Hematology Outpatient Clinic Research Articles

Pelger-Huët Anomaly (PHA) Case Report

Pelger-Huët anomaly is a rare benign hematological disorder. It was first described in the 1920s. It is inherited in an autosomal dominant manner. Nuclear hypolobulation, which can also be seen in other leukocytes, especially neutrophils, is a characteristic feature. This morphological change does not cause a defect in the immune system. It should be differentiated clinically from pseudo-Pelger-Huët anomaly (PPHA). In this study, Pelger-Huët anomaly is presented which is noticed in the peripheral smear of a 22-year-old female patient who came to the hematology outpatient clinic due to her sibling having Hodgkin’s lymphoma. The family members of the case were also evaluated in this respect.

Rosai Dorfman Disease: A Rare Disease Case Report

Rosai Dorfman Disease (RDD) is a rare benign condition. It was first described by Rosai and Dorfman in the 1960s. The etiology, pathogenesis and true frequency of the disease remain unknown. The disease is characterized by the proliferation of histiocytes in the lymph nodes. Clinically, this enlargement of the lymph nodes must be distinguished from other infectious or malignant causes. Although it is often observed at early ages, in this study, a case of RDD detected in a 66-year-old male patient who came to the hematology outpatient clinic due to axillary swelling was presented. The patient is still being monitored without any symptoms,without treatment.

The effects of spiritual wellbeing on life satisfaction in hematologic cancer patients aged 65 and older in Turkey: mediating role of hope.

The mediating role of hope in the effects of spiritual wellbeing on life satisfaction in elderly haematologic cancer patients in Turkey was investigated in the present study. The study was conducted in a descriptive, cross-sectional and correlational design. The study was conducted with 150 patients aged 65 and older who were diagnosed with haematologic cancer and who were referred to a university hospital haematology clinic and outpatient clinic. Research data were collected with Descriptive Information Form, Dispositional Hope Scale (DHS), Spiritual Well-being Scale (FACIT-Sp-12) and Satisfaction with Life Scale (SWLS). FACIT-Sp-12 score was 37.25 ± 7.29; DHS score was 40.42 ± 8.29, SWLS score was 16.24 ± 8.79. FACIT-Sp-12 (β = 0.668) and DHS (β = 0.226) were found to affect SWLS positively. In terms of the effect of FACIT-Sp-12 on SWLS, DHS has a mediating role and makes the positive effect of FACIT-Sp-12 on SWLS stronger (β = 0.771). Spiritual wellbeing levels of the participants in our study were found to be high, while their levels of satisfaction with life and hope were found to be moderate. It was also concluded that spiritual wellbeing had a direct effect on satisfaction with life and an indirect effect through the mediating role of hope.

Understanding the physical literacy development of 8- to 12-year-old children living with chronic medical conditions: A comprehensive, mixed methods inquiry.

Physical literacy is a concept used to describe the combined physical, affective and cognitive capacities facilitating an active lifestyle. Physical activity participation is essential for children living with chronic medical conditions, but knowledge of physical literacy among this group is scarce. An explanatory, sequential mixed methods design was used to comprehensively describe the physical literacies of children with chronic medical conditions (CMCs). Participants were recruited from paediatric cardiology, respirology/cystic fibrosis, neurology, haematology and endocrinology outpatient clinics. All participants completed the Canadian Assessment of Physical Literacy (2nd Edition), and those with higher and lower scores were invited to a semi-structured interview. A deductive-inductive thematic analysis was applied using Margaret Whitehead's conceptualization of physical literacy. Using normative strata, 80.0% of the 99 children assessed (mean age = 9.97 ± 1.3years, 48% girls) were considered beginning or progressing in their overall physical literacy (mean score = 56.5 ± 13.8/100). Meanwhile, physical literacy informed participants' approach to new, active experiences and may have contributed to a strong sense of self. There was a significant difference between endocrinology and haematology patients on total physical literacy score (p = 0.03) but not domain scores. Participants scored high on motivation/confidence (mean = 22.9 ± 5.0/30) but obtained low physical competence (mean = 11.8 ± 5.6/30) and daily behaviour scores (n = 72, mean = 15.5 ± 7.1/30). Main themes represent salient experiences of children with CMCs within the domains of physical literacy, including their need to evaluate active contexts, self-regulate activity intensity and manage physical limitations. Children with CMCs can achieve recommended levels of physical literacy without meeting normative standards for physical competence. Participants would benefit from a physical literacy intervention that targets the development of bodily self-regulation skills and risk evaluation in active settings.

