 2014 and August 2022. The primary endpoint was all-cause mortality at day 30. Secondary endpoints included 7-day and 30-day clinical cure. Multivariable Cox regression analysis was employed to identify mortality-related risk factors. Results: 100 patients infected with CRPA bacteremia were included and 29 patients accepted allogenic-hematopoietic stem cell transplantation. 24 received ceftazidime-avibactam (CAZ-AVI)-based therapy and 76 received other traditional antibiotics. 30-day mortality was 21.0%. Multivariable cox regression analysis showed neutropenia >7 days after bloodstream infections (BSI) (P=0.030, HR: 4.068, 95%CI: 1.146~14.434), higher Pitt bacteremia score (P<0.001, HR:1.824, 95%CI: 1.322~2.517), higher Charlson comorbidity index (P=0.01, HR: 1.613, 95%CI: 1.124~2.315) and bacteremia due to multidrug-resistant Pseudomonas aeruginosa (MDR-PA) (P=0.024, HR:3.086, 95%CI: 1.163~8.197) were identified as independent risk factors of 30-day mortality. After controlling for confounders, an additional multivariable cox regression analysis revealed definitive regimens containing CAZ-AVI were associated with lower mortality in CRPA bacteremia (P=0.016, HR: 0.150, 95%CI: 0.032~0.702), as well as in MDR-PA bacteremia (P=0.019, HR: 0.119, 95%CI: 0.020~0.709). Conclusions: For patients with hematological diseases and CRPA bacteremia, 30-day mortality rate was 21.0% (21/100). Neutropenia >7 days after BSI, higher Pitt bacteremia score, higher Charlson comorbidity index and bacteremia due to MDR-PA increased 30-day mortality. CAZ-AVI-based regimens were effective alternatives for bacteremia due to CRPA or MDR-PA.

Severe hemolytic disease of the newborn caused by JKb antibody: Two case reports and literature review.

BACKGROUND: JKb antibody rarely causes severe hemolytic disease in the newborn except in 1 case, required blood exchange transfusion but later died of intractable seizure and renal failure. Here we describe 2 cases of JKb-induced severe neonatal jaundice requiring blood exchange transfusion with good neurological outcome. CASE PRESENTATION: Two female Chinese, ethnic Han, term infants with severe jaundice were transferred to us at the age of 5- and 4-day with a total bilirubin of 30.9 and 25.9 mg/dL while reticulocyte counts were 3.2% and 2.2%, respectively. Both infants were not the firstborn to their corresponding mothers. Direct and indirect Coombs' tests were positive, and JKb antibody titers were 1:64 (+) for both mothers. Phototherapy was immediately administered, and a blood exchange transfusion was performed within 5 hours of admission. Magnet resonance image showed no evidence of bilirubin-induced brain damage, and no abnormal neurological finding was detected at 6 months of life. CONCLUSION: JKb antibody-induced hemolytic disease of the newborn usually leads to a benign course, but severe jaundice requiring blood exchange transfusion may occur. Our cases suggest good outcomes can be achieved in this minor blood group-induced hemolytic disease of the newborn if identified and managed early enough.

A Pilot Survey of Integrative Oncology in Hospitalized Children Undergoing Aggressive Therapy for Cancer and Blood Disorders at a Single Pediatric Cancer Center.

OBJECTIVES: To assess treatment modalities and patients' attitude regarding integrative oncology with a special focus on Kampo in hospitalized children for hematological diseases and solid tumors. METHODS: All children who were hospitalized for hematological or oncological diseases at the Department of Pediatrics, Nagoya University Hospital, between January 25 and February 25, 2018, were invited to participate in this prospective survey. RESULTS: Forty-eight patients responded to the survey. These included 27 patients aged ≤6 years, 11 aged ≥13 years, and 10 aged 7 to 12 years; 19 were diagnosed with a hematological malignancy, 9 with a nonmalignant hematological/immunological disease, and 20 with solid tumors. In all, 42% of patients were administered pharmaceutical-grade Kampo extracts, and 80% reported high effectiveness. Other modalities were used much less frequently. Oral administration of herbal extracts was challenging in children treated with Kampo. Integrated use of Kampo in pediatric hematology/oncology was desired in 77%, and 79% wished for more information about Kampo. In all, 90% desired to be seen by a pediatric hematologist/oncologist specializing in Kampo. CONCLUSION: Contribution of Kampo to pediatric hematology/oncology was highly appreciated during aggressive therapy for cancer and blood disorders.

