Genetic Patterns of Oral Cavity Microbiome in Patients with Sickle Cell Disease.

The Middle Eastern prevalence of sickle cell anemia, a genetic disorder that affects red blood cells, necessitates additional research. On a molecular level, we sought to identify and sort the oral microbiota of healthy individuals and those with sickle cell anemia. Furthermore, it is crucial to comprehend how changes in the genetic makeup of the oral microbiota impact the state of sickle cell anemia. Using next-generation sequencing, the 16S rRNA amplicon was examined using saliva samples from 36 individuals with sickle cell anemia and healthy individuals. These samples were obtained from sickle cell anemia patients (18 samples) and healthy control participants (controls, 18 samples). Various analyses are conducted using bioinformatic techniques to identify distinct species and their relative abundance. Streptococcus, followed by Fusobacterium nucleatum, Prevotella, and Veillonella were the most prevalent genera of bacteria in the saliva of the SCA and non-SCA individuals according to our findings. Rothia mucilaginosa, Prevotella scoposa, and Veillonella dispar species were the dominant species in both sickle cell anemia and non-sickle cell anemia subjects. Streptococcus salivarius, Actinomyces graevenitzii, Actinomyces odontolyticus, and Actinomyces georgiae spp. were the most prevalent bacterial spp. in the studied SCA cases. The sequencing of the 16S rRNA gene yielded relative abundance values that were visualized through a heatmap analysis. Alterations in the oral microflora's constitution can significantly affect the susceptibility of sickle cell anemia patients to develop more severe health complications. Salivary diagnosis is a potential tool for predicting and preventing oral microbiome-related diseases in the future.

Association of blood group with COVID-19 disease susceptibility and severity in Saudi Arabia.

BACKGROUND: Novel SARS-CoV-2 (COVID-19) virus has rapidly spread worldwide and was declared a pandemic, making identifying and prioritizing individuals most at risk a critical challenge. The literature describes an association between blood groups and the susceptibility to various viral infections and their severity. Knowing if a specific blood group has more susceptibility to COVID-19 may help improve understanding the pathogenesis and severity of the disease. We aimed to assess the association between ABO/RhD and COVID-19 susceptibility and severity, and to compare results with similar studies in Saudi Arabia. STUDY DESIGN AND METHODS: This study was conducted between March and October 2021 on 600 patients confirmed positive for COVID-19 infection. Patients' data were collected and analyzed. As a control, 8423 healthy blood donors were enrolled as a sample representative of the population for blood group distribution. RESULTS: More individuals had blood group B in the COVID-19 group in comparison with the control group (24.2% vs. 18%), The opposite was observed among individuals of group O (39.5% vs. 47.3%). The B blood group was predictive of higher risk of mortality. No significant difference in the distribution of RhD was observed between the COVID-19 and the control groups. Neither ABO nor RhD was significantly associated with the severity of COVID-19. DISCUSSION: Although there was no significant association with the disease severity, the B blood group may be associated with a higher risk for COVID-19 infection. Further studies with a larger sample size are necessary to evaluate this correlation.

A Saudi multicenter experience on therapeutic plasma exchange for patients with thrombotic thrombocytopenic purpura: A call for national registry.

BACKGROUND: The improvement in the clinical care for patients with thrombotic thrombocytopenic purpura (TTP) is evolving, and many efforts are being put to standardize it. Here, we aimed to assess the provided care at a national level and identify deficiencies. METHODS: A national Saudi retrospective descriptive study was carried out at six tertiary referral centers and included all patients who underwent therapeutic plasma exchange (TPE) for the diagnosis of TTP between May 2005, and July 2022. Collected information included demographic data, clinical features on presentation, and the results of laboratory investigations at admission and discharge. In addition, the number of TPE sessions, days till the first session of TPE, usage of immunological agents, and clinical outcomes were all collected. RESULTS: One hundred patients were enrolled, predominantly female (56%). The mean age was 36.8 years. At diagnosis, 53% of patients showed neurological involvement. The mean platelet count at presentation was 21 × 109 /L. All patients had anemia (mean hematocrit 24.2%). Schistocytes were present in the peripheral blood film of all patients. The mean number of TPE rounds was 13 ± 9.3, and the mean days to start TPE since admission for the first episode was 2.5 days. ADAMTS13 level was measured in 48% of patients and was significantly low in 77% of them. Assessing for clinical TTP scores, 83%, 1000%, 64% of eligible patients had an intermediate/high PLASMIC, FRENCH, and Bentley scores, respectively. Caplacizumab was used on only one patient, and rituximab was administered to 37% of patients. A complete response for the first episode was achieved in 78% of patients. The overall mortality rate was 25%. Neither time to TPE, the use of rituximab or steroid affected survival. CONCLUSIONS: Our study shows an excellent response to TPE with a survival rate approximate to the reported international literature. We observed a deficiency in using validated scoring systems in addition to confirming the disease by ADAMTS13 testing. This emphasizes the need for a national registry to facilitate proper diagnosis and management of this rare disorder.

