Prevalence of neutropenia among adult Arabs in Qatar: Relation to other hematological parameters and anthropometric data.

Neutropenia ranges from a normal variant to life-threatening acquired and congenital disorders. This study aims at providing baseline information regarding the prevalence and spectrum of neutropenia in the Arab blood donors who are living in Qatar. This retrospective cohort study was conducted to review the data of healthy Arab individuals (≥18 years) who donated blood between January 1, 2015 to May 15, 2019. A complete blood count was performed using automated analyzers. The prevalence of neutropenia was 10.7%. The prevalence in females was 32% and in males, it was 6%. Absolute neutrophil count (ANC) below 1 × 109/L was detected in 10% of Arab females and 1.8 % of Arab males. In females, the neutropenic group had significantly lower hemoglobin (Hb) levels and higher red cell distribution width, and lower total white blood cells and lymphocyte counts (P < .001) compared to the group with ANC > 1.5 × 109/L. Significant correlations were found between the ANC and Hb (r = 0.33, P < .05) and ANC and total white blood cells (r = 0.45, P < .01). The prevalence of neutropenia is considerably high in Arab adult females compared to other ethnic groups. Besides the genetic constitution of Arabs, the lower Hb and higher red cell distribution width in females suggest that iron deficiency could contribute to the development of neutropenia.

Hematological indices reference intervals for a healthy Arab population in Qatar: Effect of age, gender, and geographic location.

ABSTRACT: Hematologic reference intervals vary with gender, age, ethnicity, and geographic area. Therefore, local or national laboratory reference ranges are essential to enhance the accuracy when diagnosing health conditions. Still, no comprehensive list of reference ranges tailored to the Arab population living in Qatar. Accordingly, this study aims at establishing a hematology reference guide for Arabs in Qatar.This is a retrospective study where 750 healthy volunteers (18-69 years) from 2015 to 2019 were included, analyzed by an automated hematology analyzer. Arab adults were divided into African (Egypt, Libya, Tunisia, Morocco) and Asian (Syria, Lebanon, Jordon, Palestine, Qatar). The Cell-Dyn and Sysmex were used for measuring hematological parameters.The mean +/- 2SD were established for all the study groups. Arab males had significantly higher Hb, Hct, red cell distribution width, absolute neutrophil count, lymphocytes, and monocyte counts than females. Asian-Arab males had significantly higher Hb concentration and higher WBC, lymphocytes, and eosinophils than African Arabs. Asian-Arab young (>18: < 40 years) males had significantly higher Hb and lymphocytes and lower monocytes than older males (>40 years). African-Arab young males had significantly higher lymphocytes and lower monocytes than older males. Asian-Arab young females had higher WBC and absolute neutrophil count than older Asian Arabs.The findings of this study will help in establishing specific reference intervals in the Arab world. The differences in hematology reference intervals considering age, gender, and geographical location highlight the importance of establishing blood reference intervals in each country considering the ethnic diversity of each country.

Assessing Bone Marrow Activity with [18F]FLT PET in Patients with Essential Thrombocythemia and Prefibrotic Myelofibrosis: A Proof of Concept.

Objectives: This study aims to assess the value of FLT-PET as a non-invasive tool to differentiate between patients with ET and Pre-PMF. This study is a pilot study to have a proof of concept only. Methods: This is a prospective, interventional study where a total of 12 patients were included. Each patient underwent FLT PET imaging as well as bone marrow examination (gold standard). In addition, semi-quantitative (SUVmax and SUVmean) measurements of FLT uptake in the liver, spleen, and Lspine, SUVmean, as well as the Total Lesion Glycolysis (TLG) of the Lspine were performed. Results from the two patient cohorts were compared using = Kruskal-Wallis statistical test. A P-value of <.05 is considered to be statistically significant. Results: The differences in FLT SUVmax and SUVmean measurements in the three organs (liver, spleen, and LSpine) between the ET and Pre-PMF patients were not statistically significant (P > .05). In contrast, TLG measurements in the LSpine were statistically different (P = .013), and therefore, compared to gold standard bone marrow results, TLG can separate ET and Pre-PMF patients. Conclusion: This study is a proof of concept about the potential to discriminate between ET and pre-PMF patients in a non-invasive way. TLG of the LSpine in FLT PET images is a potential quantitative parameter to distinguish between ET and pre-PMF patients.

Ophthalmologic manifestations as the initial presentation of chronic myeloid leukemia: A review.