History and current standard of postnatal management in hemolytic disease of the fetus and newborn.

Since the discovery of the Rh blood group system in 1940, a greater understanding of hemolytic disease of the fetus and newborn (HDFN) was gained. In the years thereafter, researchers and clinicians came to the current understanding that fetal and neonatal red blood cells (RBC) are hemolyzed by maternal alloantibodies directed against RBC antigens potentially leading to severe disease. Preventative measures, such as Rhesus(D) immunoprophylaxis (RhIG), have greatly decreased the prevalence of Rh(D)-mediated HDFN, although a gap between high-income countries and middle- to low-income countries was created largely due to a lack in availability and high costs of RhIG. Other important developments in the past decades have improved the identification, monitoring, and care of pregnancies, fetuses, and neonates with HDFN. Prenatally, fetal anemia may occur and intrauterine transfusions may be needed. Postnatally, pediatricians should be aware of the (antenatally determined) risk of hemolysis in RBC alloimmunization and should provide treatment for hyperbilirubinemia in the early phase and monitor for anemia in the late phase of the disease. Through this review, we aim to provide an overview of important historic events and to provide hands-on guidelines for the delivery and postnatal management of neonates with HDFN. Secondarily, we aim to describe recent scientific findings and evidence gaps. CONCLUSION:  Multiple developments have improved the identification, monitoring, and care of pregnancies and neonates with HDFN throughout the centuries. Pediatricians should be aware of the (antenatally determined) risk of hemolysis in RBC alloimmunization and should provide treatment for hyperbilirubinemia in the early phase and monitor for late anemia in the late phase of the disease. Future studies should be set in an international setting and ultimately aim to eradicate HDFN on a global scale. WHAT IS KNOWN: • Developments have led to a greater understanding of the pathophysiology, an improved serological identification and monitoring of at-risk cases and the current pre- and postnatal treatment. WHAT IS NEW: • This review provides the pediatrician with hands-on guidelines for the delivery and postnatal management of neonates with HDFN. • Future studies should be set in an international setting with the ultimate aim of eradicating HDFN.

Clinical practice guidelines for the optimization of hemato-oncological care: the ICOPraxis.

INTRODUCTION: The Catalan Institute of Oncology (ICO) is a Comprehensive Cancer Center (CCC) responsible for the oncological care of 46% of the Catalan population. OBJECTIVE: Given the increasing and ongoing approval of onco-hematological treatments, the professionals at the ICO decided to have clinical practice guidelines (called ICOPraxis) based on available evidence. In this report, we intend to share how the ICOPraxis has developed and what its impact has been in the 14 years it has been running. RESULTS: In the 14 years, since the project has been running, 17 clinical practice guidelines (some of them with several editions) have been prepared for major onco-hematology clinical conditions. These guidelines will be utilized in the four ICO centers (Girona, Badalona, Tarragona, and Hospitalet) and ICO works in a network with 18 regional hospitals. Between 2018 and 2022, the guidelines have been viewed 38.645 times and downloaded 24.614 times, with an average time spent on each page of 3 min. The ICOPraxis have been consulted in 25 countries in America (3.163 views), 20 countries in Europe (35.365 views), 10 countries in Asia (36 views), and 3 countries in Africa (12 views). The country with the highest number of downloads is Spain with a total of 34.742 downloads (Analytics [Internet]). CONCLUSION: The ICOPraxis have succeeded in establishing an evidence-based system that facilitates prescription decision-making according to the established harmonization process and reduction in variability in treatments, increasing equity in our population.

Exploring Yoga Instructors' Experiences Delivering Yoga to Children and Adolescents Affected by Cancer or Blood Disease.