Does Lymphocyte Count Impact Dosing of Anti-Thymocyte Globulin in Unrelated Donor Stem Cell Transplantation?

Anti-thymocyte globulin (ATG) is used to reduce the incidence and severity of graft-versus-host disease (GVHD) with hematopoietic cell transplantation, yet optimum dosing has yet to be determined. We have previously demonstrated that 2.5 mg/kg ATG in conditioning can reduce the incidence of GVHD in unrelated donor transplants. Recent literature has suggested that ATG dosing based on absolute lymphocyte count (ALC) could lead to more optimum exposure of the drug. We sought to determine if ALC at the time of transplant could impact clinical outcomes. We conducted a retrospective single-center study analyzing all consecutive patients at The Ottawa Hospital who received a matched unrelated donor stem cell transplant with ATG between 2009 and 2014. Patients received rabbit ATG (thymoglobulin) at 0.5 mg/kg on day -2 and 2.0 mg/kg on day -1. Univariate and multivariate analyses were used to determine if any patient- or transplant-related factors, including weight, ALC, and total ATG dose given, impacted GVHD, relapse, or mortality. In total, 111 patients met inclusion, with a median age of 50 years (range, 19 to 70). The most common diagnoses were acute myelogenous leukemia (43%), Myelodysplasia/myeloproliferative neoplasms (13%), and lymphoma (12%). The median weight at time of conditioning was 80.3 kg (range, 45 to 216). The median ALC on the first day of ATG administration was 0.1 × 109/L (range, 0 to 190). The median total dose of ATG received was 201 mg (range, 112 to 540 mg). The incidence of acute and chronic GVHD was 35.1% and 21.6%, respectively. In the multivariate model, the actual dose of ATG given to patients was not associated with GVHD (hazard ratio [HR], 1.11; 95% confidence interval [CI], 0.99 to 1.25; P = .07), relapse (HR, 1.13; 95% CI, 0.98 to 1.30; P = .1), or mortality (HR, 1.09; 95% CI, 0.92 to 1.28; P = .32). Similarly, the pretransplant ALC was not associated with GVHD (HR, 1; P = .82), relapse (HR, 1; P = .90), or mortality (HR, 1; P = .39). If patients had received ALC-based dosing according to previously published work (Admiraal et al., Lancet Haematol 2017), the mean total dose of ATG received would have been 1205 mg, more than 5 times the mean dose that was actually given based on weight. With GVHD outcomes being similar to that published by Admiraal et al. and ALC not independently associated with outcomes in our study, further studies are still needed to compare standard weight-based dosing to ALC-based dosing of ATG in matched unrelated donor stem cell transplant.

Profiling of microRNAs by next-generation sequencing: Potential biomarkers for diffuse large B-cell lymphoma.