The presentation of chronic myeloid leukemia (CML) can be variable and related to the phase of the disease. It can manifest a wide range of symptoms and signs; ocular involvement is reported in patients with leukemia at the time of diagnosis. We describe ophthalmic manifestations as an initial presentation in patients with CML. We identified 38 publications between 1971 and 2020 describing ocular manifestations in CML. Ophthalmic problems occur either from direct or indirect infiltration of neoplastic cells or from secondary causes. Although nearly all ocular structures may be affected, leukemic retinopathy is the most frequent clinical manifestation. Others include iris infiltration, anterior uveitis, hypopyon, exudative/serous retinal detachment, and optic nerve infiltration.

A study of 18F-FLT positron emission tomography/computed tomography imaging in cases of prefibrotic/early primary myelofibrosis and essential thrombocythemia.

The objectives of this research project are to study in patients with primary myelofibrosis (PMF) and Essential Thrombocythemia (ET); (1) the uptake patterns of FLT-PET (FLT-PET) and its value in diagnosing, staging, and treatment response monitoring of malignant hematopoiesis, (2) compare imaging findings from FLT-PET with bone marrow biopsy (standard of care), and (3) associate FLT-PET uptake patterns with genetic makeup such as JAK2 (Janus kinase 2), CALR (Calreticulin), MPL (myeloproliferative leukemia protein), Triple negative disease, and allele burden.This trial is registered in ClinicalTrials.gov with number NCT03116542. Protocol version: Mar 2017.

Rituximab Twice Weekly for Refractory Thrombotic Thrombocytopenic Purpura in a Critically Ill Patient with Acute Respiratory Distress Syndrome.

Thrombotic thrombocytopenic purpura (TTP) is a rare, serious, life-threatening disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, and hypercoagulability. The etiology is a deficiency of ADAMTS13 which is usually caused by acquired antibodies. Plasma exchange and steroids is the standard of care in the treatment of TTP. However, there are refractory cases of TTP which require further management. Rituximab appears to be a safe and effective therapy for refractory and relapsing TTP. Here we report a challenging case of TTP that responded to treatment with rituximab twice weekly. According to our knowledge, rituximab twice weekly has never been used for TTP before.

Acute Myeloid Leukemia Presenting with Numb Chin Syndrome: A Case Report and Review of Literature.

BACKGROUND Numb chin syndrome is a rare and under diagnosed neuropathy of the inferior alveolar branch of the trigeminal nerve usually causing a lower lip and chin anesthesia or paresthesia. The syndrome is commonly associated with broad-spectrum malignant and non-malignant conditions. CASE REPORT Here we report a case of a 30-year-old male who presented with numb chin syndrome in the form of jaw pain, paresthesia, and hypoesthesia of the mental area as the presenting symptoms of acute of myeloid leukemia with t(8;21) treated with (3+7) protocol (3 days anthracycline+7 days cytarabine). The pain and paresthesia improved but hypothesia persisted. CONCLUSIONS Acute myeloid leukemia is one of the most serious causes of numb chin syndrome which should not be overlooked.

Ophthalmologic Manifestations as Initial Presentation of Patients with Chronic Myeloid Leukemia: Report of Two Cases.

Chronic myeloid leukemia (CML) is a myeloproliferative neoplasm, classically described as triphasic disease (chronic, accelerated, and blast). Despite the significant efforts made by different groups to optimize treatment and outcome, there are still unmet needs and unanswered questions. Ophthalmologic manifestations are among the therapeutic challenges. The best available therapy for patients presenting with ophthalmologic manifestations is still unclear. Here we present two cases of CML (chronic phase) with ophthalmologic manifestations as initial presentation, trying to shed light on this important type of presentation and proposing recommendations for hematologists in view of the current literature.

Paroxysmal Nocturnal Hemoglobinuria with Glucose-6-Phosphate Dehydrogenase Deficiency: A Case Report and Review of the Literature.

In this study, we are describing a female patient with paroxysmal nocturnal hemoglobinuria (PNH) and glucose-6-phosphate dehydrogenase (G6PD) deficiency. Both diseases are known to cause hemolytic anemia that mediates the hemolysis of RBCs through several mechanisms. In PNH the hemolysis is mediated through complement activation and oxidative stress. G6PD enzyme is crucial in preventing damage to cellular structures caused by oxygen-free radicles. In G6PD deficiency the hemolysis is mediated through the oxidative stress created by oxygen-free radicles. Since both diseases mediate hemolysis through the oxidative stress, we hypothesize that both conditions have facilitated an effect on each other and this will reflect on the response to treatment, and this response to treatment could vary based on whether the two mutations occurred in the same gene or in two different X chromosomes. Having diagnosed PNH, the management is very expensive and not all the patients can afford it, especially our patient who is a maid by occupation. So, the real challenge in our case is to monitor her in subsequent visits and to plan the treatment keeping in mind her financial status.

