Hematology Clinic Research Articles

Effects of extracorporeal photopheresis on survival in chronic graft versus host disease

ABSTRACT Objective: Chronic graft versus host disease (cGVHD) develops after allogeneic hematopoietic cell transplantation, when immune cells from a non-identical donor initiate an immune reaction against the transplant recipient. Extracorporeal photopheresis (ECP) can be used in combination with prednisone in steroid-resistant cGVHD. In this study, the effect of ECP use on survival in cGVHD was examined. Patients and Methods: Twenty-six patients who were followed up in the adult Hematology Clinic of Inonu University Turgut Ozal Medical Center for cGVHD were included in the study. Stem cell transplantation and ECP application parameters that may affect the survival of the patients were examined. Results: The degree of involvement in cGVHD affects survival. Involvements with clinical and laboratory scores of 2 and above according to the National Institutes of Health consensus criteria, significantly reduced survival. The development time of cGVHD was found to be associated with survival, and that it had a positive impact on survival, especially when the disease developed after 220 days after the transplantation. It was observed that steroid dose taken during ECP, patient age and cGVHD prophylaxis used affected survival. Conclusion: The use of ECP may be effective in survival, especially, in patients who develop cGVHD, 220 days after allogeneic transplantation. Concurrent use of steroids with ECP affects survival.

A Novel Discriminating Tool for Microcytic Anemia in Childhood.

Accurate and timely interpretation of microcytic anemia can be diagnostically challenging in the primary care setting. We sought to develop a novel model for distinguishing iron-deficiency anemia from thalassemia trait in the modern pediatric population. Demographic history and red blood cell indices were retrospectively characterized for 76 children referred to our pediatric hematology clinic for evaluation of microcytic anemia. Statistically significant variables were sequentially added into a logistic regression model to develop the final model. The final discriminating model incorporates red cell distribution width, mean corpuscular hemoglobin concentration, and red blood cell values. Favorable predictive performance is seen in the initial (sensitivity 89.2%, specificity 92.3%) and external validation cohort (sensitivity 84.4%, specificity 88.9%). This novel tool may aid in determining the cause of hypochromic, microcytic anemia in the primary care setting. Finally, the study cohort reflects an underrepresented group in the development of screening tools, and thus offers generalizability.

Neurological abnormalities among pediatric patients with sickle cell disease in Saudi Arabia: a single-center retrospective study.

Sickle cell disease (SCD) is a common inherited blood disorder characterized by the production of abnormal sickle-shaped red blood cells. SCD can lead to various complications including neurological issues. Early detection and treatment are crucial for preventing these complications. This study aimed to describe the neurological manifestations, radiological findings, and neurological diagnosis related to SCD in Saudi children with the aim of contributing to the formulation of population-based guidelines for screening and treating SCD-related neurological complications. This descriptive retrospective study included pediatric patients aged < 14 years diagnosed with SCD who were regularly followed up at the hematology clinic in KAMC, Jeddah, Saudi Arabia, from January 2008 to January 2022. Demographic and clinical data were collected from the clinical charts of 101 participants. This study included 101 patients with SCD with a mean age of 23 months at diagnosis. Among these, 59% had SCD and high fetal hemoglobin (HbF) levels. Neurological sequelae, including seizures, stroke, and other abnormalities, were observed in 26.7% of patients. There were no significant differences in the onset of neurological issues between the patients with SCD-high HbF and those with other SCD phenotypes. This study highlights the increased risk of brain injury and neurocognitive deficits in children with SCD. The occurrence of neurological sequelae in many patients emphasizes the need for early detection and intervention. Some patients experience neurological complications despite having high HbF levels, suggesting that further interventions are needed. This study has some limitations, including its small sample size and retrospective nature. Early detection and intervention are crucial for neurological complications in patients with SCD. This study emphasizes the need for further research and effective treatment strategies considering the presence of neurological complications despite the presence of high HbF levels. Large-scale studies and population-specific guidelines are warranted for better understanding and management of SCD-related neurological complications in the Saudi population.

Sleep experiences of parents of children 18 years or younger with sickle cell disease during the COVID-19 pandemic: a qualitative study.

