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OBJECTIVE: To investigate the benefits of menstrual management in women undergoing hematopoietic stem cell transplantation (HSCT), in whom heavy menstrual bleeding (HMB) can be an underestimated bleeding complication. METHODS: This was a retrospective cohort study. Patients who had undergone HSCT in the Gynecological Endocrinology Clinic of Peking University People's Hospital were included over 10 years. The data of hematology and menstruation for all participants were collected. The management methods of the intervention group include gonadotropin-releasing hormone agonists (GnRHa), combined oral contraceptives (COC), or low-dose mifepristone. Patients who did not receive management were included in the control group. RESULTS: There were 112 patients included in the intervention group and 218 patients included in the control group. In all, 90.0%(297/330) of patients presented with HMB before HSCT. In the control group, 83.5%(182/218) of patients experienced menstruation in the laminar air-flow room (LAFR), whereas only 22.3%(25/112) did in the intervention group. After leaving the LAFR, the incidence of recurrent uterine bleeding was significantly reduced in the intervention group (17.9%(20/112/) versus 50.9%(111/218), p < 0.001). Patients who did not undergo menstrual management had a higher risk of bleeding than those who did (odds ratio 18.12, p < 0.001). CONCLUSION: Menstrual management significantly reduces the incidence of HMB in HSCT patients and acts as a protective factor to prevent menstrual bleeding in the LAFR.

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Myelofibrosis (MF), a complex hematological malignancy, presents a diverse array of symptoms, including anemia, constitutional symptoms, bone marrow insufficiency, and splenomegaly. The latter, often necessitating blood transfusions, poses an essential obstacle to MF management. While conventional approaches predominantly involve the use of JAK inhibitors, the potential for exacerbating anemia introduces complexity to the treatment. Nonetheless, Momelotinib stands out as a promising pharmaceutical compound with the potential to revolutionize the field. Momelotinib is an ACVR1 antagonist and a dual inhibitor of the JAK1 and JAK2 enzymes. By targeting MF's hematological and fibrotic aspects, Momelotinib influences iron metabolism by regulating hepcidin. This results in reduced hepcidin expression and increased iron availability, ultimately leading to improved anemia and reduced dependency on blood transfusion. This study aims to provide a concise overview of the pathogenesis of MF and elucidate the mechanism of action of Momelotinib. Subsequently, our review offers a practical summary encompassing the effects of Momelotinib in monotherapy, combined comparative drug therapy, and its associated side effects. Additionally, we explore the application of Momelotinib in other cancer types and investigate predictors for treatment success. Furthermore, we examine the utilization of Momelotinib in patients with liver and kidney failure.

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OBJECTIVES: The incidence of infections in patients with malignant hematologic diseases is extremely high and significantly affects their prognosis. Identifying early and precise biomarkers for infection is crucial for guiding the treatment of infections in these patients. Previous studies have shown that procalcitonin (PCT) can serve as an early diagnostic marker for bloodstream infections in patients with malignant hematologic diseases. This study aims to compare serum PCT levels in these patients with different pathogens, disease types, infection sites, and severity levels. METHODS: Clinical data and laboratory results of infected patients with malignant hematologic diseases treated at the Department of Hematology, the Third Xiangya Hospital of Central South University from January 2018 to August 2023 were collected. General patient information was retrospectively analyzed. Serum PCT levels were compared among patients with different pathogens, types of malignant hematologic diseases, infection sites, and infection severity; Receiver operator characteristic (ROC) curves were used to determine the cut-off values and diagnostic value of serum PCT levels in diagnosing bloodstream infections versus local infections and severe infections versus non-severe infections. Mortality rates after 4-7 days of anti-infective treatment were compared among groups with rising, falling, and unchanged PCT levels. RESULTS: A total of 526 patients with malignant hematologic diseases were included. The main pathogens were Gram-negative bacteria (272 cases, 51.7%), followed by Gram-positive bacteria (120 cases, 22.8%), fungi (65 cases, 12.4%), viruses (23 cases, 4.4%), and mixed pathogens (46 cases, 8.7%). The main types of malignant hematologic diseases were acute myeloid leukemia (216 cases, 41.1%), acute lymphoblastic leukemia (107 cases, 20.3%), and lymphoma (93 cases, 17.7%). Granulocyte deficiency was present in 68.3% (359 cases) of the patients during infection, with severe infection in 24.1% (127 cases). Significant differences in serum PCT levels were found among patients with different types of pathogens (P<0.001), with the highest levels in Gram-negative bacterial infections. Significant differences in serum PCT levels were also found among patients with different types of malignant hematologic diseases (P<0.05), with the highest levels in lymphoma patients. Serum PCT levels were significantly higher in systemic infections and severe infections compared to local infections and non-severe infections (both P<0.001). ROC curve analysis showed that the cut-off values for diagnosing bloodstream infections and severe infections were 0.22 and 0.28 ng/mL, with areas under the curve of 0.670 and 0.673, respectively. After 4-7 days of anti-infective treatment, the mortality rates of the PCT declining, PCT unchanged, and PCT rising groups were 11.9%, 21.2%, and 35.7%, respectively, and pairwise comparisons were statistically significant (all P<0.05). CONCLUSIONS: PCT can be used as an auxiliary indicator for early identification of different pathogens, infection sites, and severity levels in patients with malignant hematologic diseases combined with infections. Dynamic monitoring of PCT levels after empirical antibiotic treatment provides important guidance for assessing patient's prognosis.