Causes of acquired isolated neutropenia in adulthood: a single center study

Aims: Neutropenia, associated with several hereditary and acquired causes, is detected in a significant portion of patients presenting to the hematology outpatient clinic today. We conducted this study since we failed to find any Turkish publication on acquired isolated neutropenia in adults. Methods: This retrospective study examined data belonging to patients aged 18 years and older who applied to the adult hematology outpatient clinic whose absolute neutrophil count (ANC) was below the normal reference values (? 1.5 x 109/L). Results: 103 adult patients, 93 women and 10 men, were included in the study. Our study detected the cause of acquired isolated neutropenia as chronic idiopathic neutropenia (CINA) in 39.8% (n=41) of patients, autoimmune diseases in 18.4% (n=19), drugs in 14.6% (n=15), folate deficiency in 13.6% (n=14), infection in 9.7% (n=10), and hematological malignancy in 2.9% (n=3) and cyclic neutropenia in 1% (n=1). 92 patients (89.3%) were found to display mild neutropenia while 9 (8.7%) displayed moderate neutropenia and 2 (2%) displayed severe neutropenia. Interestingly, folate deficiency was detected in 6 of 9 (66.7%) moderately neutropenic patients. Conclusion: In our study, CINA was determined as the most common cause of acquired isolated neutropenia in adults, while autoimmune neutropenia was the second most common. In addition, the detection of folate deficiency in 6 of 9 patients (66.7%) with moderate neutropenia and the fact that these patients were in the advanced age group [mean 68 (range 62-81)] was a major finding of the study.

Assessing Psychological Disorders in Turkish Adolescents with Transfusion-Dependent Thalassemia.

We investigated depression and anxiety levels and related psychological disorders in adolescents with transfusion-dependent thalassemia (TDT) in this study. The study was conducted in two pediatric hematology outpatient clinics and included adolescents with TDT (14.8 ± 2.4 years, n = 40) in the study and compared them with the healthy age-matched control group (14.3 ± 2.3 years, n = 62). The Turkish version of the Revised Child Anxiety and Depression Scale (RCADS) was used to determine depression, anxiety, and related psychologic disorders (obsession, panic disorder, social phobia). Depression, anxiety, obsession, panic disorder, and social phobia scores were significantly higher in the patient group compared with the control (all p < 0.05). Ferritin levels were positively correlated with total depression, general anxiety, separation anxiety, and social phobia scores, but transfusion frequency and young age were the confounding factors. Patients in early adolescence and those who require more frequent blood transfusions are at higher risk of developing psychological disorders; routine screening for mood disorders should be warranted. Serum ferritin level may be a good warning indicator for early recognition of psychologic disorders in TDT patients.

Children with bicytopenia and pancytopenia – clinical picture, etiological spectrum, and outcome