The coronavirus disease pandemic has highlighted disparities in health care. Parents assist in sickle cell disease management in children. Understanding sleep in parents of children who are diagnosed with sickle cell disease is an important facet of disease management. Our objectives were to identify sleep characteristics of parents of children with sickle cell disease age 18 years or younger during the coronavirus disease pandemic, to investigate measures used by parents to achieve restful sleep, and to discuss how sleep may be improved in parents of children diagnosed with sickle cell disease. This study conducted 14 face-to-face semistructured interviews with parents of children diagnosed with sickle cell disease. A majority of the interviews (93%) were conducted at a hematology clinic. Qualitative thematic analysis was used to analyze data. All parents reported that their child experienced pain because of sickle cell disease. Most parents (86.7%) reported that their child was diagnosed with sickle cell anemia. Four themes were identified: difficulty obtaining restful sleep during their child's sickle cell crisis, sleep during the coronavirus disease pandemic, factors affecting sleep, and effects of sleep disturbance and deprivation on parental performance. This research has highlighted some of the difficulties experienced by parents of children who are diagnosed with sickle cell disease and allows for additional insight into the sleep experiences of parents as they attempt to manage their child's disease. Osborne JC, Odlum M, Sedrak A. Sleep experiences of parents of children 18 years or younger with sickle cell disease during the COVID-19 pandemic: a qualitative study. J Clin Sleep Med. 2024;20(5):777-782.

Evaluation the in vitro Anti-Sickle Cell Potential of Two Medicinal Plant Species, Rhynchospora corymbosa and Olax subscorpioïdea on the Inhibition of Falciformation of SS Genotype Erythrocytes

Aims: Sickle cell disease is one of the world's leading genetic disorders. It is characterized by the presence of an abnormal hemoglobin HbS in the blood. The anomaly affects the primary structure of hemoglobin, through the substitution of valine for glutamic acid. In traditional medicine, two plant species, Rhynchospora corymbosa and Olax subscorpioïdea, are used to treat this condition.&#x0D; Place and Duration of Study: Agro-valuation Laboratory, Hematology Clinic laboratory of Cocody Hospital University (CHU), between June-Aout 2021 and June-July 2022.&#x0D; Methodology: Following a triphytochemical test, the effect of each species on the in vitro inhibition of SS erythrocyte falciformation was evaluated.&#x0D; Results: Thus an abundance of alkaloids, tannins, saponins, polyterpenes and sterols were found in the families of molecules identified in Rhynchospora corymbosa. As for the decocted Olax subscorpioïdea, in addition to the families of molecules identified in Rhynchospora corymbosa the polyphenols, leuco-anthocyanins and flavonoids were found. In addition, at an equal concentration of 0.312 mg/mL, decoctions of Rhynchospora corymbosa and Olax subscorpioïdea gave an inhibition rates of 80 and 84 % respectively. Both extracts showed activity on in vitro falciformation inhibition. This activity is thought to be due to the presence of chemical groups with anti-oxidant and anti-inflammatory properties.&#x0D; Conclusion: The use of Rhynchospora corymbosa and Olax subscorpioïdea in the treatment of sickle cell disease in Côte d'Ivoire would be justified.

Chronic neutropenia in childhood: experience of a tertiary center

ObjectiveTo assess the prevalence of chronic neutropenia (CN) and the clinical profile of patients with CN aged up to 18 years, followed in the pediatric hematology, rheumatology, or immunology outpatient clinic of a tertiary medical center from May 1, 2018, to 30 April 2019. MethodsRetrospective observational study carried out by collecting data from the patient's medical charts. CN was defined as absolute neutrophil count (ANC) below 1.5 × 109/L lasting over three months. Autoimmune neutropenia (AIN) was defined by clinical criteria and an over twofold increase in ANC after glucocorticoid stimulation. AIN was considered secondary when associated with autoimmune or immunoregulatory disorders. Wilcoxon and Fisher's exact tests were used to compare variables; the significance level was 5 %. ResultsA total of 1,039 patients were evaluated; 217 (20 %) presented CN. Twenty-one (2 %) had AIN, classified as primary in 57 % of the cases. The average age at the onset of symptoms was 38.6 months. During follow-up, patients had 4.2 infections on average; frequency was higher among patients with secondary AIN (p = 003). Isolated neutropenia occurred in 43 % of the patients with AIN. Neutropenia resolved in eight (38 %) of the 21 patients with AIN within 19.6 months on average. Eight patients with secondary AIN met the criteria for Inborn Errors of Immunity. ConclusionAIN prevalence was 2 %. Most cases were first evaluated by a pediatric immunologist or rheumatologist rather than a pediatric hematologist. This study highlights the need for a multidisciplinary approach involving a pediatric immunologist, rheumatologist, and hematologist.