Concomitant BRAF Mutation in Hairy Cell Leukemia and Papillary Thyroid Cancer: Case Report.

Hairy cell leukemia (HCL) is rare type of leukemia. This neoplasm is well-known to present with pancytopenia and splenomegaly. HCL is associated with BRAF mutation in 100% of cases. It is also associated with hematological and oncological malignancies such as melanoma and papillary thyroid cancer. Although the association of both cancers (HCL and papillary thyroid cancer) with BRAF mutation is well established in the literature, as far as we know it has not been reported before in the same patient. Here we report 48-year-old male diagnosed with HCL and papillary thyroid cancer and who is BRAF positive in both diagnostic tissues.

Hypercalcemia and acute pancreatitis in a male patient with acute promyelocytic leukemia and pulmonary tuberculosis.

We report a rare case of hypercalcemia and acute pancreatitis in a subject with acute promyelocytic leukemia (APL) and pulmonary tuberculosis, during all-trans-retinoic acid (ATRA) treatment. Both associated complications were potentially due to several causes. A careful monitoring and exclusion of all causative factors must be addressed. Further research is necessary to improve our understanding of risk factors for these complications in patients with (APL). Studying these patterns may help us to improve outcomes for all children and young adults with hematologic malignancies.

The emergence of multidrug-resistant Pseudomonas aeruginosa in cystic fibrosis patients on inhaled antibiotics.

INTRODUCTION: Multidrug-resistant Pseudomonas aeruginosa (MDR-PA) is an important and growing issue in the care of patients with cystic fibrosis (CF), and a major cause of morbidity and mortality. OBJECTIVE: The objective of the study was to describe the frequency of MDR-PA recovered from the lower respiratory samples of pediatric and adult CF patients, and its antibiotic resistance pattern to commonly used antimicrobial agents including ß-lactams, aminoglycosides, and fluoroquinolones. MATERIALS AND METHODS: The lower respiratory isolates of P. aeruginosa were obtained from inpatients and outpatients CF clinics from a tertiary care teaching hospital for the period from October 2014 to September 2015. The identification and antimicrobial susceptibility for all the isolates were performed by using the BD Phoenix™ and E-test in compliance with Clinical and Laboratory Standards Institute (CLSI) guidelines. RESULTS: A total of 61 P. aeruginosa samples were isolated from thirty CF patients from twenty families. Twelve sputum samples were positive for MDR-PA (seven nonmucoid and five mucoid isolates) from five CF patients (five families) with moderate-to-very severe lung disease given MDR-PA frequency of 19.7%. The median age of the study group was 20 (range 10-30) years. Three CF patients were on chronic inhaled tobramycin and two on nebulized colistin. The antimicrobial patterns of isolates MDR-PA showed the highest rate of resistance toward each gentamycin, amikacin, and cefepime (100%), followed by 91.7% to ciprofloxacin, 75% to tobramycin, 58.3% to meropenem, and 50% to piperacillin-tazobactam. None of the isolates were resistant to colistin during the study period. CONCLUSION: The study results emphasize that the emergence of a significant problem in the clinical isolates of P. aeruginosa in CF patients that dictate appropriate attention to the antibiotic management after proper surveillance.

Cytomegalovirus-induced Hemorrhagic Colitis in a Patient with Chronic Myeloid Leukemia (Chronic Phase) on Dasatinib as an Upfront Therapy.

Dasatinib is a kinase inhibitor indicated for the treatment of newly diagnosed adults with Philadelphia chromosome-positive (Ph+) chronic myeloid leukemia (CML) in chronic phase and accelerated (myeloid or lymphoid blast) phase, and CML with resistance or intolerance to prior therapy including imatinib and in adults with Ph+ acute lymphoblastic leukemia1 The most common adverse reactions (≥15%) in patients with newly diagnosed chronic-phase (CP) CML include myelosuppression, fluid retention, and diarrhea, whereas in patients with resistance or intolerance to prior imatinib therapy, side effects include myelosuppression, fluid retention, diarrhea, headache, dyspnea, skin rash, fatigue, nausea, and hemorrhage. We report a 39-year-old Ethiopian female patient who received dasatinib as upfront therapy for the treatment of CP-CML who experienced chronic diarrhea for two months, which progressed to hemorrhagic colitis due to cytomegalovirus (CMV) infection of the colon. To our knowledge, this is the first case of CMV colitis in a patient receiving dasatinib as upfront therapy.