Background: Lymphoma ranks fifth in prevalence among common cancer types worldwide. This lymphatic system cancer arises from T or B cells. Diffuse large B cell lymphomas (DLBCLs) are associated with most non-Hodgkin lymphomas. Non-coding microRNAs (miRNAs) greatly affect gene expression. A single miRNA can target numerous genes, thus largely influencing gene expression networks. MiRNAs can act as oncogenes or tumor suppressors in controlling DLBCL progression. This study investigated the roles of miRNAs in patients with DLBCL through next-generation sequencing, which was found to be sensitive, accurate, and robust. Methods: The study involved seven patients with DLBCLs and three controls at a hematology-oncology clinic. MiRNA was extracted from existing formalin-fixed, paraffin-embedded (FFPE) tissue specimens. Illumina next-generation sequencing was used to sequence samples for miRNA profiling. Results: Samples from patients showed expression of various hsa-mir miRNAs (1248, 3607, 21, 142, 1244, 182, 6516, 766, 1291, 4449, and 181a), whereas those from healthy individuals showed expression of hsa-mir 1248, 3607, 21, 142, and 877. Hsa-mir-877-3p is known to target multiple genes, and miRNAs such as hsa-mir-877-3p, hsa-mir-1291, and hsa-mir-181a-5p interact primarily with target genes. Conclusions: MiRNA profiling in FFPE tissues from patients with DLBCL suggested that miRNA levels can distinguish patients with DLBCL from controls, and therefore may provide prognostic or diagnostic biomarkers for DLBCL. Altered genes and miRNAs may also be potential therapeutic targets.

COVID-19-induced immune thrombocytopenia management approach: A case report and literature review.

Key Clinical Message: Vincristine therapy can be effective in refractory Immune thrombocytopenia (ITP) following COVID-19 vaccination. Our case report highlights the need for further research to establish standard management guidelines for COVID-19-vaccine-associated ITP. Abstract: Adult immune thrombocytopenia (ITP) can occur as a rare complication following several viral infections or a rare adverse event or complication of vaccination. In this paper, we report a case of a 39-year-old male patient with severe refractory ITP that began 4-weeks after receiving his third (booster) dose of the COVID-19 vaccine (BNT162b2, Pfizer-BioNTech). He was given oral dexamethasone 40 mg daily for 4 days followed by prednisone at 1 mg/kg (85 mg daily) for 10 days. In the following weeks, we attempted several other lines of therapy to treat his ITP, including anti-RhD immunoglobulin, which, unfortunately, caused moderate hemolysis requiring packed red blood cell transfusion, intravenous immunoglobulin (given at a subtherapeutic dose of 0.4 g/kg for only 1 day since it was not available), rituximab, and eltrombopag. The patient, unfortunately, showed no response to any of these treatments. This was an indicator to initiate salvage therapy with vincristine 2 mg weekly for 3 weeks. The patient's platelet count started to increase remarkably during the third week of vincristine and normalized after 4 weeks. We review the findings, clinical characteristics, and management approaches that were reported in the literature regarding COVID-19-vaccine-induced ITP. More in-depth research is needed to delineate standard guidelines for the management of such cases. This report underscores the importance of resorting to vincristine and eltrombopag as great options for severe and refractory ITP related to the COVID-19 vaccine.

Cardiovascular Consequences of Sickle Cell Disease.

Background: Sickle cell disease is an inherited blood disorder which can lead to severe complications, particularly in the cardiovascular and respiratory systems, potentially resulting in arrhythmias, pulmonary hypertension (PH), and cardiomegaly. This study aims to investigate the risk of PH and arrhythmias in adult SCD patients. Methods: Retrospective analysis of medical records from King Abdulaziz University Hospital (KAUH) for patients with SCD aged 15 and above between 2009 and 2021. The study included 517 patients, with echocardiograms and electrocardiograms assessed according to the European Society of Cardiology/the European Respiratory Society (ESC/ERS) guidelines for categorizing PH risk (low, moderate, high) and detecting arrhythmias. Data analysis employed the Statistical Package for the Social Sciences (SPSS), utilizing quantitative and qualitative data representation. Multivariate logistic regression identified independent risk factors with odds ratios at a 95% confidence interval (CI). Results: Among participants, 50.3% were male, with a total sample average age of 34.45 ± 9.28 years. Results indicated that 1.4% of patients experienced arrhythmias, 3.7% had a moderate PH risk, and 3.3% were classified as high PH risk. Logistic regression revealed significant independent risk factors for PH and arrhythmia in patients with SCD, with chronic kidney disease (CKD) carrying the highest odds (26.4 times higher odds of PH and 15.36 times higher odds of arrhythmias). Conclusion: Patients with SCD are at risk for developing PH and various arrhythmias but are often underdiagnosed. Key risk factors for PH included CKD, liver cirrhosis, and pre-existing cardiac conditions. Arrhythmias were significantly associated with CKD and pre-existing cardiac conditions. To mitigate these risks, we recommend involving a multidisciplinary healthcare team in the care of adult patients with SCD. Future prospective studies are advised for early detection of PH and arrhythmias in hemoglobinopathy patients, potentially reducing mortality.