Primary Central Nervous System Lymphoma, Treatment Outcomes - 10 Year Experience. Single Center Study.

Primary central nervous system lymphoma(PCNSL) is an aggressive, rare form of Non-Hodgkin lymphoma, characterized by the absence of systemic disease. There are limited data and no strictly defined guidelines for management of PCNSL. The aim of this study was to report a 10 year experience of PCNSL treatment, to evaluate treatment outcomes and asses Progression Free and Overall Survival of these patients. Study was conducted on the Haematology Clinic, Clinical center University of Sarajevo, BH, in the period from January 2012.-December 2022. Total sample of 24 patients were enrolled. All have undergone diagnostic surgery. Patients were treated with regimens based on High dose Methotrexate, with/without whole brain radiotherapy as consolidation. Treatment response was captured by imaging techniques. Patients who have relapsed were evaluated with imaging techniques and treated according to Methotrexate-based treatment protocols. We have captured equal gender distribution. The median age of patients was 59.5 years (range 20-79). Pathohistological analysis confirmed DLBCL diagnosis in 22 patients, T cell lymphoma and anaplastic large cell lymphoma, each in 1 patient. Chemotherapy, chemotherapy combined with WBRT and radiotherapy were given to 5, 18 and 1 patients, respectively. The overall complete response rate (CR) was 87,15%. Those receiving combined modality-treatment had higher CR than those receiving chemotherapy (94,4% versus 60%). Out of 24 patients, 11 of them relapsed. The median time to relapse was 29 months (from 1 to 105). After second line of the treatment, CR was 54,5%, while 45,45% of patients died during the treatment. 4 patients relapsed for the second time with median time to relapse of 9 months (from 2 to 77). 2 year OS rate was 67%, and the median OS rate was 45,9 months. 2 year PFS rate was 31%. The OS and PFS rates indicate the usage of new drugs and consolidation with autologous stem cell transplantation in patients with PCNSL in order to achieve better treatment outcomes.

Association Between Clinical, Laboratory, and Ultrasound Characteristics and Cancer Incidence of Peripheral Lymphadenopathy in Children

Objective: Peripheral lymphadenopathy is very common in childhood. Although they are usually benign, they are often seen in pediatric outpatient clinics because they are a major cause of concern for families. However, physicians cannot afford to overlook the possibility of cancer, which is why many of these patients are referred to pediatric hematology and oncology outpatient clinics. In this study, we calculated the proportion of patients requiring biopsy and the frequency of malignancy, and investigated the characteristics that may be associated with malignancy.

Ocular surface expression of inflammatory cytokines in GvHD and their association with disease development and clinical manifestations