Yoga may offer benefits for children and adolescents affected by cancer or blood disease, yet there are challenges in translating evidence to practice. Yoga instructors are critical for the delivery of yoga. Understanding yoga instructors' experiences offering yoga to children and adolescents affected by cancer or blood disease could provide information to guide required competencies and training, as well as elucidate factors to consider in future research and programs. Therefore, the present study sought to understand yoga instructors' lived experiences preparing for and facilitating yoga for children and adolescents affected by cancer or blood disease. Fourteen yoga instructors with experience facilitating yoga for this population participated in semi-structured interviews, which were transcribed verbatim and analyzed using principles of interpretive description and thematic analysis. Five unique themes were identified: (1) "I believe in and see the perceived benefits of yoga on and off the mat"; (2) "I feel equipped to deliver yoga but desire further training"; (3) "what I need to deliver a safe yoga program"; (4) "I must be adaptable to successfully facilitate a yoga program"; and (5) "what I need to ensure yoga is widely available." Findings highlight the varied and comprehensive training opportunities yoga instructors sought, while elucidating their training limitations. Yoga instructors shared their perspectives on concerted attention to safety, effectiveness, and access when developing research and programs. This study represents a first step toward defining required competencies for delivering yoga to this population and lays the foundation for future research and programs.

Analysis of dose and duration dependent effects of Allium sativum Linn and other hypocholesterolemic agents exhibited on dyslipidemia in patients with essential hypertension.

Globally, cardiovascular diseases (CVD) are one of the most significant cause of organ failure, mortality and substantial escalation of health care cost. Dyslipidemia and hypertension poses significant risks in cardiovascular diseases and morbidity and mortality can be minimized by altering risks factors. Dyslipidemia is one of the major leading cause of rise of global incidences and socioeconomic burden which necessitates to explore the pharmacological options of significant antilipemic activity with minimal untoward effects at affordable price. Allium Sativum (Garlic) proved medicinally effective in different clinical trials, but further investigations are required to investigate its effects on diabetes, hypertension and dyslipidemia based on variable doses and duration. In this study dose and duration dependent effects of Garlic were evaluated on hypertensive patients with dyslipidemia. Study was randomized, single blind and placebo controlled. Effects of tablets of garlic (KWAI) 300 mg in doses of daily 0.3g, 0.6g, 0.9g, 1.2g and 1.5g for 24 weeks were compared in humans. Each tablet contains 1.3 percent of alliin and 0.6 % of allicin. Results showed remarkable improvements in different serum lipid levels (Cholesterol, Triglycerides, Low density lipoproteins and High density lipoproteins) based on different doses and duration as compared to placebo and standard Lipid-lowering agent simvastatin.

Evaluation of a mobile application tool (BiliNorm) to improve care for newborns with hyperbilirubinemia in Indonesia.

BACKGROUND: Severe hyperbilirubinemia is more frequent in low- and middle-income countries such as Indonesia than in high-income countries. One of the contributing factors might be the lack of adherence to existing guidelines on the diagnosis and treatment of hyperbilirubinemia. We developed a new national guideline for hyperbilirubinemia management in Indonesia. To help healthcare workers use this guideline, a web-based decision support tool application may improve both the adherence to the guideline and the care for infants with hyperbilirubinemia. METHODS: We developed a web-based application (BiliNorm) to be used on a smartphone that displays the bilirubin level of the patient on the nomogram and advises about the treatment that should be started. Healthcare workers of two teaching hospitals in East Java, Indonesia, were trained on the use of BiliNorm. At 6 months after the introduction, a questionnaire was sent to those who worked with the application enquiring about their experiences. An observational study was conducted in two time epochs. A chart review of infants with hyperbilirubinemia in the two hospitals was sent. The appropriateness of hyperbilirubinemia management during a 6-month period before BiliNorm introduction was compared to that during a 7-month period after its introduction. RESULTS: A total of 43 participants filled in the questionnaire, the majority (72%) of them indicated that BiliNorm was well received and easy to use. Moreover, 84% indicated that BiliNorm was helpful for the decision to start phototherapy. Chart review of 255 infants before BiliNorm introduction and that of 181 infants after its introduction indicated that significantly more infants had received treatment according to the guideline (38% vs 51%, p = 0.006). Few infants received phototherapy, but bilirubin level was not measured (14% vs 7%, p = 0.024). There was no difference in the proportion of infants who were over- and under-treated (34% vs 32% and 14% vs 10%, respectively). CONCLUSIONS: The web-based decision tool BiliNorm appears to be a valuable application. It is easy to use for healthcare workers and helps them adhere to the guideline. It improves the care for infants with hyperbilirubinemia and may help reduce the incidence of severe hyperbilirubinemia in Indonesia.

Discharge of newborns with risk factors of severe hyperbilirubinemia: description of a hospital at home-based care monitoring and phototherapy.