Clinical profile of carpal tunnel syndrome in a teaching hospital.

OBJECTIVES: The aim of this study was to review the clinical characteristics and demography of Carpel Tunnel Syndrome (CTS) cases presented to a university hospital. METHODOLOGY: A retrospective study was done for 336 consecutive patients (290 females and 46 males), referred with a clinical diagnosis of CTS to the Neuro-diagnostic laboratory at King Abdulaziz University Hospital, Jeddah, Saudi Arabia, between January 2007 and December 2010. All subjects had clinical evaluation and standardized nerve conduction studies (672 hands) performed by the same examiner. RESULTS: Carpal tunnel syndrome was confirmed in 640 hands (95.23%) with female predominance (86.3%). The mean age was 52.4 in females and 54.4 years in males with overall highest occurrence among the age group 45- < 55 years. Bilateral CTS was confirmed in the majority of the patients, i.e., 304(90.5%), and remaining were unilateral. Among unilateral pattern, 22 (68.8%) had right CTS and others had left CTS. Most of the patients were treated conservatively 85.4% while the rest had surgical decompression 14.6%. CONCLUSION: Overall predominant age group was 45-<55 years with female dominancy. Majority of subjects had bilateral CTS as well as conservative treatment was frequent.

Blastocystis sp.-An emerging cause of diarrhea post stem cell transplantation.

Key Clinical Message: Protozoans are endemic in the Middle East and diarrhea workup post stem cell transplant should always include stool examination for ova and parasites. Abstract: We present a case of Blastocystis sp. infection in a multiple myeloma patient who developed diarrhea on day five of post-autologous stem cell transplantation. Diarrhea is a major complication and a potential cause of mortality in this population. Testing for endemic protozoan is recommended.

Outcomes of Cholelithiasis Among Patients With Sickle Cell Disease: A Tertiary Care Center Experience.

BACKGROUND: Sickle cell disease (SCD) is a significant health burden in Saudi Arabia that leads to chronic hemolysis with subsequent formation of cholelithiasis. The prevalence of cholelithiasis in the Middle East varies in patients with SCD. The aim of our study was to determine the prevalence of cholelithiasis among SCD patients at a large tertiary care center, King Abdulaziz University Hospital, Jeddah, Saudi Arabia, where more than 300 patients with hemoglobinopathies were followed up. METHODS: In this cross-sectional retrospective study conducted from May 2006 to May 2022, we reviewed 414 patients with SCD who were divided into two groups according to the presence or absence of cholelithiasis. Demographic data, SCD phenotype, splenectomy, cholecystectomy, and hydroxyurea were reviewed from the patient's medical records. They were analyzed to suggest a correlation between the incidence of cholelithiasis and the chances of cholecystectomy. RESULTS: A total of 414 patients with SCD were reviewed. The mean age of participants was 31 years (10-82), with 52% male. Patients with homozygous sickle hemoglobin (HbSS) constituted 73% of the cohort. The rest (26%) had HbS/ß-thalassemia. Thirty-three patients (8%) had splenectomy done. Compliance with hydroxyurea was observed in 174 patients (42%). A total of 64.7% of patients had cholelithiasis (n=269), out of which 159 patients (59.1%) had cholecystectomy done. Surprisingly, a significant association was found between cholecystectomy and the use of hydroxyurea (p=0.003). Additionally, there was a significant association found between the development of cholelithiasis and increasing age (p=0.037). CONCLUSION: There was a high prevalence of cholelithiasis found in patients with SCD. It correlated significantly with high-age groups. Further research is warranted to confirm the relationship between hydroxyurea and cholelithiasis.

Unusual Presentation of Leukemic-Phase Mantle Cell Lymphoma: A Case Report.