The aim of this study was to investigate the tear cytokine profiles of patients with chronic ocular graft versus host disease (GvHD) and evaluate their association with clinical disease severity. Consecutive patients attending the Haematology clinic with a known or suspected diagnosis of chronic ocular GvHD were invited to participate. To be included, patients had to fulfil either or both of the National Institute of Health (NIH) or the International Chronic Ocular Graft‐vs‐Host‐Disease Consensus Group (ICCGvHD) diagnostic criteria.Clinical parameters recorded included best correct visual acuity (BCVA), Schirmers I test, tear break up time (TBUT), and Oxford surface staining (OSS) along with the overall grading of ocular GvHD severity as defined by the ICCGvHD. Ocular tear samples were collected and levels of cytokine production (IFN‐γ, IL‐1β, IL‐2, Il‐4, IL‐6, IL‐8, IL‐10, IL‐12p70, IL‐13, and TNF‐ α) were assayed using Meso Scale Discovery (MSD) multi‐array technology. The results were compared to those of healthy matched controls using unpaired t test. Results were correlated with clinical parameters using Pearson r correlation.Thirteen patients took part in the study (8 male, 61.5%; 5 female, 38.5%) with an average age of 43.9 years (±13.4). With regards to ocular examinations, patients were found to be heavily dependent on lubricant eye drops (average 8.5 times/day). Patients suffered from both aqueous deficient and evaporative DED with an average Schirmer's measurement of 4.2 mm (±5.7, range of 0–21) and average TBUT of 3.8 s (±1.3, range 1–6). The average Oxford surface staining score was 7.9 (±4.2, range of 0–15). According to the ICCGvHD grading system, and using the average data across both eyes, seven of the patients had ‘Mild/Moderate’ disease (53.8%) and the remainder had ‘Severe’ disease (n = 5, 38.5%).The levels of IFN‐γ, IL‐1β, IL‐2, IL‐6, IL‐8, IL‐10, IL‐12p70, IL‐13, and TNF‐ α were found to be statistically elevated when compared to healthy age and gender matched controls (FDR p value&lt;0.05). In those with early‐stage disease (n = 5), IFN‐γ was found to strongly correlate with lubricant usage (0.98, p = 0.03), and IL‐2 with the severity of ocular surface staining grade (1.00, p = 0.03), implying that these cytokines in particular may play a role in the development of chronic ocular GvHD and represent promising treatment targets for patients in the initial stages of disease.This study demonstrates that ocular tear cytokines are elevated in patients with chronic ocular GvHD, reiterating the role of inflammation in the pathogenesis of disease. Furthermore, the correlation of IFN‐γ and IL‐2 with clinical parameters of disease severity suggest that these cytokines play a role in early disease development. These findings support the implementation of anti‐inflammatory treatment such as topical calcineurin inhibitors, in conjunction with tear replacement and preservation to avoid irreparable damage to the ocular surface, and this may be best utilized in the initial stages of the disease development.

Prevalence and predictors of pulmonary hypertension in patients with myeloproliferative neoplasms: single-center study

Background Pulmonary hypertension is a major complication of several hematologic disorders including myeloproliferative neoplasms (MPNs). Despite the potential significance of PH to the management and prognosis of MPN, accurate estimates of its prevalence in MPNs and risk factors for the condition are poorly established. Aim To determine the prevalence of pulmonary hypertension in patients with MPNs at Sohag University Hospital and to analyze data of those patients to find predictors of pulmonary hypertension in our patients. Patients and methods A total of 120 patients were diagnosed with MPNs according to the revised 2016 WHO diagnostic criteria who attended the Outpatient Hematology Clinic at Sohag University Hospital in the period from April 2021 to October 2022. Systolic pulmonary artery pressure (SPAP) by using Transthoracic echocardiography (TTE) is determined in all MPNs patients enrolled in our study. Results The overall prevalence of PH in MPN patients was found to be 26.7%. We found a significant relationship between old age, long MPN duration since diagnosis, presence of PMF, anemia, leukocytosis, hyperuricemia, and occurrence of PH. Conclusion The prevalence of PH in patients with MPNs was 26.7%. According to MPNs subtypes, the highest PH prevalence was in PMF patients. Our findings suggest that old age, long MPNs duration since diagnosis, presence of PMF, and hyperuricemia are valuable for predicting PH occurrence in patients with MPNs.

Classical Hematology Referrals at an National Cancer Institute-Designated Cancer Center: Lessons Learned.