Background: Children who develop cytopenias present with several etiological causes, posing diagnosis, and management difficulties for both physicians and pathologists. With these in mind, we assessed the clinical profile and etiology of children who attended a healthcare facility in South India with bicytopenia or pancytopenia. Aims and Objectives: (1) The primary objective of this study was to find out the clinical and etiological spectrum in children with bicytopenia and pancytopenia who were admitted to the Institute of Child Health and Research Center, Government Rajaji Hospital, Madurai. (2) The secondary objective of this study is to follow-up children with bicytopenia and pancytopenia to find out the outcome and prognosis. Materials and Methods: This study was in the Department of Pediatrics at the Government Rajaji Hospital in Madurai, Madurai Medical College’s Institute of Child Health and Research Center, which is where we carried out the study. Two hundred and sixty-four patients with bicytopenia and 36 patients with pancytopenia were included in this study. Once in 2 weeks, all children with bicytopenia and pancytopenia were assessed in our Saturday hematology outpatient clinics. Results: Fever was the most prevalent sign of both pancytopenia and bicytopenia in this investigation. Etiological variables among cases of pancytopenia, cancer, infections, non-infections, and idiopathic causes were recorded in 11%, 58%, 17%, and 14% of cases, respectively. Similarly, bicytopenia, malignancy, infections, non-infections, and idiopathic causes were found in 23%, 70%, 4%, and 3% of cases, respectively. According to the prognosis, 28%, 33%, 6%, and 33% of cases with pancytopenia recovered, passed away, were lost to follow-up, or were undergoing treatment, respectively. Similar to those individuals, 67%, 11%, 4%, and 18% of bicytopenia cases were treated, recovered, or lost to follow-up, respectively. Conclusion: In children, there is a wide etiological range of cytopenias. Infections and dangerous diseases like acute leukemia are among the reasons. The geographical location of the hospital, the frequency of malnutrition, and the regional presence of certain diseases such as malaria and enteric fever determine the causes reported in a hospital environment.

The role of a Hematology outpatient clinic in improving access to bone marrow transplantation in low-middle-income countries.

Discutir a elegibilidade precocemente no Transplante de Medula Óssea (TMO) reduz o tempo de espera, a busca de doadores e acelera o acesso aos cuidados pré-transplante. O estudo descreve a otimização do acesso ao TMO por meio de um ambulatório para avaliação de pacientes pré-transplante. Estudo retrospectivo, 2021-2023 no Hospital Universitário Walter Cantídio. Dados analisados ​​no SPSS. Mostra 226 transplantes de 3.646 consultas. A clínica encaminhou 45 pacientes, contribuindo com 19,1%, 11,7% e 22,1% dos transplantes em 2021, 2022 e 2023, respectivamente, em comparação com &lt;5% nos anos anteriores. Este aumento destaca a importância do acesso rápido aos cuidados pré-transplante, resultando num maior número de pacientes elegíveis para transplante.

Comparison of Echocardiography and Multi-Planar Gated Acquisition Scans for Predicting Cancer-Treatment-Related Cardiovascular Dysfunction

Current guidelines recommend using sequential cardiac imaging to monitor for cancer treatment-related cardiac dysfunction (CTRCD) in patients undergoing potentially cardiotoxic chemotherapy. Multiple different imaging cardiac modalities are available and there are few prospective head-to-head comparative studies to help guide treatment. To perform an exploratory prospective cohort study of "real-world" CTRCD comparing multigated acquisition nuclear ventriculography (MUGA) at the referring cancer specialist's discretion with a novel echocardiographic strategy at an Australian tertiary hospital. Patients were recruited from haematology and oncology outpatient clinics if they were scheduled for treatment with anthracyclines and/or trastuzumab. Patients underwent simultaneous MUGA-based cardiac imaging (conventional strategy) at a frequency according to evidenced-based guidelines in addition to researcher-conducted echocardiographic imaging. The echocardiographic imaging was performed in all patients at time points recommended by international society guidelines. Outcomes included adherence to guideline recommendations, concordance between MUGA and echocardiographic left ventricular ejection fraction (LVEF) measurements, and detection of cardiac dysfunction (defined as >5% LVEF decrement from baseline by three-dimensional [3D]-LVEF). A secondary end point was accuracy of global longitudinal strain in predicting cardiac dysfunction. In total, 35 patients were recruited, including 15 with breast cancer, 19 with haematological malignancy, and one with gastric cancer. MUGA and echocardiographic LVEF measurements correlated poorly with limits of agreement of 30% between 3D-LVEF and MUGA-LVEF and 37% for 3D-LVEF and MUGA-LVEF. Only one case (2.9%) of CTRCD was diagnosed by MUGA, compared with 12 (34.2%) cases by echocardiography. Four (4) patients had >10% decrement in 3D-LVEF that was not detected by MUGA. Global longitudinal strain at 2 months displayed significant ability to predict CTRCD (area under the curve, 0.75, 95% confidence interval, 0.55-0.94). The MUGA correlates poorly with echocardiographic assessment with substantial discrepancy between MUGA and echocardiography in CTRCD diagnosis. Echocardiographic and MUGA imaging strategies should not be considered equivalent for imaging cancer patients, and a single imaging modality should ideally be used per patient to prevent misdiagnosis by inter-modality variation These findings should be considered hypothesis-generating and require confirmation with larger studies.