Mantle cell lymphoma (MCL) is a rare subtype of B-cell lymphoma that can present in a variety of ways, including the leukemic phase, where it can occasionally be mistaken for acute leukemia due to the unusually high or rapidly growing number of leukocytes and the presence of circulating cancer cells that are morphologically similar to leukemic blasts in myeloid or acute lymphoblastic leukemia. We present the case of an 83-year-old Yemeni woman with multiple comorbidities who presented with abdominal pain and constitutional symptoms. She was found to have diffuse lymphadenopathy on clinical and radiological assessments. Her white blood cell count at presentation was 221 × 109/L with marked monocytosis (72.8%). Lymph node biopsy and bone marrow studies, including CCND1/IGH molecular studies, confirmed MCL, the pleomorphic subtype. The patient was deemed unfit for standard-of-care chemotherapy and was started on single-agent rituximab with a slow introduction to ibrutinib but succumbed to death after two weeks of ibrutinib 280 mg daily. This case serves as a reminder to keep an open mind and take into account atypical disease presentations when formulating differential diagnoses to prevent late diagnosis and any unnecessary intervention that can postpone appropriate therapy.

Antiphospholipid Antibody Testing: An Audit on Testing Practices in a Public Tertiary Care Center.

BACKGROUND: Antiphospholipid antibodies (aPLs) are antibodies directed against cell membrane components and can be associated with clinical features or be asymptomatic. Testing and interpreting these antibodies is associated with many challenges and pitfalls in clinical practice. OBJECTIVE: To review all antiphospholipid antibody testing and describe the testing practices, indications for testing and interpretation of results to infer local challenges with aPL testing and subsequently address ways to overcome those challenges. METHODS: This is a retrospective analysis of all aPL testing done in a tertiary center between 2014 and 2018. Characteristics of study patients collected through chart review were described using the mean and standard deviation for continuous variables and proportion for categorical variables. Group differences were compared between patients with any aPL-positive result and those with no positive result using chi-square or Fisher's exact test as appropriate for categorical variables and a simple regression model for numerical variables. RESULTS: Among 414 patients undergoing aPL testing, mainly adult females, 62 (14.9%) patients had at least one positive antibody, of those, 26 (42%) had repeat testing done. Testing was mostly done for obstetric indication (107, 25.8%), with 36 patients having one or two early pregnancy losses <10 weeks as their testing indication. A total of 27 (6.5%) patients were labeled with APS/possible APS based on chart review, but on review of the testing of those patients according to classification criteria, only nine patients satisfied the criteria for APS. CONCLUSION: This study highlights the clinical challenges associated with aPL testing, including the controversies around indication for testing, the low rates of repeat testing to confirm persistence, and the common misinterpretation of results. Having an aPL testing profile, explicit reference ranges, results commentary, and close interaction between ordering physicians and laboratory staff might be starting points to overcome these challenges.

Predisposing Factors and Incidence of Venous Thromboembolism among Hospitalized Patients with Sickle Cell Disease.

Though patients with sickle cell disease (SCD) are at risk of developing venous thromboembolism (VTE), clear estimates of its incidence and predisposing factors in hospitalized SCD patients are not available. Therefore, this issue was addressed to facilitate an early diagnosis and initiate appropriate prophylactic and treatment strategies. A retrospective observational study was conducted on patients with SCD who were admitted to an academic center in Saudi Arabia over a 10-year period. We identified 1054 admissions of 394 patients with SCD. Of the 3% of patients identified with VTE, 50% experienced pulmonary embolism (PE), 34.3% exhibited deep vein thrombosis (DVT), 6.3% exhibited cerebral vein thrombosis, and 9.4% showed other forms of VTE. In pregnant SCD patients, 6.4% developed a VTE event during their hospital admission. Of the risk factors, high white blood cell count, length of stay, and presence of any additional risk factor for VTE was associated significantly with higher risk of VTE. In our study, this risk seems to be much lower, which is likely attributed to the use of VTE prophylactic strategies implemented in our center. Nevertheless, further studies are needed to establish the ideal prophylactic strategy in patients with SCD.

Incidence and Risk Factors of Infections Among Diffuse Large B-cell Lymphoma and Classical Hodgkin's Lymphoma Patients in a Tertiary Care Center in Saudi Arabia: A Retrospective Cohort Study.