To understand the spectrum and volume of classical hematology (CH) referrals to hematology clinics at a National Cancer Institute (NCI)-designated cancer center (CC) to plan for the delivery of effective and equitable care for this population. One referral office at the Academic CC located in Bexar County, TX, handles all adult hematology referrals. From October 1, 2021, to September 30, 2022, all nonmalignant hematology (MH) referrals were triaged daily to define the category of CH problem. Declined referrals (confirmed at triage that no CH problem was evident) are included as part of this analysis. Electronic consultation (opinion rendered without patient seen) at our CC is available and is not part of this analysis. One thousand nine hundred forty-five CH referrals were received in the 12-month period. Seventy-six referrals (3.9%) were declined. During the study period, there were 2,289 medical oncology referrals and 779 referrals for MH. CH referrals therefore comprise 39% of all hematology-oncology referrals and 71% of all hematology referrals at the CC. Anemia and thrombotic disorders were the most common categories of the accepted CH referrals at 487 (26%) and 393 (21%), respectively. Video visits were used for 447 of all CH referrals (23%), and the rest were in person. Nearly 40% of all referrals to hematology and medical oncology at our NCI-designated CC are for CH. Effective management of the CH population of patients will allow ideal care for CH problems and also allow cancer-focused care to improve.

Updated threshold, renewed problems: should the diagnostic criteria of polycythemia vera be reconsidered? A retrospective cross-sectional cohort study.

This aim of this study was to evaluate hemoglobin and hematocrit values of polycythemia vera and secondary polycythemia patients with updated World Health Organization thresholds. In addition, by determining our own threshold values, we aimed to demonstrate the necessity of bone marrow biopsy and genetic analysis to be used for further diagnosis in patients with high-normal hematocrit and hemoglobin values. A cross-sectional and retrospective study was performed with the medical records of patients from Eskisehir City Hospital hematology clinics and outpatient clinics between July 1, 2019 and July 1, 2020. The study included patients with polycythemia, divided into two groups according to polycythemia vera and secondary polycythemia. A bone marrow biopsy was performed on patients with either Janus kinase mutation positivity and/or subnormal erythropoietin levels. Receiver operating characteristics analysis was used to find threshold values, and the diagnostic efficiency of these values in differentiating World Health Organization thresholds in 2008 and 2016 was evaluated. A total of 73 patients were included. The median age was 43.5 years (min: 18; max: 84). The hematocrit value of 54.1 was predicted to diagnose polycythemia vera with a sensitivity of 45% and a specificity of 80%. Subsequent analysis revealed that an hemoglobin value of 17.7 was indicative of diagnosing polycythemia vera with a sensitivity of 60% and a specificity of 63%. The mean follow-up length was 6.4 months (2-12). Our study demonstrated that modified World Health Organization criteria might lead to unnecessary additional tests for polycythemia vera patients with high-normal hemoglobin and hematocrit values.

Depressive Symptoms among Children with Sickle Cell Anemia in a Tertiary Health Facility in North-western Nigeria

Abstract Context: Sickle cell anemia (SCA) being a chronic debilitating condition places affected children at risk of depression. Aims: The aim of this study is to determine the prevalence of depressive symptoms in children with SCA, the perceived causes, intervention sought, and associated factors. Settings and Design: This was a cross-sectional survey involving children with SCA aged 7–15 years attending pediatric hematology clinic of Usmanu Danfodiyo University Teaching Hospital, Sokoto. Materials and Methods: Information on depressive symptoms was obtained using the short mood and feelings questionnaire for children and adolescents. Statistical Analysis Used: Data were analyzed using IBM-SPSS version 25.0. Results: Respondents consisted of 291 child–caregiver pair with M:F of the children 1.6:1 and mean age of 11.0 ± 2.7 years. Majority [239 (82.1%)] of the caregivers were females with mean age 38.4 ± 7.4 years. Most [217 (74.6%)] of the caregivers were Hausa by tribe, married (269, 92.5%), in a monogamous setting, 159 (58.5%) with 129 (44.3%) of them having low socioeconomic status. One hundred and forty-nine (51.2%) children had depressive symptoms. Requirement for daily drug intake (125, 83.9%), missed school days (119, 79.9%), recurrent illness (115, 77.2%), and stress (114, 76.5%) were the commonest perceived causes. Prayers (138, 94.5%) and use of distraction (100, 68.5%) were the commonest interventions adopted. Child age category (P = 0.002), caregiver’s gender (P &lt; 0.001), family setting (P &lt; 0.001), socioeconomic status (P = 0.008), comorbidity (P = 0.021), frequent painful crises (P = 0.001), and frequent hospital admission (P = 0.024) were associated with depressive symptoms and remained independent predictors of depressive symptoms on logistic regression. Conclusions: Depressive symptoms were common among the subjects and associated with some sociodemographic and medical factors.