An Unusual Complication In A Patient With Hodgkin Lymphoma: Bilateral Auricular Hematoma

Hodgkin lymphoma is lymphoid neoplasm in which the malignant cells admixed with a heterogeneous population of non-neoplastic inflammatory cells. A combination of Doxorubicin, Bleomycin, Vinblastine, and Dacarbazine (ABVD) is the most commonly used chemotherapy regime for Hodgkin lymphoma. In this case report, we present a patient with Hodgkin lymphoma who developed bilateral auricular hematoma after a first time administrition of ABVD treatment. A 27-year-old female patient was presented to the hematology outpatient clinic with a swelling in the neck for two weeks. An excisional biopsy performed from the left axillary region. The pathology report demonstrated classical Hodgkin lymphoma was noticed to be consistent with mixed cellular type. First dose ABVD protocol was administered to the patient. One week after chemotherapy, swelling and discoloration were observed in the patient's bilateral auricles without a history of trauma. The patient was consulted to the Department of Otorhinolarngology-Head and Neck Surgery. Bilateral drainage of auricular hematoma was performed immediately. Intravenous ciprofloxacin was also administered. The auricular hematoma did not reoccur after the subsequent chemotherapy treatments.

Is Immune Thrombocytopenia and its Treatment Associated with Sarcopenia?

Immune thrombocytopenia (ITP) is defined as an isolated platelet count less than 100 × 109/L in the absence of other causes of thrombocytopenia. Sarcopenia is a body-wide muscular disorder with a progressive nature that leads to reduced mobility, physical disability, falls, and poor quality of life. We aimed to evaluate the frequency of objectively diagnosed sarcopenia in patients with ITP and to determine whether ITP therapies have sarcopenic effects. This prospective study included patients who were followed up with ITP in the hematology outpatient clinic. Patients who had received corticosteroids within 3 months were excluded. The handgrip strength test, appendicular skeletal muscle mass (ASMM), ASMM/height2 value, soft lean mass (SLM), trunk soft lean mass (SLMT), and the 6-min walking speed test were applied for muscular evaluations and physical performance assessment. We included 53 patients (female/male: 73.58%/26.42%). While sarcopenia was not observed in 77.36% of ITP patients, possible sarcopenia was diagnosed in 9.43% and confirmed sarcopenia in 13.21%. Severe sarcopenia was not seen in any of the patients. Loss of muscle strength was observed in 22.64% of patients. SLM was found to be low in 92.45%. Sarcopenia may be more frequent among patients with ITP compared to the population, and it is important to note that 92.45% of patients had low SLM and 54.72% had low SLMT. Eltrombopag therapy might be beneficial as demonstrated by higher SLM, ASMM, and ASMM/height2 values.

Eligibility for hematopoietic stem cell transplantation in a cohort of children with sickle cell disease: a single-center report