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Accumulated evidence emerges that dynamic changes in human gut microbiota and microbial metabolites can alter the ecological balance of symbiotic hosts. The gut microbiota plays a role in various diseases through different mechanisms. More and more attention has been paid to the effects that human microbiota extends beyond the gut. This review summarized the current understanding of the roles that gut microbiota plays in hematopoietic regulation and the occurrence and development of benign and malignant hematologic diseases. The progress of the application of microbiota in treatment was discussed in order to provide new insights into clinical diagnosis and treatment in the future.

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Periodontitis is a common oral condition that can have a significant impact on the overall health of the body. In recent years, attention has been paid to potential relationships between periodontitis and various hematological disorders. This publication aims to present information available in the literature on this relationship, focusing on examples of red blood cell disorders (such as aplastic anemia and sickle cell anemia) and white blood cell disorders (such as cyclic neutropenia, maladaptive trained immunity, clonal hematopoiesis, leukemia, and multiple myeloma). Understanding these associations can help physicians and dentists better diagnose, monitor, and treat patients associated with both groups of conditions, highlighting the need for interdisciplinary care for patients with oral disorders and hematologic diseases.

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Non-Hodgkin lymphoma (NHL) is one of the most common hematological cancers in the United States. The mortality rate of NHL in the United States is the sixth highest among all cancers. Our cross-sectional study aims to examine the trends and disparity in NHL mortality. We analyzed death certificate data from the Centers for Disease Control and Prevention's Wide-Ranging Online Data for Epidemiologic Research (CDC WONDER) United States to determine the NHL mortality trends among the U.S. population aged ≥15 years. NHL (ICD-10 C82-85) was listed as the underlying cause of death. Age-adjusted mortality rates (AAMRs) per 100,000 individuals and joinpoint trend analysis were performed to determine the average annual percent change (AAPC) in AAMR trends. From 1999 to 2020, NHL accounted for 457,143 deaths in the United States, of which 54% are men and 46% are women. The NHL AAMR decreased significantly from 10.59 to 6.21 per 100,000 individuals with an AAPC of -2.55. Men had a higher AAMR than women (10.10 vs 6.29 per 100,000 individuals). Whites recorded the highest AAMR (8.43 per 100,000 individuals), followed by Hispanics (6.32 per 100,000 individuals), Blacks (5.71 per 100,000 individuals), American Indians (5.31 per 100,000 individuals), and Asians (5.10 per 100,000 individuals). Those who lived in the Midwest and the rural areas had the highest AAMR at 8.60 and 8.35 per 100,000 individuals respectively. Despite the declining NHL mortality rate, this study calls for targeted intervention to improve outcomes for susceptible individuals affected by NHL.