INTRODUCTION:  Non-Hodgkin's lymphoma (NHL) ranked fourth among all cancer types in Saudi Arabia, as reported by the Saudi Health Council in 2015. Diffuse large B-cell lymphoma (DLBCL) is the most common histological type of NHL. On the other hand, classical Hodgkin's lymphoma (cHL) ranked sixth and had a modest tendency to affect young men more frequently. Over recent decades, DLBCL patients were treated with cyclophosphamide, doxorubicin hydrochloride, oncovin, and prednisolone (CHOP) alone. Adding rituximab (R) to the standard regimen (CHOP) shows significant improvement in overall survival. However, it also has a considerable effect on the immune system, impacting complement-mediated and antibody-dependent cellular cytotoxicity and causing an immunosuppressive state through modulating T-cell immunity via neutropenia, which can let the infection spread. AIMS AND OBJECTIVES:  This study aims to evaluate the incidence and risk factors associated with infections in DLBCL patients in comparison to patients with cHL treated with doxorubicin hydrochloride (Adriamycin), bleomycin sulfate, vinblastine sulfate, and dacarbazine (ABVD). MATERIALS AND METHODS:  This study is a retrospective case-control study that included 201 patients acquired between January 1st, 2010, and January 1st, 2020. Sixty-seven patients had a diagnosis of cHL and had received ABVD, and 134 had DLBCL and had received rituximab. Clinical data were obtained from the medical records. RESULTS:  During the study period, we enrolled 201 patients, of whom 67 had cHL, and 134 had DLBCL. DLBCL patients had a higher serum lactate dehydrogenase upon diagnosis than cHL (p = 0.005). Both groups have similar response rates with complete remission/partial remission. Compared to cHL, patients with DLBCL were more likely to have advanced disease when they first presented (stage III/IV, DLBCL: 67.3 vs. cHL: 56.5; p = 0.005). DLBCL patients had an increased risk of infection as compared to cHL patients (DLBCL: 32.1 % vs. 16.4%; p = 0.02). However, patients with a poor response to treatment had an increased risk of infection compared to patients with a favorable response regardless of the type of disease (odds ratio: 4.6; p = <0.001). When using multivariate analysis, it is revealed that unfavorable therapeutic response continues to be the only predictor raising the probability of infection in the population (odds ratio: 4.2; p = 0.003). CONCLUSIONS:  Our study explored all potential risk factors for the occurrence of infection in DLBCL patients who received R-CHOP versus cHL. The most reliable predictor of an increased risk of infection during the follow-up period was having an unfavorable response to medication. To assess these results, additional prospective research is required.

Association of Genetic Variant FVIII Gene and Factor VIII: A Pilot Study Among Hemophilia A Female Relatives in Saudi Arabia.

Hemophilia A (HA) is an X-linked recessive disorder that results from mutations in the factor VIII gene (FVIII). Most affected patients are males due to the inheritance of mutations in the FVIII gene from their mothers. Females are mostly found to be carriers unless they inherited the mutation from both parents. Obligate carriers of HA are mothers whose sons are affected with HA, or daughters who inherit the mutation from their affected fathers. A possible carrier of HA could be any female who has one or more affected relatives with HA in her family. Hemophilia A carriers (HACs) could present with similar symptoms to affected patients, including low factor VIII level, and risk of bleeding especially after surgical procedures or postpartum hemorrhage. OBJECTIVES:  Assessing the phenotype of possible HAC and its association with genetic variants in the FVIII gene for better screening methods for HAC. METHODS: From the period between 25 June and 25 October 2021, the study was conducted at King Abdulaziz University Hospital in Jeddah, Saudi Arabia. We recruited seven mothers whose sons were affected with HA, and 18 possible HAC who are relatives to sever affected patients with HA. All 25 candidates were assessed for the FVIII level, activated partial thromboplastin time (APTT), and bleeding risk and sequenced a part of Exon14 in their FVIII gene. RESULTS: Twenty-five percent of the participants show a low level of FVIII, however, none of them have prolonged bleeding nor suffer from bleeding tendency. We also identified two missense variants in six of the candidates, but the clinical significance of these variants has not been determined previously. CONCLUSION: This pilot study is the first to explore the phenotype of several HAC in Saudi Arabia. A larger scale study with more HA patients and their female relatives is needed to understand the correlation between phenotype and genotype for better screening for HAC.