BackgroundHematopoietic stem cell transplantation (HSCT), is the only currently available curative option for SCD. Yet, the eligibility of SCD patients for HSCT is always limited by the significant associated toxicity and lack of suitable donors. At Cairo University’s pediatric hematology outpatient clinic, we aimed to determine hematopoietic stem cell transplantation (HSCT) candidates among a sickle cell disease (SCD) cohort, estimate the number of possible donors, and analyze the differences between patients with and without an HSCT indication.MethodsThis study was a cross-sectional analytic study including 128 SCD children. Their demographic, clinical, and laboratory profiles, total number, and number of siblings with SCD were obtained from their medical records.ResultsSixty-nine (53.9%) had at least one HSCT indication. Recurrent severe pain episodes despite hydroxyurea were the most common indication. Hemoglobin was lower, while reticulocyte count, serum ferritin, and aspartate aminotransferase were higher in HSCT candidates (p value < 0.001). Additionally, the prevalence of splenomegaly, the dose of hydroxyurea, and the number of transfusions were noticeably higher in HSCT candidates (p value = 0.013, 0.005, and < 0.0001 respectively). Among those indicated for HSCT; 75.3% had at least one healthy sibling who might be a potential donor.ConclusionMore than half were eligible for HSCT which should always be considered to provide a possible cure for the disease. Of the transplantation-eligible cases, about two-thirds had at least one healthy sibling who might potentially serve as a donor. Those meeting the requirements for HSCT eligibility should routinely undergo human leukocyte antigen (HLA) testing of their unaffected siblings.

Eating Behavior, Nutritional Status, and Taste Perception Alteration in Children with Cancer.

The aim of this study was to investigate eating behavior, nutritional status, and taste alterations in children with cancer. The population of the study consisted of children 8 to 18 years of age and their parents who were followed up and received chemotherapy in the pediatric hematology and oncology clinic and outpatient clinic of a University Faculty of Medicine Oncology Hospital. Data were collected using the Child Identification Form, the Children's Eating Behavior Questionnaire (CEBQ), the Taste Alteration Scale for Children Receiving Chemotherapy (TAC-TAS), and the Subjective Total Taste Acuity Scale (STTA). Body Mass Index (BMI) Z score was between -2 and +2 (normal) in 92.5% of the children and below ≤-2 (malnutrition) in 7.5%. The mean CEBQ subdimensions scores were food craving 12.48±5.36, emotional overeating 5.28±1.45, enjoyment of food 16.83±5.41, passion for drinking 9.72±5.13, satiety enthusiasm 22.93±6.65, slow eating 9.81±4.95, emotional undereating 16.38±4.41, and food selectivity 10.72±2.86, and the mean total TAC-TAS score was 8.66±10.22. A negative, moderate correlation was determined between food craving and enjoyment of food and taste alteration, with food craving and enjoyment decreasing as food alteration increased. A positive moderate correlation was observed between slow eating and taste alteration, with eating slowing down as taste alteration increased.

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.

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.

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.

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.

Improving Interprofessional Communication in the Outpatient Hematology Clinic at a Metropolitan Cancer Center

Background: Effective communication within a healthcare team leads to improved patient safety, more effective interventions, enhanced employee morale, and increased patient and family satisfaction. Our study aims to improve interprofessional communication between hematologists and referring providers in our ambulatory hematology clinic. First, we assessed the frequency with which hematologists communicated patient care recommendations to referring providers. After we identified a gap in communication, we implemented a quality improvement intervention to address the gap. Methods: A retrospective review was conducted to identify new patients ≥18 years old referred to the hematology clinic from January 1, 2022, to July 31, 2022. The data assessed included demographics, reason for referral, show rate, time from referral to first visit, and whether there was documentation of recommendations communicated from the hematologist to the referring provider. Communication was also counted if the patient was referred from an internal provider who had access to the medical record. After a gap in communication was identified, we implemented a quality improvement measure to document referring providers' names and contact information in every initial office visit note and then fax over all new patient notes with hematology recommendations to the referring provider at the end of each week. All fax communications were documented in patients' electronic charts. Results: A total of 217 new patients were referred to the hematology clinic from January 1, 2022, to July 31, 2022. The median age was 53 (range, 18-94) years; 43% of patients were seen in the clinic, and 56% were no-shows. Primary referral reasons were anemia (44%), thrombosis (21%), leukocyte abnormalities (12%), and thrombocytopenia (11%). The average time between referral date and first visit was 95 days. Of the 43% (n=93) of patients seen in the clinic, only 3% (n=3) had documented communication between the hematologist and referring provider prior to the quality intervention. In the second phase of the study, data was collected after implementation of the intervention from October 1, 2022, to June 30, 2023. There were 245 new patient referrals, with 49% of patients seen in clinic. The average time between referral date and first visit was 70 days. The rate of communication between the referring provider and hematologist improved to 86%. Conclusion: At our outpatient hematology clinic, we identified a gap in communication between the referring providers and hematologists. Often a provider refers a patient to hematology but is unaware of the recommendations that were made. This can lead to unclear recommendations and treatment plans, poor patient care, and frustration among patients and providers. We implemented an intervention to improve communication with referring providers. Our intervention was successful in improving the communication flow and enhanced communication from 3% to 86%. We recommend adoption of our intervention by other clinics at our institution to enhance their communication flow between referring providers and specialists. Our study identified additional issues, including a high no-show rate and long wait time from referral date to first visit. Although this was not the primary focus of our quality improvement project, additional quality improvement measures aimed at addressing these gaps are needed.