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AIMS: Myeloid neoplasms (MNs) with germline predisposition have been recognised as a distinct entity. Emerging evidence suggests that sporadic myelodysplastic syndromes may also harbour undetected germline predispositions. We investigated germline alterations in a cohort of 122 adult Thai MNs. METHODS: MN patients were recruited and tested for germline variants using deep targeted next-generation sequencing. The germline variant was filtered using American College of Medical Genetics classifications and then evaluated for the association with clinical characteristics and outcomes. RESULTS: Our findings revealed pathogenic/likely pathogenic germline alterations in 12 (10%) of the patients. These germline lesions were commonly found in the DNA damage response pathway (n=6, 50%). We also identified novel deleterious FANCA A1219GfsTer59 variants in two patients diagnosed with secondary acute myeloid leukaemia (sAML) from aplastic anaemia and AML with myelodysplasia related. Among sAML, individuals with germline mutations had inferior overall survival compared with those with wild-type alleles (2 months vs 12 months) with HR 4.7 (95% CI 1.0 to 20), p=0.037. Therefore, the presence of pathogenic or likely pathogenic mutations may be linked to inferior survival outcomes. CONCLUSIONS: Our study highlighted that the prevalence of germline predisposition in Southeast Asian populations is comparable to that in Caucasians. This underscores the importance of germline genetic testing within the Asian population.

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Background: Patients with leukemia rely on social and family support. This study aimed to explore the knowledge, attitude, and practice (KAP) toward leukemia among family members of patients with leukemia and the general population in southeast China. Methods: A cross-sectional study was conducted in September 2022 in southeast China (Anhui Province). The KAP scores and demographic data were assessed by questionnaire and analyzed by multivariable logistic regression and structural equation modeling. Results: A total of 760 valid questionnaires were collected, including 117 (15.39%) answered by family members of patients with leukemia. The mean knowledge (8.30 ± 2.79 vs. 8.72 ± 2.56, P = 0.103), attitude (52.17 ± 5.52 vs. 52.27 ± 5.53, P = 0.862), and practice (8.06 ± 2.00 vs. 8.18 ± 2.05, P = 0.547) scores were comparable among family members and the general population. Higher knowledge scores [OR = 1.18 (1.10, 1.27), P < 0.001] and higher attitude scores [OR = 1.05 (1.02, 1.09), P = 0.002] were independently associated with better practice scores. Being a family member of a patient with leukemia had no significant effect on the KAP scores. Conclusion: The participants demonstrated satisfactory knowledge, positive attitude, and appropriate practices toward leukemia, suggesting that access to information about leukemia to the general public might be sufficient in China. Health education might effectively improve knowledge, which could translate into improved attitude and practice.

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OBJECTIVE: To investigate the quality of life (QoL) and the impact of caregiving in family caregivers of hematological cancer patients and its association with patient symptom burden. METHODS: A cross-sectional study including Danish patients (n = 375) and caregivers (n = 140). Caregivers completed scales for anxiety and depression using the Hospital Anxiety and Depression Scale, sleep quality using the Pittsburgh Sleep Quality Index, health related QoL using the 12-item Short-Form Health Survey, and caregiver roles using the Caregiver Roles and Responsibilities Scale. Patients reported symptoms using the MD Anderson Symptom Inventory. Analysis of covariance was used to examine associations between patient symptom burden and caregivers' QoL outcomes. RESULTS: The results show that caregivers experience sleep difficulties, moderate anxiety, and reduced QoL. Patient symptom burden was significantly associated with caregiver anxiety (p = 0.009), and mental well-being (p = 0.002), while patient treatment status was a significant factor associated with caregiver anxiety (p = 0.016), depression (p = 0.009), emotional well-being (p = 0.002), and sleep (p = 0.01). CONCLUSION: Caregivers of patients with hematological cancers undergoing active treatment face a high symptom burden, which significantly impacts their QoL, including sleep, psychological well-being, and emotional health. Patients reported a high symptom burden, and patient symptom burden was significantly associated with caregiver QoL. Adequate patient and caregiver support is needed to promote their well-being and mitigate adverse health effects in caregivers, and this should be acknowledged in the context of caring for patients with hematological cancer.