Primary Central Nervous System Burkitt Lymphoma, Presenting with Long-Term Fluctuating Level of Consciousness: A Case Report and Literature Review on Challenges in Diagnosis and Management.

BACKGROUND Burkitt lymphoma (BL) is an aggressive subtype of B-cell non-Hodgkin lymphoma (NHL) rarely affecting the central nervous system (CNS) as a primary disease. Over the past years, only a few cases of primary CNS Burkitt lymphoma were reported. There is a challenge in early recognition and diagnosis of this type of brain lymphoma. Furthermore, there is no specific treatment protocols for primary CNS Burkitt lymphoma, which adds to the difficulty in managing those patients. We introduce a case of a 65-year-old who presented with fluctuating memory disturbance diagnosed as cerebral Burkitt lymphoma. CASE REPORT A 65-year-old man developed a gradual decrease in his level of consciousness over a span of 4 days, associated with fluctuating memory disturbances. A CT scan showed a hyperdense mass in the region of the trigon of the left lateral ventricle and marked obstructive hydrocephalus involving the temporal, occipital horns, and the left lateral ventricle, with no evidence of other suspicious lesions. A brain biopsy of the lesion revealed features of encephalitis initially, but the patient presented later with worsening symptoms, and a repeated brain biopsy showed features of Burkett lymphoma, with normal pan-CT scan. CONCLUSIONS Primary CNS Burkitt lymphoma (PCNSBL) is a rare disease with no clear evidence in the literature of how to deal with it. Reporting such cases provides a better understanding of how to approach such unusual presentations. Treatment of PCNSBL is challenging and even with the currently adopted approaches, the disease still has a very poor outcome.

COVID-19 Vaccine Perception and Hesitancy Among Patients With Sickle Cell Disease in the Western Region of Saudi Arabia.

BACKGROUND:  In the era of the coronavirus disease-2019 (COVID-19) pandemic, the race toward shielding the public through vaccination is still going. Patients with sickle cell disease (SCD) require special consideration given their medical needs and the common side effects of immunization, affecting their decision. Therefore, we aimed to assess the perception and hesitancy toward COVID-19 vaccination in this population and explore the possible factors when it comes to vaccination decisions. METHODS:  The present cross-sectional phone interview study was conducted between May 10 and 20, 2021. The questionnaire was administered by the medical staff. The participants were all patients with SCD presented to King Abdulaziz University Hospital in Jeddah, Saudi Arabia.  Results: Out of 346 patients, 147 patients agreed to participate. Only 52 (35.37%) patients received at least one dose of the nationally available vaccines, and there were no reported serious side effects. Among the unvaccinated participants, 45 patients (47.8%) were undecided. The most reported reasons for hesitancy were the fear of developing complications as their acquaintance had and the fear of developing brain blood clots post vaccination. CONCLUSIONS:  The number of vaccinated patients with SCD was unfortunately low in our study, secondary to hesitancy. This represents a significant barrier and needs to be tackled appropriately at any proper interaction with a patient with SCD. The absence of major side effects and vaso-occlusive crises is assuring.

Low clinical utility of testing for anti-platelet factor 4 in asymptomatic individuals after ChAdOx1 nCoV-19 vaccine.