Selenium Deficiency and Chronic Anemia in an Outpatient Hematology Clinic

Background Selenium (Se) is an essential micronutrient with roles in reproduction, metabolism, immunity, and the cellular cycle, including DNA synthesis and prevention of cell death. Its role in malignancy has also been studied, specifically for esophageal, gastric, lung, prostate cancer. Studies have shown benefit with Se supplementation for those malignancies. There is evidence that low Se levels are independently associated with worse outcomes regarding treatment response and long-term survival for Non-Hodgkin's Lymphoma (NHL) and cutaneous T-cell lymphoma, and additional studies on its association in acute myeloid leukemia (AML), follicular lymphoma (FL), and Hodgkin's lymphoma (HL) have revealed worsening outcomes as well. However, Se was not predicative of treatment response or survival for AML, FL and HL, therefore it's role in the development of hematological malignancies is not clear. Previously, the role of Se in myelodysplastic syndrome (MDS) was investigated, indicating the importance of Se for apoptosis in MDS cell lines. There is evidence that a causal relationship with decreased Se levels and chronic anemia might exist; however, there is a paucity of data regarding its role in the development of chronic anemia and hematological disorders. This descriptive single center study describes Se deficiency in patients with chronic anemia. Objectives Primary endpoint was to determine if there was evidence of Se deficiency in patients with chronic anemia in an academic hematology outpatient clinic. Methods We herein present a descriptive study on 18 patients with hematological disorders and selenium deficiency. Upon initial detection of Se deficiency, levels of hemoglobin (Hb), mean corpuscular volume (MCV), Copper (Cu), Zinc (Zn) and iron (Fe) closest to time of detection were noted if tested. Average values with standard deviation were calculated and association with malignancies were noted. Results Of the 18 patients, 13 had pre-malignant or malignant conditions, including 7/13 who had bone marrow failure/MDS, 5/13 patients with lymphoid disorders, and 1/13 with plasma cell disorders. The 5 remaining patients had chronic gastrointestinal disorders. Averages on detection of Se deficiency were noted to be: Hb (9.9 ± 2.0), MCV (96.0 ± 11.2), Se (100.5 ± 11.6), Cu (95.8 ± 28.8), Zn (70.1 ± 21.5) and Fe (81.6 ± 56.7). No association was noted between Se and other trace elements. 72% (13/18) of patients with Se deficiency also had malignant conditions. Of those with malignancy, 54% were noted to have bone marrow failure. Discussion Currently there are no clinical guidelines that recommend following levels of Se routinely in patients with chronic anemia. Our data might indicate a possible association between chronic anemia and Se deficiency. Given the high number of MDS patients in our study, there might be a link between Se deficiency and MDS. Further investigation is warranted into the role of Se in the development of chronic anemia disorders, as supplementation with Se and routine follow-up may be warranted and can improve clinical outcomes.