Neonatal jaundice is common and associated with delay in hospital discharge and risk of neurological sequelae if not treated. The objectives of the study were to report on our experience of the monitoring and treatment of neonatal jaundice in a home care setting and its feasibility and safety for neonates with high risk of severe hyperbilirubinemia. The 2-year study has been led in the greater Paris University Hospital At Home (Assistance Publique-Hôpitaux de Paris). The device of the intervention was the Bilicocoon® Bag, a light-emitting diode sleeping bag worn by the neonate when the total serum bilirubin value exceeds intensive phototherapy threshold, according to the guidelines from the American Academy of Pediatrics. One hundred and thirty-nine neonates had participated in the intervention and 39 (28%) were treated by phototherapy at home, as continuation of inpatient phototherapy or started at home. Seventy-five percent of the sample had more than two risk factors for development of severe hyperbilirubinemia. Twenty five percent of the cohort who received phototherapy at home had lower gestational age (p < 0.014) and had younger age at discharge from maternity (p < 0.09). Median length of stay in hospital at home was 5 days. Two patients needed readmission in conventional hospital (1%) for less than 24 h. In multivariate model, the length of stay decreased with the higher gestational age (p < 0.001) and increased significantly with the older age at discharge, the birth weight < 10th percentile, and a treatment by phototherapy at home.    Conclusion: Hospital at home, which is a whole strategy using an effective and convenient phototherapy device combined with a specialized medical follow-up, could be an alternative to conventional hospitalization for neonates at high risk of severe jaundice. The maternity discharge is facilitated, the mother-infant bonding can be promoted, and the risk of conventional rehospitalization is minimal, while guaranteeing the safety of this specific care. What is Known: • Managing neonatal jaundice is provided in conventional hospital with phototherapy. • Neonatal jaundice increases the risk of prolonged hospitalization or readmission. What is New: • Phototherapy is feasible in hospital at home for neonates with high risk of severe hyperbilirubinemia. • The care pathway of neonates from conventional hospital to hospital at home is described.

Music Therapy for Children with Oncology & Hematological Conditions and Their Families: Advancing the Standards of Psychosocial Care.

Background: Diagnosis and treatment of cancer and blood disorders in childhood, adolescence and young adulthood has a significant impact on patients and families. The Psychosocial Standards of Care project, initiated in 2012, resulted in 15 Psychosocial Standards (PSS) that guide the care patients and families receive throughout treatment. As members of the multidisciplinary psychosocial care team, music therapists play an important role in the advancing the PSS. Most surveys have focused on other commonly provided services (e.g., social work, child life), leaving gaps in our understanding about the availability and use of music therapy services to advance PSS. This paper offers an initial description of how music therapy services contribute to the provision of care under these Standards. Methods: We analyze how music therapy services promote PSS through synthesis of a music therapy clinical practice survey, published literature, and scope of practice documents. A brief overview of music therapy services structure, PSS that music therapy services currently address, and two clinical program descriptions are included. Results: Music therapy services address 9 of the 15 PSS and are well integrated within the larger program of psychosocial care. Findings suggest integration of music therapy services can help ensure personalized, comprehensive care and efficient use of often-limited psychosocial care resources. Discussion: Nurses, as members of the psychosocial and medical teams are uniquely positioned to identify patient and family care needs and refer patients for services. Understanding how music therapy services address PSS and most importantly, the needs of patients and families, will optimize their care.

High rates of neonatal polycythemia and hyperbilirubinemia during the first phase of COVID-19 pandemic in Italy: a single-center experience.

In our third-level Neonatal Unit in Northern Italy, we recorded a high rate of neonatal hyperbilirubinemia requiring phototherapy in March-November 2020, during the first phase of COVID-19 pandemic, compared to the previous year (198/1348, 14.2%, vs 141/1432, 9.8%, p = 0.0004). Supposing it could be the result of neonatal polycythemia, we evaluated capillary hematocrit (Hct) and the rate of hyperbilirubinemia in all newborns ≥36 weeks gestational age born in December 2020. Out of 73 neonates, 37 had Hct ≥65% (50.7%). However, as capillary blood samples may overestimate Hct by 5-15%, even downsizing all values by 15%, Hct was still ≥65% in 9/73 neonates (12.3%), much higher than 0.4-5% prevalence of polycythemia reported in healthy newborns. All those newborns were singleton and healthy, with no clinical signs of hyperviscosity and no underlying factors predisposing to polycythemia. Out of 73 newborns, 13 (17.8%) developed hyperbilirubinemia requiring phototherapy. Their mean Hct value was 66.3 ± 8.2%. Since hyperbilirubinemia is common in the offspring of women with SARS-CoV-2 infection and we recorded increased rates of neonatal hyperbilirubinemia in the first phase of COVID-19 pandemic, it could be hypothesized that even asymptomatic Sars-CoV2 infection during pregnancy might cause placental vascular malperfusion, eliciting polycythemia in the fetus as a compensatory response, that could be the link between COVID-19 in the mothers and hyperbilirubinemia in the newborns.