Diagnosis and management of systemic mastocytosis in a community hematology setting.

Systemic mastocytosis (SM) is a rare and potentially severe hematologic disorder characterized by the clonal proliferation of mast cells (MCs) into various organs. The clinical manifestations of advanced SM are caused by the uncontrolled release of cytokines and vasoactive amines from MC and disease-induced organ dysfunction. Patients with SM typically present with symptoms such as flushing, pruritus, diarrhea, and headaches, but outcomes following active treatment have not been well characterized. In this study, the clinical characteristics, treatment patterns, and natural history of an SM patient cohort diagnosed and treated within a community hematology network in the United States is described. We identified 105 patients who were diagnosed and managed in one of 19 community hematology clinics up to an index date of 1 October 2022. Data collection included patient and disease characteristics, baseline biochemistry and hematology, SM diagnostic criteria being met, biomarkers tested, CD2 and/or CD25 expression in MCs as well as serum tryptase levels at presentation. Data abstraction also included supportive care drugs and anticancer therapy used, treatment response, reason for discontinuation, and overall survival by disease subtype. A total of 105 SM patients were identified who met the inclusion criteria. The specific SM subtypes were indolent (47.6%), aggressive (9.5%), SM with an associated hematological neoplasm (19.0%), MC leukemia (1.9%), and subtype not documented (21.9%). Regardless of subtype, approximately 62% of patients did not receive SM-directed active therapy. Only 26% of patients with indolent systemic mastocytosis (ISM) received treatment compared to 65.6% with advanced subtypes. Relative to ISM cohort, the relative risk of death in patients with the advanced SM subtypes was approximately 15 times greater (hazard ratio = 15.0; 95% confidence interval: 3.3 to 66.5). SM patients present with multiple underlying symptoms, within various disease subtypes that are difficult to diagnose in a timely manner. As a result, many patients do not receive active drug therapy for their disease. Therefore, greater disease awareness is required as well as new tools for earlier disease detection.

Effects of the re-mission video game on fatigue and quality of life levels of adolescents diagnosed with cancer: a randomized controlled trial

ABSTRACT The aim of this study was to investigate the effects of the Re-Mission video game on the fatigue and quality of life of adolescents diagnosed with cancer. A total of 46 adolescents aged who received inpatient chemotherapy treatment in the pediatric hematology clinic were enrolled in the study. The data were obtained by using the Personal Information Form, Scale for the Assessment of Fatigue in Pediatric Oncology Patients Aged 13–18 and Quality of Life scale. Intergroup comparisons demonstrated that there was a significant difference between the experimental and control groups in terms of their mean scores for the overall Scale for the Assessment of Fatigue and Quality of Life calculated at the baseline of the study and one month and three months. The use of Re-mission video game positively affected levels of fatigue and quality of life of adolescents aged between 13 and 18 diagnosed with cancer.

Knowledge, attitude, and practice of virtual consultation among outpatients at a teaching hospital in Malaysia.