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BACKGROUND: To develop and internally validate a prediction model for identifying patients with hematologic diseases of fall risk. RESEARCH DESIGN AND METHODS: This is a prospective cohort study from a prospective collection of data for 6 months. We recruited 412 patients with hematologic diseases in medical institutions and home environment of China. The outcome of the prediction model was fall or not. These variables were filtered via univariable logistic analysis, LASSO, and multivariable logistic analysis. We adopt an internal validation method of K-fold cross validation. The area under the ROC curve and the H-L test were used to evaluate the discrimination and calibration of the model. RESULTS: Five influencing factors were identified multivariable logistic regression analysis. The established model equation is as follows: the H-L goodness-of-fit test of the model p > 0.05. The area under the ROC curve of train is 0.957 (95% CI: 0.936 ~ 0.978), and the area under the ROC curve of test is 0.962 (95% CI: 0.884 ~ 1), so the model calibration and discriminant validity are good. CONCLUSION: Our equation has good sensitivity and specificity in predicting the fall risk of patients with hematologic diseases, and has certain positive significance for clinical assessment of their fall risk. TRIAL REGISTRATION NUMBER: ChiCTR2200063940.

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Corynebacterium is generally considered a contaminant in clinical practice. However, it may cause bacteremia in patients with hematologic disorders, and factors that contribute to its mortality are unclear. A case series and systematic literature review identified 96 cases of Corynebacterium bacteremia inhematologic disorderpatients. The median age was 50.5 years (range: 2-93 years), with 79 (82%) patients 18 years or older, and 64 (67%) patients male. Most cases involved hematologic malignancies, and neutropenia was observed in approximately 75% cases. The most common sites of infection/symptoms were skin and soft tissue, respiratory, and catheter-related bloodstream infection. The infection-related mortality was 23%, and univariate analysis showed that age, respiratory infection/symptoms, and source control were significantly associated with infection-related mortality. Multivariate analysis indicates that infection-related mortality was significantly reduced by source control (OR: 0.24, 95% CI: 0.06-0.97, p = 0.046). Therefore, when Corynebacterium infections are suspected, early source control should be considered.

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This article discusses the orofacial clinicoradiographic features of systemic diseases that manifest in the orofacial region. The systemic diseases discussed are grouped into the following: autoimmune diseases, endocrine diseases, bone diseases, hematologic diseases, syndromes, and malignancies. The radiographic manifestation ranges from radiolucent bony destruction, increased bone density, calcification, thinning of cortical plate, loss of trabeculation, missing teeth, and supernumerary teeth. It is imperative for clinicians to be cognizant of these findings, as they may be the first manifestation of these systemic diseases.

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OBJECTIVE: Previous studies have demonstrated that various hematologic diseases (HDs) induce alterations in telomere length (TL). The aim of this study is to investigate whether genetically predicted changes in TL have an impact on the risk of developing HDs. METHODS: GWAS data for TL and 11 HDs were extracted from the database. The R software package "TwoSampleMR" was employed to conduct a two-sample Mendelian randomization (MR) analysis, in order to estimate the influence of TL changes on the risk of developing the 11 HDs. RESULTS: We examined the effect of TL changes on the risk of developing the 11 HDs. The IVW results revealed a significant causal association between genetically predicted longer TL and the risk of developing acute lymphocytic leukemia (ALL), acute myeloid leukemia (AML), chronic lymphocytic leukemia (CLL), mantle cell lymphoma (MANTLE), and hodgkin lymphoma (HODGKIN). However, there was no significant causal relationship observed between TL changes and the risk of developing chronic myeloid leukemia (CML), diffuse large b-cell lymphoma (DLBCL), marginal zone b-cell lymphoma (MARGINAL), follicular lymphoma (FOLLICULAR), monocytic leukemia (MONOCYTIC), and mature T/NK-cell lymphomas (TNK). CONCLUSIONS: The MR analysis revealed a positive association between genetically predicted longer TL and an increased risk of developing ALL, AML, CLL, MANTLE, and HODGKIN. This study further supports the notion that cells with longer TL have greater proliferative and mutational potential, leading to an increased risk of certain HDs.