INTRODUCTION: The development of anti-platelet factor 4 (PF4) antibodies is linked to a rare thrombotic complication described now as vaccine-induced immune thrombotic thrombocytopenia (VITT). This clinical syndrome with thrombosis and thrombocytopenia was reported after exposure to the Oxford-AstraZeneca COVID-19 vaccine, ChAdOx1 nCoV-19 vaccine (AZD1222), and Ad26.COV2.S vaccine (Janssen/Johnson & Johnson). In the absence of the clinical features, the incidence of positive anti-PF4 antibodies in asymptomatic individuals post-vaccination is unclear. METHODS: The aim of this study was to evaluate the development of anti-PF4 antibodies in asymptomatic individuals 14-21 days after receiving the first dose of ChAdOx1 nCoV-19 vaccine (AZD1222) and BNT162b2 vaccine. Prospectively, we collected serum from individuals before and after ChAdOx1 nCoV-19 vaccine and BNT162b2 vaccine and measured anti-PF4 antibodies using the Asserachrom HPIA IgG ELISA (Stago, Asnieres, France). RESULTS: We detected positive anti-PF4 antibodies in 5 of 94 asymptomatic individuals post-vaccine with a rate of 5.3% with low titers (OD 0.3-0.7). Four of 5 individuals who tested positive after the vaccine had also positive anti-PF4 antibodies before the vaccine, which indicates that a majority of the positive results are due to preexisting anti-PF4 antibodies. We did not find a relation between the development of anti-PF4 antibodies and the immune response to the vaccine, status of prior COVID-19 infection, and baseline characteristics of participants. None of the participants developed thrombosis nor thrombocytopenia. CONCLUSION: Our results provide new evidence to guide the diagnostic algorithm of suspected cases of VITT. In the absence of thrombosis and thrombocytopenia, there is a low utility of testing for anti-PF4 antibodies.

Immunogenicity of The BNT162b2 COVID-19 mRNA and ChAdOx1 nCoV-19 Vaccines in Patients with Hemoglobinopathies.

INTRODUCTION: Studies assessing immune responses following Pfizer-BioNTech BNT162b2 mRNA COVID-19 (Pfizer) and ChAdOx1 nCoV-19 AZD1222 (AstraZeneca) vaccines in patients with hemoglobinopathy are non-existent in the literature despite being thought at high risk of infection. METHODS: Prospectively, we collected serum from patients with hemoglobinopathies at least 14 days post vaccine and measured neutralizing antibodies (nAb) in addition to binding antibodies using in-house assays. RESULTS: All 66 participants mounted a significant binding antibody response (100%), but nAbs were detected in (56/66) post-vaccine with a rate of 84.5%. Age, gender, vaccine type, spleen status, hydroxyurea use, and hyperferritinemia did not affect the rate significantly. While 23/32 (71.8%) patients receiving only one dose of the vaccine were able to mount a positive response, 33/34 (97.05%) of those who had two doses of any vaccine type had a significant nAbs response. Patients who had anti-nucleocapsid (N), signifying asymptomatic infection in the past, were able to produce nAbs (31/31). No nAbs were detected in 10/35 (28.5%) patients with no anti-N antibodies. CONCLUSION: Our results provide supportive data when advising patients with hemoglobinopathy to receive COVID-19 vaccines and ensure booster doses are available for better immunity. Whenever available, measurement of nAb is recommended.

Does Up-front Autologous Stem-Cell Transplantation at First Relapse Improve Outcome in Transplant-Eligible Follicular Lymphoma Patients Whose Disease Relapses Within 24 Months?

BACKGROUND: In Canadian adults, follicular lymphoma (FL) is the most common subtype of non-Hodgkin lymphomas. Approximately 20% of patients with FL experience progression of disease within 2 years of first-line chemoimmunotherapy. Those patients have an expected overall survival of less than 5 years. The optimal second-line treatment for these high-risk patients is unclear. PATIENTS AND METHODS: We analyzed data from the Blood and Bone Marrow Transplantation Center at Ottawa Hospital to determine whether autologous stem-cell transplantation as up-front therapy for first relapse can improve outcomes in this high-risk FL subgroup. We identified 17 patients who underwent up-front autologous stem-cell transplantation between February 2012 and February 2019. RESULTS: The disease of all patients had relapsed within 24 months after receipt of their first rituximab-based chemotherapy. Overall survival at 2 and 5 years was 86.2% (95% confidence interval [CI], 55-96) and 71.8% (95% CI, 31-91), respectively. The progression-free survival at 2 and 5 years was 62.6% (95% CI, 35-81) and 53.6% (95% CI, 25-75), respectively. CONCLUSION: Overall survival is improved when receiving autologous hematopoietic stem-cell transplantation as up-front therapy at first relapse in transplant-eligible FL whose disease relapses within 24 months of first-line therapy. Data from our single center look promising, but the data need to be replicated with a larger sample size.