Blood-Enriching Effects and Immune-Regulation Mechanism of Steam-Processed Polygonatum Sibiricum Polysaccharide in Blood Deficiency Syndrome Mice.

Polygonatum sibiricum Red. has been used as a medicinal herb and nutritional food in traditional Chinese medicine for a long time. It must be processed prior to clinical use for safe and effective applications. However, the present studies mainly focused on crude Polygonatum sibiricum (PS). This study aimed to investigate the chemical properties, blood-enriching effects and mechanism of polysaccharide from the steam-processed Polygonatum sibiricum (SPS), which is a common form of PS in clinical applications. Instrumentation analyses and chemistry analyses revealed the structure of SPS polysaccharide (SPSP). A mice model of blood deficiency syndrome (BDS) was induced by acetylphenylhydrazine (APH) and cyclophosphamide (CTX). Blood routine test, spleen histopathological changes, serum cytokines, etc. were measured. The spleen transcriptome changes of BDS mice were detected by RNA sequencing (RNA-seq). The results showed that SPSP consists predominantly of Gal and GalA together with fewer amounts of Man, Glc, Ara, Rha and GlcN. It could significantly increase peripheral blood cells, restore the splenic trabecular structure, and reverse hematopoietic cytokines to normal levels. RNA-seq analysis showed that 122 differentially expressed genes (DEGs) were obtained after SPSP treatment. GO and KEGG analysis revealed that SPSP-regulated DEGs were mainly involved in hematopoiesis, immune regulation signaling pathways. The reliability of transcriptome profiling was validated by quantitative real-time PCR and Western blot, and the results indicated that the potential molecular mechanisms of the blood-enriching effects of SPSP might be associated with the regulating of JAK1-STAT1 pathway, and elevated the hematopoietic cytokines (EPO, G-CSF, TNF-α and IL-6). This work provides important information on the potential mechanisms of SPSP against BDS.

Alpha- and Beta-thalassemia: Rapid Evidence Review.

Thalassemia is a group of autosomal recessive hemoglobinopathies affecting the production of normal alpha- or beta-globin chains that comprise hemoglobin. Ineffective production of alpha- or beta-globin chains may result in ineffective erythropoiesis, premature red blood cell destruction, and anemia. Chronic, severe anemia in patients with thalassemia may result in bone marrow expansion and extramedullary hematopoiesis. Thalassemia should be suspected in patients with microcytic anemia and normal or elevated ferritin levels. Hemoglobin electrophoresis may reveal common characteristics of different thalassemia subtypes, but genetic testing is required to confirm the diagnosis. Thalassemia is generally asymptomatic in trait and carrier states. Alpha-thalassemia major results in hydrops fetalis and is often fatal at birth. Beta-thalassemia major requires lifelong transfusions starting in early childhood (often before two years of age). Alpha- and beta-thalassemia intermedia have variable presentations based on gene mutation or deletion, with mild forms requiring only monitoring but more severe forms leading to symptomatic anemia and requiring transfusion. Treatment of thalassemia includes transfusions, iron chelation therapy to correct iron overload (from hemolytic anemia, intestinal iron absorption, and repeated transfusions), hydroxyurea, hematopoietic stem cell transplantation, and luspatercept. Thalassemia complications arise from bone marrow expansion, extramedullary hematopoiesis, and iron deposition in peripheral tissues. These complications include morbidities affecting the skeletal system, endocrine organs, heart, and liver. Life expectancy of those with thalassemia has improved dramatically over the past 50 years with increased availability of blood transfusions and iron chelation therapy, and improved iron overload monitoring. Genetic counseling and screening in high-risk populations can assist in reducing the prevalence of thalassemia.