Prevalence of Voice and Swallow Problems in Individuals Living with Sickle Cell Disease

Introduction: Sickle Cell Disease (SCD) is an inherited blood disorder with profound implications on a wide variety of tissue types (Kavanagh et al., 2022). As many as 37% of patients with SCD experience silent cerebral infarction by age 14 (DeBaun et al., 2012). Neurological insults can increase risk for voice and swallow disorders which compromise physical health and diminish quality of life for many individuals (Aronson &amp; Bless, 2009). In fact, voice disorders (dysphonia) affect 6% of adults (Roy et al., 2005), and swallowing difficulties (dysphagia) impact as many as 16% (Adkins et al., 2020). Since both phonation and deglutition require the coordination of multiple muscles and nerves (Kendall, 2008), diseases such as SCD may be associated with elevated risk for dysphonia and dysphagia. Unfortunately, the frequency of these symptoms in individuals with SCD has rarely been considered. This study examined the prevalence and nature of dysphonia and dysphagia in individuals with SCD. Methods: Adults (age &amp;gt;18) diagnosed with SCD were surveyed regarding the presence of voice and swallow complaints, symptomology, voice-use patterns, voice and swallow-related quality of life, demographic information, and medical history. Additionally, participants completed the Voice Handicap Index-10 (VHI-10), Eating Assessment Tool (EAT-10), and Reflux Severity Index (RSI). To enable comparisons with previous epidemiological studies of voice and swallow disorder prevalence, survey questions were adapted from previously published investigations (Roy et al., 2004, 2005). Participants were recruited from an outpatient hematology clinic during routine visits and completed the survey online using Redcap. Univariate and multivariate analyses were used to identify dysphonia and dysphagia risk. Results: Twenty-four patients have completed this study (males=16, females=8; mean age=35.2 years; genotype: SS=8, S/β thalassemia zero=3, SC=6, S/β thalassemia plus=7) with recruitment ongoing. Forty-five percent of patients reported a voice complaint within the last 4 years with symptoms lasting longer than 4 weeks in all cases (Table 1). Sixty-six percent of patients presented with VHI scores indicating a voice problem. Dysphonia was described as episodic, with 83.3% of cases becoming more severe during sickle cell crises. Voice problems resulted in exclusion from social activities (37.5%), missed days of work (12.5%), inability to perform daily tasks (12.5%), inability to sing (27.2%), and difficulty performing one's occupation (12.5%). Individuals who vaped were 8% more likely to report dysphonia ( p=.04), and those who drank more than 3 alcoholic beverages a week were 25% more likely to report dysphonia ( p=.03). Patients with dysphonia presented with significantly more severe VHI scores ( p=.019, dysphonia=15.1, normophonic=10.0) and RSI scores ( p=.036, dysphonia=10.5, normophonic=6.8) than those with no voice complaint. No voice use pattern predicted voice disorder risk. One third of participants reported a swallow complaint, with 75% of cases occurring within the last 2 years (Table 1). Forty-one percent of patients presented with EAT-10 scores indicating dysphagia. Swallowing problems were evident with meats (8.3%), bread-like consistencies (20.8%), pills (29.2%), and thin liquids (4.2%). Dysphagia symptoms were episodic in 87.5% of cases, becoming more severe during sickle cell crisis in 37.5% of cases. Patients with dysphagia symptoms presented with significantly more severe EAT-10 scores than those without swallow symptoms ( p&amp;lt;.001, dysphagia group=6.12, no dysphagia=2.06). No patients reported seeing a medical professional for dysphonia or dysphagia symptoms. Conclusions: Dysphonia and dysphagia were markedly elevated in patients with SCD when compared with the general population. Although these symptoms diminished quality of life, no patient had received treatment or even evaluation of these symptoms. Given the medical complexity of patients with SCD, diagnostic overshadowing might have precluded thorough workups of symptoms. This is unfortunate as impairments in voice and swallow function greatly impact quality of life and, in some cases, can be life-threatening. If the current findings are confirmed by future large-scale studies, individuals with SCD may benefit from proactive systematic monitoring and treatment of the subsystems responsible for voice and swallow.