Acute myeloid leukemia presenting with hepatic dysfunction: Should induction be dose reduced?

The combination of de novo hepatic dysfunction and AML in a medically fit patient presents an unusual predicament. Cases present with obstructive jaundice and imaging typically shows diffuse hepatosplenomegaly, with some cases visualizing myeloid sarcomas causing biliary ductal dilatation. Guidelines for use of anthracyclines in hepatic dysfunction recommend dose reduction based on bilirubin blood levels, either to 50% or even omitting anthracycline.

Genomic Deletion of PFKFB3 Decreases In Vivo Tumorigenesis.

Rapidly proliferative processes in mammalian tissues including tumorigenesis and embryogenesis rely on the glycolytic pathway for energy and biosynthetic precursors. The enzyme 6-phosphofructo-2-kinase/fructose-2,6-bisphosphatase-3 (PFKFB3) plays an important regulatory role in glycolysis by activating the key rate-limiting glycolytic enzyme, 6-phosphofructo-1-kinase (PFK-1). We have previously determined that decreased PFKFB3 expression reduced glycolysis and growth in transformed cells in vitro and suppressed xenograft growth in vivo.

Emicizumab for acquired hemophilia A: Report of two cases and dosing strategies.

Acquired hemophilia A (AHA) is a rare autoimmune bleeding disorder caused by autoantibodies against FVIII. Severe AHA is life-threatening. Currently, licensed hemostatic agents for the treatment of severe AHA have short half-lives and require intravenous administration, leading to a need for hospitalization, higher costs, and negative effects on quality of life.

Clinical Utility of Neutrophil to Lymphocyte Ratio in Sickle Cell Disease With Vaso-Occlusive Crisis.

Background And Objectives: The neutrophil-to-lymphocyte ratio represents a universally accessible value that correlates with inflammation and prognosis in several disease states; however, the role of this biomarker in sickle cell disease remains poorly explored. Hence, the objective of the present study was to determine its potential clinical utility in patients with sickle cell disease.

Patients: Herein, we retrospectively reviewed 143 patients with sickle cell disease who presented to the emergency department with fever and painful vaso-occlusive crisis.

Coagulopathy following Crotaliπae snakebites in northeast Florida.

Effects of Crotalinae envenomation vary by geographical areas and research into coagulopathy and effects of antivenom are needed to optimize management. This was a single-center retrospective review with testing on presentation and 4 h after; antivenom administration was noted and data analyzed overall and comparing envenomations. One hundred and nineteen snakebites evaluated with 59 identified as Crotalinae and half receiving antivenom.

Procalcitonin as a diagnostic marker for infection in sickle cell disease.

Background: Patients with sickle cell disease (SCD) are at increased risks of infection. Fever often occurs with vaso-occlusive crisis (VOC), posing a diagnostic challenge in SCD. Procalcitonin (PCT) is an infectious biomarker validated in the general population but with limited data on use in SCD.

Sinonasal renal cell-like adenocarcinoma arising in von Hippel Lindau (VHL) syndrome.

Sinonasal renal cell-like adenocarcinoma (SNRCLA) is a rare and relatively novel diagnosis. Hereditary and somatic genomic signatures are not well defined in this disease. We report the case of a 35-year-old African-American male with von Hippel Lindau (VHL) syndrome who developed SNRCLA.

Novel splicing (c.6529-1G>T) and missense (c.1667G>A) mutations causing factor V deficiency.

Congenital factor V deficiency (FVD) is a rare bleeding disorder. In this study, we investigated the genetic basis in an African American patient with factor V activity 3%. Custom sequence capture and targeted next-generation (NGS) sequencing of the F5 gene were undertaken followed by PCR and Sanger sequencing.

Timely Delivery of Discharge Medications to Patients' Bedsides: A Patient-centered Quality Improvement Project.

Introduction: Patients who are unable to fill prescriptions after discharge are at risk of hospital readmission. Ensuring that patients have prescriptions in hand at the time of discharge is a critical component of a safe and effective discharge process. Using a "Meds to Beds" program, we aimed to increase the percentage of patients discharged from Holtz Children's Hospital with medications in hand from 49% to 80%, reduce turnaround time (TAT) from electronic prescription signature to bedside delivery from 4.

Anti-PF4/heparin antibodies are increased in hospitalized patients with bacterial sepsis.

Heparin-induced thrombocytopenia (HIT) is caused by antibodies targeting platelet factor 4 (PF4)/heparin complexes. The immune response leading to HIT remains perplexing with many paradoxes. Unlike other drug induced reactions, anti-PF4/heparin antibody generation does not follow the classic immunologic response.

Temporality of heparin-induced antibodies: a retrospective study in outpatients undergoing hemodialysis on unfractionated heparin.

Background: Heparin-induced antibodies (HIA) are responsible for causing heparin-induced thrombocytopenia and thrombosis. Research has shown that the temporality of heparin-induced antibodies does not follow the classic immunologic response. The immunobiology of HIA generation remains unclear with varying in vitro and in vivo data.

Acute glomerulonephritis secondary to .

is a clinically important pathogen that is emerging globally but remains poorly investigated. Here, we report the first case of acute glomerulonephritis resulting from infection with Glomerulonephritis is typically caused by and reports secondary to other strains including and exist. Infection with in this patient was associated with acute nephritis (haematuria, oedema and hypertension), nephrotic syndrome and progressive azotemia.

Severe carvedilol toxicity without overdose - caution in cirrhosis.

Background: Carvedilol is used in the management of hypertension, ischemic heart disease, heart failure and most recently, portal hypertension. It has been associated with improved outcomes regarding variceal bleeding, hepatic decompensation and death when compared to propranolol and endoscopic band ligation. The main cause of portal hypertension is cirrhosis and therefore carvedilol is increasingly used in these patients.