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BACKGROUND: Diagnosing and treating rare diseases pose significant challenges within global healthcare systems due to their low prevalence and varying criteria for defining them. In Albania, the absence of a dedicated registry for rare diseases exacerbates these challenges. Recognising this gap, a retrospective cross-sectional study was conducted from January 2005 to December 2022 to analyse the incidence and prevalence of rare haematologic diseases in the country, diagnosed in the Hematology Service at the University Hospital Centre "Mother Teresa," which is the sole diagnostic center for blood diseases in Albania. This study aims to provide insights into the frequency of these diseases within the adult Albanian population and seeks to underscore the critical need for improved data collection and research in this field of healthcare. OBJECTIVE: The primary objective of this study is to assess the incidence and prevalence of rare hematologic diseases diagnosed at the Hematology Service of the University Hospital Centre "Mother Teresa" in Albania from January 2005 to December 2022. MATERIALS AND METHODS: This retrospective cross-sectional study employed a descriptive study design, focusing on the analysis of rare hematologic disease incidence and prevalence. The study was conducted exclusively at the University Hospital Centre "Mother Teresa" in Albania, the primary diagnostic center for blood-related disorders in the country. Data collection spanned a period of 18 years, from January 2005 to December 2022, encompassing patient records. Inclusion criteria encompassed adult patients aged 15 years and older who had received diagnoses of rare hematologic diseases during the specified timeframe, without specific operational definitions applied. Non-probability convenience sampling was used, including all eligible cases identified within the study's timeframe, obviating the need for formal sample size calculation. Data were extracted from the records of the Hematology Service at the University Hospital Centre "Mother Teresa," primarily using medical records containing essential patient information. Data analysis utilised software such as EXCEL 16.0 and SPSS (v. 25.0), applying descriptive statistical methods, including frequencies and percentages, to assess the incidence and prevalence of rare hematologic diseases. The study's findings were summarised and presented in a tabular format to provide a clear and concise overview of the results. RESULTS: Our study identified 64 cases of rare hematologic diseases among adults. Notably, primary myelofibrosis (MF) exhibited the highest incidence and prevalence rate, followed by Waldenström macroglobulinemia (WM) and Gaucher disease (GD) emerging as the most prevalent diagnoses after MF, with 16 and 10 cases, respectively. Several ultra-rare diseases, such as Fanconi anemia and chronic eosinophilic leukemia, were also detected, indicating a significant disease burden, while diseases such as Factor X deficiency and Niemann-Pick disease type C were exceptionally rare. CONCLUSION: Diagnosing and treating rare diseases remain formidable challenges in healthcare systems worldwide. This study underscores the need for enhanced awareness, research, and the pressing need for dedicated registries, collaborative research initiatives, and heightened attention to these conditions to enhance our understanding and management of rare hematological diseases, particularly within the Albanian healthcare context.

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Objective To analyze the influencing factors of unplanned extubation(UE)occurrence of peripherally inserted central catheter(PICC)in the patients with hematological diseases.Methods A retro-spective cohort study method was adopted.The data of 7 298 patients with hematological diseases implanted with PICC catheter and followed up to its removal from January 1,2016 to December 31,2020 in the Hematol-ogy Hospital of Chinese Academy of Medical Sciences were collected,including the demographic information,catheterization records,maintenance and extubation records.According to whether UE occurring,they were divided into the UE group(n=262)and normal extubation group(n=7 036).The general data were com-pared between the two groups.The COX regression was used to analyze the influencing factors of UE in pa-tients with hematological diseases.The dose-effect relationship between age and PICC UE occurrence risk was studied by the restrictive cubic spline method.Results The incidence rate of UE was 3.6％(262/7 298).The COX regression analysis results showed that the gender,disease diagnosis,fibrinogen,prothrombin time,PLT,catheter material,number of punctures during catheterization,positioning method of catheter tip,num-ber of catheter-related complications occurrence were related to PICC UE occurrence in the patients with he-matological diseases(P＜0.05).The results of restricted cubic spline showed that there was a"U"-type non-linear relationship between age and UE risk(X2=17.710,P＜0.05),and the risk of UE was the lowest when the age was 30 years old.Conclusion In PICC,the emphasis should be paid to the male patients with hemato-logical malignancies who have repeated punctures during catheterization,no intracardiac electrocardiographic positioning during catheterization,bleeding tendency,indwelling polyurethane catheters and repeated catheter-related complications in order to decrease the UC occurrence probability.