During the coronavirus disease 2019 (COVID-19) pandemic, the use of virtual consultations has accelerated to ensure continued access to healthcare despite lockdowns and physical distancing measures. We aimed to determine the knowledge (awareness) of, attitude (acceptability) to, and practice (exposure) [KAP] of virtual consultations (VC), the demographic factors associated with poor KAP, and the correlation between the three KAP domains. A cross-sectional study, using a convenience sampling technique, was conducted from 13 September, 2021 to 28 November, 2021. We designed a 45-item VC KAP questionnaire. This was distributed to outpatient users attending cardiovascular, dermatology, geriatrics, haematology, endocrine, respiratory, gastroenterology, rheumatology, or neurology clinics at the University Malaya Medical Centre. It was completed during face-to-face, online, or telephone interviews. The data were analysed using SPSS version 24.0. Binary logistic regression was used to determine the demographic factors associated with KAP. Correlation between KAP domains was determined using Spearman's rho (r). A p-value of <0.05 was considered statistically significant. A total of 366 questionnaires were completed. Knowledge (awareness), attitude (acceptability), and practice (exposure) were considered good in 69.7%, 80.9%, and 24.6% of participants, respectively. There were no significant relationships between age, gender, ethnicity, and duration of hospital attendance (years) with knowledge (awareness), attitude (acceptability), and practice (exposure). A moderate positive correlation was seen between knowledge (awareness) and attitude (acceptability) (Attitude total [Atotal]) (r = 0.48, p<0.001), with no significant correlation between knowledge (awareness) and practice (exposure) (r = 0.04, p = 0.45), and attitude (acceptability) (Atotal) and practice (r = 0.01, p = 0.82). Overall, outpatient clinic users had good knowledge (awareness) of and were receptive towards VC but had poor practice (exposure). More opportunities for VC use in healthcare can increase exposure and subsequent utilisation. Interventions to increase the effectiveness of VC use should be explored in future studies.

Plasmablastic Lymphoma: The 'Rare' Frequency Among Immunocompromised Patients

Limfoproliferations associated with HIV infection/AIDS are rare conditions, with an incidence of malignant lymphomas of approximately 10% among the 25-40% malignancies that can occur in these immunosuppressed patients (1). Plasmablastic lymphoma (PBL), with an incidence of only 2% (out of the total lymphomas), is a borderline form (morphologically and immunophenotypically) between lymphoma and myeloma, defined by its high aggressiveness and unfavorable prognosis (2). Objectives: identifying the clinical, histopathological, and biological characteristics of PBL that require a differential diagnosis within other malignant hematological conditions associated with HIV infection. Materials and Methods: We present three comparative cases of PBL that were observed at the Colțea Hematology Clinic in a short time window (4 months). All patients were diagnosed with PBL in the context of HIV/AIDS infection, with an average age of approximately 41 years, predominantly male (66.6%), and with a symptomatology onset characteristic of immunocompromised individuals (manifesting at the oral, lymph node, and cutaneous levels), but with specific aspects related to associated comorbidities (e.g., psychiatric pathology, dilated cardiomyopathy), risk behaviors (e.g., intravenous drug use for 20 years), and infectious/ non-infectious complications, which influenced the patients' evolution. Conclusions: Plasmablastic lymphoma still represents a highly aggressive condition with an unfavorable prognosis, possibly having a higher clinical incidence than that described in the literature and requiring a adequate multidisciplinary approach.

Renal outcomes in pediatric patients with sickle cell disease: a single center experience in Saudi Arabia.

Sickle cell nephropathy (SCN) is a significant complication of sickle cell disease (SCD) with an asymptomatic onset in childhood and potential progression to chronic kidney disease (CKD). The clinical findings of SCN include hyposthenuria, hematuria, proteinuria, hyperfiltration, and CKD. Data on renal manifestation among patients with SCD in Saudi Arabia is lacking. Therefore, this study aimed to evaluate renal outcomes in patients with SCD who visited a hematology clinic at the National Guard Hospital, Jeddah. We conducted a retrospective chart review of renal complications in patients with SCD who are within 0-14 years of age and on regular follow-ups at the pediatric hematology clinic in King Abdulaziz Medical City-Jeddah, Saudi Arabia. Among the 140 patients with SCD, 99 met the inclusion criteria. The median age at diagnosis was 18 (1-108) months. Two SCD phenotypes were observed, with 82 (83%) patients having sickle cell anemia (HbSS) and 17 (17%) having HbS/B+ thalassemia. Of the total patients, 92 (93%) were administered hydroxyurea (HU), with a median starting age of 48 (9-168) months. The most common renal complication observed during routine urinalysis was hematuria (38%), followed by proteinuria (11%). After stratifying the sample into four age groups (0-3 years old, 4-7 years old, 8-11 years old, and 12-14 years old), the mean glomerular filtration rate (GFR) values were 96.16, 101.36, 112.69, and 120.11 ml/min/1.73 m2 respectively. Renal imaging revealed abnormal findings in 27 (29%) patients. The most common abnormality observed on US was increased echogenicity (43%). SCN is a significant complication of SCD. In this study, we assessed the renal outcomes in pediatric patients with SCD. After analyzing the clinical findings of SCN, we concluded that the presence of renal complications in pediatric patients presented a progressive pattern.

