Pediatric Philadelphia-Negative Myeloproliferative Neoplasms in the Era of WHO Classification: A Systematic Review.

BACKGROUND: Philadelphia-negative myeloproliferative neoplasms (MPN) are most prevalent in the older population (median age at the diagnosis is above 60 years) and rarely diagnosed in pediatrics. Thus, our knowledge about the clinical presentation, mutational status, and complications of MPNs in pediatrics is limited. METHODS: The literature in English (PubMed, SCOPUS, and Google Scholar) was searched for studies, reviews, case series, and case reports of patients with Philadelphia-negative MPNs (including essential thrombocythemia, polycythemia vera, primary myelofibrosis, and profibrotic myelofibrosis) in the pediatrics age group (less than 18 years). Only studies that fulfilled WHO 2008 or 2016 criteria for MPNs were included. We aimed to describe the clinical characteristics, vascular and long-term complications, types of driver mutations, and treatment approaches in pediatric patients with MPNs. RESULTS: We reviewed 33 articles of available published literature from 2008 to 2022 and collected data from a total of 196 patients of the pediatric population. Among the cohort of patients, 139 had essential thrombocythemia (ET), 20 had polycythemia vera (PV), and 37 had primary myelofibrosis (PMF). The median age at the time of diagnosis for each disease varied, with 8.8 years for ET, 10 years for PV, and 3.6 years for MF. There was a slight difference in gender prevalence between both gender groups and all three diseases. The presenting symptoms were not mentioned in more than 50% of studies. We found that JAK2 was the most prevalent among all mutations. Both bleeding and thrombosis were present equally in ET, with 9% of cases complicated by bleeding and 9% complicated by thrombosis. Hemorrhagic events did not occur in patients with PV; thrombosis in children with MF was also not found. The progression into AML occurred in two patients with PV and one with ET. CONCLUSION: Given the rarity of MPNs in pediatrics and their different characteristics compared with adults, we believe there is a need for unique diagnostic criteria to match the different molecular statuses in pediatrics. Based on our review, the incidence of MPN complications in pediatrics, including thrombotic events, hemorrhage, and leukemic transformation, differs from that in adults.

Hematologic Outcomes of COVID-19 Patients with and without G6PD Deficiency: A Comparative Study.

INTRODUCTION: Patients with multiple comorbidities who have coronavirus disease 2019 (COVID-19) have high morbidity and mortality. Glucose-6-phosphate dehydrogenase (G6PD) deficiency has been shown to have an enhanced effect on coronavirus in an earlier study. METHODS: We conducted this comparative observational study to evaluate the effects of COVID-19 disease on G6PD deficiency based on the hematologic parameters, COVID-19-related hospitalizations, and mortality in the state of Qatar between January 2020 and May 2020 at four designated COVID-19 facilities. We identified 41 patients with G6PD deficiency who had documented COVID-19 infection. We compared the results with 241 patients with COVID-19 infection who tested negative for G6PD deficiency.: Results: Comparing the COVID-19 positive G6PD deficient with COVID-19 positive G6PD normal activity showed that G6PD normal group had higher white blood cell count (WBC), absolute neutrophil count (ANC), lymphocytes, eosinophils, and monocytes counts versus the G6PD deficient group (p < 0.001). CONCLUSIONS: When compared with COVID-19 patients with normal G6PD, patients with COVID-19 infection and G6PD deficiency had lower total WBC, ANC, lymphocyte, monocyte, and eosinophil counts. However, no evidence of increased hemolysis, thrombosis, morbidity, or mortality was observed in COVID-19 patients with G6PD deficiency.

Endomyocardial fibrosis in a non-tropical patient who presented with chest pain mimicking ACS and left ventricular thrombus, case report.

Endomyocardial fibrosis (EMF) is a disease known to cause restrictive cardiomyopathy. It shows a high prevalence in tropical countries. Several triggering factors have been proposed. However, the pathogenesis is still a mystery. The disease is progressive, and the outcome is generally unfavorable. The most common symptom is heart failure. However, an atypical presentation may be expected. Our case presented with symptoms suggestive of ischemia and missed diagnosis initially as ischemic cardiomyopathy. This report aims to increase the attention and awareness of this disease. We present a case of a 53-year-old man referred to the emergency department for sudden chest pain, left-sided and non-radiating lasted for several minutes, awoke him from sleep with no associated symptoms. He is known to have Diabetes type-2 and hypertension on oral therapy. Cardiac markers were within the normal limit. The patient was discharged home with an appointment at the cardiology outpatient clinic. Echocardiography was done and revealed mildly reduced left ventricular (LV) systolic function, Ejection Fraction of 46%, asymmetric LV hypertrophy affecting the apical segments with aneurysm, and calcified apical thrombus. CT coronary angiography was done with non-significant Left Anterior Descending artery lesions and left ventricular hypertrophy affecting the apex with calcified apical thrombus. Further investigation by cardiac MRI revealed apical thrombus and late apical uptake suggesting Endomyocardial Fibrosis of possible eosinophilic etiology. The patient continued to have attacks of similar chest pain, for which stress cardiac MRI was done and was negative for ischemia. Another diagnostic workup was done, including hematological and serological tests such as Antinuclear Antibodies and Schistosoma Antibodies. The patient was kept on valsartan and Bisoprolol with oral anticoagulant (vitamin K antagonist) and Rosuvastatin. EMF may have a heterogeneous presentation and should be considered in a patient with calcific apical thrombus without previous history of cardiac problem, even in the non-tropical region.

Dasatinib-Induced Pleural and Pericardial Effusions.

Chronic myeloid leukemia (CML) is a myeloproliferative disease associated with the Philadelphia chromosome and BCR-ABL1 fusion gene. Tyrosine kinase inhibitors (TKI) are now the standard therapy for this condition. Among the approved TKIs for CML is dasatinib. We present a case of a 58-year-old Egyptian male who developed bilateral pleural (grade II) as well as pericardial effusions (grade II) secondary to dasatinib 100 mg once-daily dosing. He was managed by interrupting dasatinib and introducing diuretics and steroids. The objective is to raise awareness about this unfavorable effect as it may affect the patient's quality of life and increase rates of treatment withdrawal.