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AIMS: The prognostic impact of programmed death-ligand 1 (PD-L1) cells in classic Hodgkin lymphoma (cHL) tumour microenvironment remains undefined. METHODS: Model development via Transparent Reporting of a multivariable prediction model for Individual Prognosis Or Diagnosis guidelines were followed. PD-L1+ and CD30+ tumoral Reed-Sternberg cells were quantified through whole slide imaging and digital image analysis in 155 digital histopathological slides of cHL. Univariate and multivariate survival analyses were performed. The analyses were reproduced for patients with advanced stages (IIB, III and IV) using the Advanced-stage cHL International Prognostic Index. RESULTS: The PD-L1/CD30 ratio was statistically significantly associated with survival outcomes. Patients with a PD-L1/CD30 ratio above 47.1 presented a shorter overall survival (mean OS: 53.7 months; 95% CI: 28.7 to 78.7) in comparison with patients below this threshold (mean OS: 105.4 months; 95% CI: 89.6 to 121.3) (p=0.04). When adjusted for covariates, the PD-L1/CD30 ratio retained prognostic impact, both for the OS (HR: 1.005; 95% CI: 1.002 to 1.008; p=0.000) and the progression-free survival (HR: 3.442; 95% CI: 1.045 to 11.340; p=0.04) in a clinical and histopathological multivariate model including the male sex (HR: 3.551; 95% CI: 0.986 to 12.786; p=0.05), a percentage of tumoral cells ≥10.1% (HR: 1.044; 95% CI: 1.003 to 1.087; p=0.03) and high risk International Prognostic Score (≥3 points) (HR: 6.453; 95% CI: 1.970 to 21.134; p=0.002). CONCLUSIONS: The PD-L1/CD30 ratio identifies a group of cHL patients with an increased risk of treatment failure. Its clinical application can be performed as it constitutes an easy to implement pathological information in the diagnostic work-up of patients with cHL.

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Purpose: Patients with hematological malignancies are at an increased risk of severe infection with coronavirus disease 2019 (COVID-19). However, developing an adequate immune response after vaccination is difficult, especially in patients with lymphoid neoplasms. Since the long-term effects of the BNT162b2 vaccine are unclear, the humoral immune response 5 months after the two vaccinations in patients with hematological disorders was analyzed. Materials and Methods: Samples were collected from 96 patients vaccinated twice with BNT162b2 and treated with at least one line of an antitumor or immunosuppressive drug in our hospital from November 2021 to February 2022. Serum anti-severe acute respiratory syndrome coronavirus 2 (anti-SARS-CoV-2) spike (S) antibody titers were analyzed. Patients were age- and sex-matched using propensity matching and compared with a healthy control group. Patients with serum anti-SARS-CoV-2 S antibodies were defined as 'responder' if >50 U/mL. The patients had B-cell non-Hodgkin lymphoma (B-NHL), multiple myeloma, chronic myeloid leukemia, etc. Results: Patients had significantly low antibody levels (median, 55.3 U/mL vs. 809.8 U/mL; p<0.001) and a significantly low response rate (p<0.001). Multivariate analysis showed that patients with B-NHL, aged >72 years, were associated with a low response to vaccination. There were no significant differences between patients with chronic myeloid leukemia and healthy controls. Conclusion: Our study shows that patients with hematological disorders are at risk of developing severe COVID-19 infections because of low responsiveness to vaccination. Moreover, the rate of antibody positivity differed between the disease groups. Further studies are warranted to determine an appropriate preventive method for these patients, especially those with B-NHL.

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The classification of haematological neoplasms recently underwent revision, generating two separate schemes-the International Consensus Classification and the fifth edition of the WHO classification. The new division into separate classification systems presents challenges for haematopathologists, haematologists/oncologists and patients. While it is too early to assess the full clinical impact, we sought to identify diagnostic discordance which may arise from applying separate classification schemes in myeloid neoplasia, and particularly in the challenging category of myelodysplastic syndrome/myeloproliferative neoplasms. A review of 64 such cases found 1 case with a significant discrepancy between the WHO and International Consensus Classification systems, and 9 cases with nominal discrepancies. Confusion from the use of conflicting diagnostic terms represents a potential source of patient harm, increased pathologist workload and burnout and erosion of clinician and patient trust.

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