As EM physicians, we are taught how to manage and treat many serious and life threatening conditions, most of them we know like the back of our hands, and some we look up on shift. The most important things to know are the “think on your feet” situations, when patients are actively trying to die in front of you, and you don’t have the time to look things up. We all know how to manage a bleed, whether internal or external, but do you know how to treat bleeding in a Hemophilia patient?! I didn’t until April, 2018 when my newborn son was diagnosed with Hemophilia A. In this segment, I will explain and simplify hemophilia for you down to the “need to know” essential information so when you are managing a hemophilia patient in the ED, like my son, you will know what to do. ...Read More
What is it HLH?
Hemophagocytic Lymphohistiocytosis (HLH) is a rare and often fatal syndrome of uncontrolled and ineffective inflammatory response to a certain trigger. It is characterized by excessive proliferation of lymphocytes and macrophages (histiocytes), hence the name “lymphohistiocytosis”. This results in the overproduction of cytokines, responsible for many of the clinical features present in this syndrome.
Familial, or genetic, HLH occurs as a result of a genetic mutation leading to impaired cytotoxic function. There have been several genetic mutations indicated in the development of HLH, including an association with congenital immunodeficiency syndromes, such as Chediak-Higashi, Griscelli and X-Linked Lymphoproliferative Syndromes. This form most often occurs within the first year of life (median age 8 months), with the majority of pediatric cases occurring <2 years of age, but can range from infancy to adulthood.
Acquired HLH occurs in the setting of an underlying condition, such as immunodeficiency, malignancy, or autoimmune disease. When HLH is secondary to a predisposing autoimmune disease, it is referred to as macrophage activating syndrome (MAS). Acquired HLH is the most common cause of this syndrome in adults, but this form can be seen in all ages. Overall, the syndrome is most often triggered by an infectious agent in an otherwise healthy person....Read More
Background: Factor Xa inhibitors have gained more use over the past several years due to the ease of administration and easier monitoring. However, bleeding, namely intracranial hemorrhage (ICH) is still a risk and the lack of a proven antidote is a cause for concern. Guidelines for the treatment of ICH published in 2016 recommended the administration of prothrombin complex concentrates (PCCs, both activated [aPCC] and 4 factor) . In 2018 andexanet alfa gained accelerated approval by the FDA for the reversal of factor Xa inhibitors. Despite this new antidote, many organizations such as the American Society of Hematology and the European Stroke Organization still recommend the use of PCCs. Research thus far has been performed in healthy volunteers, or small (<100 total patients with ICH) trials leaving a gap in the literature of what agent to use at the bedside....Read More