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.

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.

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.

Rare Presentation of FLT3-ITD-Positive Acute Myeloid Leukemia With Monocytic Differentiation: A Case Report.

Acute myeloid leukemia (AML) is a hematological malignancy that affects adults and has various presenting symptoms, the most common being shortness of breath, bleeding, and infection. Thrombosis is also believed to be a rare presenting symptom of AML; however, information about the association between AML and thrombosis is scarce. Here, we report the case of a 27-year-old female who presented with extensive coagulation disturbances leading to various thromboembolic complications (including multiple strokes and renal and splenic infarcts) and was eventually diagnosed with AML. Owing to the patient's functional status at diagnosis, chemotherapy induction was withheld, and close observation along with supportive treatment was initiated. The findings, in this case, provide useful information on the presentation of such unusual cases, so we aim to enrich and contribute to medical evolution.

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.

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.

The Prevalence of Depression and Anxiety Among Sickle Cell Disease Patients in King Abdulaziz University Hospital.

Background Sickle cell disease (SCD) is a recessive hereditary condition. The physical changes caused by SCD affect the quality of life (QoL) by negatively impacting psychological aspects. Objective This study aimed to assess the prevalence of depression and anxiety in SCD patients based on different sociodemographic characteristics in Jeddah, Saudi Arabia. Method A cross-sectional study was conducted at King Abdulaziz University Hospital (KAUH) in Jeddah from 13 July to 30 August 2021. The included patients were 18 years of age and above and affected with sickle cell disease. Medical staff interviewed the patients and filled the Patient Health Questionnaire (PHQ)-9 and General Anxiety Disorder (GAD)-7. Result One hundred nineteen (119) patients were included in this study. The median age of participants was 32 and mostly male (n=72, 60.5%). The rate of depression was 45.4%. On the other hand, the rate of anxiety was 22.7%. The median of the PHQ-9 score was 8±8 while the median of the GAD-7 score was 5±8. Moreover, the study showed that anxiety and depression in relation to sociodemographics were higher in the patient age groups of 30-34 years old, male, single, unemployed, and with higher education. There was a significant association between depression rate and the two variables: patient employment status (49.3%; p=0.047) and a family history of SCD (51%). Conclusion Depression in patients with sickle cell disease is prevalent and correlated to demographic and social factors.

Response to Hydroxyurea in a Patient With Sickle Cell Hepatopathy: A Case Report.

Sickle cell hepatopathy is an underreported entity lacking clear management guidelines. This case highlights the potential role of hydroxyurea (HU) in improving the hepatic dysfunction seen among patients with sickle cell disease (SCD). We herein present the clinical course of a patient prior to and after the initiation of hydroxyurea with an emphasis on long-term outcomes and the patterns of liver injury over a 15-year time course.

Incidence and outcomes of antenatally detected congenital hydronephrosis.

BACKGROUND AND OBJECTIVES: Antenatally detected urinary tract abnormalities (ADUTA) are increasingly recognized. Our aims were to determine the incidence and outcomes of antenatally diagnosed congenital hydronephrosis in a large cohort. DESIGN AND SETTINGS: We recorded the number of total deliveries over 4 years at King Abdulaziz University Hospital (KAUH) between January 2008 and December 2011 from the number of nursery and neonatal intensive care unit (NICU) admissions. PATIENTS AND METHODS: We reviewed the records of 18 853 deliveries between January 2008 and December 2011 at KAUH, Saudi Arabia. ADUTA were recorded, and their postnatal medical records were reviewed for demographic and radiological data. RESULTS: ADUTA were diagnosed in 327 fetuses (1.7%). The commonest pathology was congenital hydronephrosis (n=313, 95.7%). Cystic renal anomalies were reported in 4 babies (1.2%), and 10 children (3.1%) were reported to have other renal anomalies, including duplex kidneys or a single kidney. A total of 240 babies with congenital hydronephrosis were followed up. Hydronephrosis resolved in 99 children (41.2%) within 2 months of birth. A total of 29 subjects had underlying renal anomalies (12.1%), including vesicoureteral reflux (n=12, 5%), pelvi-ureteric junction obstruction (n=14, 5.8%), and posterior urethral valve (n=3, 1.3%). The best predictor for nonresolving congenital hydronephrosis and underlying anatomical abnormalities was the anteroposterior diameter on the first postnatal scan. A cut-off point of 5 mm was found to be 83% sensitive in predicting nonresolving hydronephrosis, while 7 mm was 88% sensitive and 10 mm was 94% sensitive. CONCLUSIONS: Congenital hydronephrosis is the commonest ADUTA. A large percentage resolved within 2 months of birth, but underlying anatomical abnormalities were found in 12.1%. All babies with antenatally detected hydronephrosis should be examined by ultrasound postnatally but further radiological investigations should only be performed for persistent significant AP dilatation >=10 mm.