Patient Demographics and Socioeconomic Characteristics of an Ambulatory Care Clinic Served by a University PCCM Training Program.

OBJECTIVES: Continuity clinics are an important aspect of pulmonary medicine fellowship training. We provide a description of a pulmonary outpatient clinic in an inner city, county-owned, university-affiliated hospital. METHODS: This is a descriptive study of administrative data on consecutive patient visits to the University of Tennessee Regional One Health at Memphis ambulatory clinic (Medplex) between January 2000 and August 2006. We describe demographics, socioeconomic characteristics, and the frequency of a pulmonary diagnosis of the outpatient population served by our training program. Continuous data were described by mean ± standard deviations and categorical data were described by percentage. RESULTS: The dataset included 2549 patients, 81% were African American with a mean age of 48.7 ± 13.7, 64.4% were women. Female/male body mass index was 34.6 ± 11.6 vs. 29.2 ± 10.3. Tenncare (Medicaid) covered 59.6 % of patients, whereas 11.1% were uninsured. CONCLUSIONS: We provide evidence that one clinical setting may not be enough exposure to the entire lung pathology for pulmonary trainees. There is a need for further, larger, and prospective data collections to evaluate and guide changes regarding the structure of training programs.

Dasatinib Induced Avascular Necrosis of Femoral Head in Adult Patient with Chronic Myeloid Leukemia.

Chronic myeloid leukemia (CML) is a myeloproliferative neoplasm characterized by the presence of the Philadelphia (Ph) chromosome resulting from the reciprocal translocation t(9;22)(q34;q11). The molecular consequence of this translocation is the generation of the BCR-ABL fusion gene, which encodes a constitutively active protein tyrosine kinase. The oncogenic protein tyrosine kinase, which is located in the cytoplasm, is responsible for the leukemia phenotype through the constitutive activation of multiple signaling pathways involved in the cell cycle and in adhesion and apoptosis. Avascular necrosis of the femoral head (AVNFH) is not a specific disease. It occurs as a complication or secondary to various causes. These conditions probably lead to impaired blood supply to the femoral head. The diagnosis of AVNFH is based on clinical findings and is supported by specific radiological manifestations. We reported a case of a 34-year-old Sudanese female with CML who developed AVNFH after receiving dasatinib as a second-line therapy. Though the mechanism by which dasatinib can cause avascular necrosis (AVN) is not clear, it can be postulated because of microcirculatory obstruction of the femoral head. To the best of our knowledge and after extensive literature search, this is the first reported case of AVNFH induced by dasatinib in a patient with CML.

Effect of 24-hour mandatory versus on-demand critical care specialist presence on quality of care and family and provider satisfaction in the intensive care unit of a teaching hospital.

OBJECTIVE: The benefit of continuous on-site presence by a staff academic critical care specialist in the intensive care unit of a teaching hospital is not known. We compared the quality of care and patient/family and provider satisfaction before and after changing the staffing model from on-demand to continuous 24-hr critical care specialist presence in the intensive care unit. DESIGN: Two-year prospective cohort study of patient outcomes, processes of care, and family and provider survey of satisfaction, organization, and culture in the intensive care unit. SETTING: Intensive care unit of a teaching hospital. PATIENTS: Consecutive critically ill patients, their families, and their caregivers. INTERVENTIONS: Introduction of night-shift coverage to provide continuous 24-hr on-site, as opposed to on-demand, critical care specialist presence. MEASUREMENTS AND MAIN RESULTS: Of 2,622 patients included in the study, 1,301 were admitted before and 1,321 after the staffing model change. Baseline characteristics and adjusted intensive care unit and hospital mortality were similar between the two groups. The nonadherence to evidence-based care processes improved from 24% to 16% per patient-day after the staffing change (p = .002). The rate of intensive care unit complications decreased from 11% to 7% per patient-day (p = .023). When adjusted for predicted hospital length of stay, admission source, and do-not-resuscitate status, hospital length of stay significantly decreased during the second period (adjusted mean difference -1.4, 95% confidence interval -0.3 to -2.5 days, p = .017). The new model was considered optimal for patient care by the majority of the providers (78% vs. 38% before the intervention, p < .001). Family satisfaction was excellent during both study periods (mean score 5.87 +/- 1.7 vs. 5.95 +/- 2.0, p = .777). CONCLUSIONS: The introduction of continuous (24-hr) on-site presence by a staff academic critical care specialist was associated with improved processes of care and staff satisfaction and decreased intensive care unit complication rate and hospital length of stay.