Evaluation of the frequency of hepatitis b virus reactivation and the importance of hepatitis b prophylaxis in hematology patients receiving immunosuppressive therapy: a single-center study

Aims: The aim of this study was to evaluate the rate of hepatitis B virus reactivation (HBVr) in hematology patients receiving immunosuppressive therapy in our center and the clinical characteristics of patients with HBVr. We will also investigate the importance of effective prevention of this potentially life-threatening event and management of hepatitis B virus (HBV) prophylaxis. Methods: In this study, hepatitis B prophylaxis and its effects on patients over 18 years of age receiving immunosuppressive therapy in the hematology clinic were analyzed. 122 patients were included in the study. The HBV markers of the patients were determined by the chemiluminescence method. In the study, HbsAg(+) and isolated antiHbc IgG-positive patients received prophylactic antiviral treatment. The differential diagnosis of HBV reactivation and the criteria determined to define HBV reactivation were performed. Clinical characteristics and descriptive information of patients receiving HBV prophylaxis were analyzed using SPSS 25.0. Results: The median age of 122 patients (59.8% male) was 58 years. It was determined that five HbsAg-positive patients had no prior follow-up and did not receive antiviral treatment. 117 patients had isolated anti-HBc IgG positivity. The median duration of prophylaxis was 15 (9- 21.25) months, and the total follow-up period was 19.5 (11.75- 30.25) months. 81.1% of the patients received regular HBV prophylaxis treatment; 59% of them received entecavir, and the rest received tenofovir disoproxil. Bone marrow transplantation was performed in 25 patients. HBV reactivation was detected in only 4 patients (3.3%); one of these patients had received allogeneic and one autologous bone marrow transplantation; and three patients had received chemoimmunotherapy including Rituximab. The diagnoses of the patients with HBVr were acute myeloid leukemia, lymphoma, and chronic lymphocytic leukemia. During the follow-up period, 29 patients (23.8%) died due to their primary disease, but there were no deaths due to HBV reactivation. Conclusion: The data obtained in this study show that effective hepatitis B prophylaxis treatment is successful in preventing HBV reactivation in hematology patients. HBVr was observed in four patients who did not take HBV prophylaxis medication regularly.

The relationship between immunologic system and ferritin levels in patients with transfusion dependent thalassemia: a retrospective single-center study

Aims: Thalassemia is an inherited anemia characterized by reduced or absent expression of ?- or ß-globulin genes (1). Transfusion dependent thalassemia (TDT) has an increased risk for serious infections, suggesting that a fundamental defect Methods: The study included 61 patients with TDT between the ages of 18 and 45 who were followed up in the hematology clinic of our tertiary care center between 2018 and 2023. For the determination of mononuclear cell surface markers, peripheral blood was processed according to standard procedures, labeled with monoclonal antibodies specific for CD4, CD8, CD3, CD19, and CD56, and analyzed by cytofluorometric analysis with a FACScan flow cytometer (Sysmex UF 1600). Results: In our study, CD4, CD8, CD3, CD19, and CD56 values, which are lymphocyte markers, were examined to evaluate the immune system of patients diagnosed with TDT, and it was observed that there was no significant correlation between these values and the annual infection frequency and mean ferritin values of the patients. Conclusion : The mean age of the 61 patients included in the study was 24.83±6.02 years, and 33 were female and 28 were male. The mean ferritin value was 3150.88±2553.51 ng/ml, and transferrin saturation was 42.42±14.43. The mean number of transfusions per year was 15.39±1.90, and the mean number of detectable infections per year was 2.85±2.02.