Serum Pharmacochemistry Combining Network Pharmacology to Discover the Active Constituents and Effect of Xijiao Dihuang Tang Prescription for Treatment of Blood-Heat and Blood-Stasis Syndrome-Related Disease.

Xijiao Dihuang Tang (XDT), a classic TCM prescription, has been used to clinically treat blood-heat and blood-stasis syndrome- (BHSS-) related diseases, including hemorrhagic stroke and sepsis. However, the active constituents and mechanism of XDT in the treatment of BHSS-related diseases have not been elucidated due to the lack of appropriate methodologies. In this study, serum pharmacochemistry and network pharmacology were used to explore the active constituents and the mechanism of XDT in the treatment of BHSS-related diseases. The effects of XDT were evaluated using dry yeast-induced rats as rat models with BHSS, which demonstrated the antipyretic and anticoagulant properties of XDT. The HPLC-QTOF/MS/MS assay was used to identify 60 serum constituents of XDT (SCXDT). Then, 338 targets of 60 SCXDT were predicted by integrating multiple databases and the MACCS fingerprint similarity prediction method. The degree of topological properties with targets of 19 key active constituents in SCXDT was identified and evaluated in glutamate-induced PC12 cells. Subsequently, 338 targets of 60 SCXDT were mainly involved in biological processes such as inflammation, coagulation, cell proliferation, and apoptosis, as well as oxidative contingencies via compound-target-disease network analysis. The core targets including IL-1ß, IL-6, TNF, NOS3, and MAPK1 were identified using protein-protein interaction network analysis, whereas dozens of signaling pathways such as the p38MAPK signaling pathway were identified using functional pathway enrichment analysis. The results indicated that XDT has broad therapeutic and neuroprotective effects on inflammation, coagulation, oxidative stress, cell proliferation, and apoptosis in dry yeast-induced rats with BHSS and glutamate-induced PC12 cells by regulating the p38MAPK signaling pathway. This study not only discovered the active constituents of XDT but also elaborated its mechanisms in the treatment of BHSS-related diseases by intervening in a series of targets, signaling pathways, and biological processes such as inflammation, coagulation, oxidative stress, neuroprotection. The findings in this study provide a novel strategy for exploring the therapeutic efficacy of TCM prescriptions.

Induced Pluripotent Stem Cells as a Tool for Modeling Hematologic Disorders and as a Potential Source for Cell-Based Therapies.

The breakthrough in human induced pluripotent stem cells (hiPSCs) has revolutionized the field of biomedical and pharmaceutical research and opened up vast opportunities for drug discovery and regenerative medicine, especially when combined with gene-editing technology. Numerous healthy and patient-derived hiPSCs for human disease modeling have been established, enabling mechanistic studies of pathogenesis, platforms for preclinical drug screening, and the development of novel therapeutic targets/approaches. Additionally, hiPSCs hold great promise for cell-based therapy, serving as an attractive cell source for generating stem/progenitor cells or functional differentiated cells for degenerative diseases, due to their unlimited proliferative capacity, pluripotency, and ethical acceptability. In this review, we provide an overview of hiPSCs and their utility in the study of hematologic disorders through hematopoietic differentiation. We highlight recent hereditary and acquired genetic hematologic disease modeling with patient-specific iPSCs, and discuss their applications as instrumental drug screening tools. The clinical applications of hiPSCs in cell-based therapy, including the next-generation cancer immunotherapy, are provided. Lastly, we discuss the current challenges that need to be addressed to fulfill the validity of hiPSC-based disease modeling and future perspectives of hiPSCs in the field of hematology.

How we coordinate care for uninsured children with nonmalignant hematologic disorders.

Providing medical care for uninsured children with nonmalignant hematologic diagnoses presents unique challenges to medical providers and multidisciplinary staff. Financial and psychosocial stressors can hinder optimal care of the uninsured child. Maximizing coverage of medical costs through patient enrollment in state and charity care programs and capitalizing upon community partnerships can help providers achieve comprehensive care for these children. Collaboration between primary care providers, subspecialists, and multidisciplinary teams can be optimized to facilitate provision of hematology care for uninsured children. We detail our experience in establishing these collaborations to improve access to subspecialty care for children with nonmalignant hematologic disorders.