The American Society of Hematology (ASH) released new guidelines on Apr. 1 for the diagnosis and management of severe acquired aplastic anemia, a rare and life-threatening bone marrow failure disorder. The guidelines were developed by a multidisciplinary expert panel, including a patient with lived experience, and are published in Blood Advances.
The updated recommendations are intended to provide evidence-based best practices to improve outcomes for people living with severe acquired aplastic anemia. This disorder causes the bone marrow to stop producing enough blood cells, leading to fatigue, increased risk of infections, and dangerous bleeding episodes. It affects one to two million people each year in western countries and has higher incidence rates in Asia.
Robert Negrin, MD, president of ASH, said: "The Society is proud to provide this robust and much-needed resource for clinicians caring for individuals with aplastic anemia. These guidelines offer important consensus for the field while helping raise awareness of a serious yet often overlooked disorder."
Most cases of aplastic anemia are immune-mediated or idiopathic but can also be triggered by infections, medications, toxic substances, chemicals or other environmental exposures. The guidelines focus on severe cases characterized by very low counts of neutrophils (less than 500 per microliter), reticulocytes (less than 60,000 per microliter), and platelets (less than 20,000 per microliter).
The document outlines 33 specific recommendations as well as four good practice statements aimed at improving diagnosis and treatment in both children and adults with severe acquired aplastic anemia. Emphasis is placed on shared decision making between patients and providers as well as the need for further research.
Phil Scheinberg, MD—head of hematology at Hospital Beneficência Portuguesa Hospital in São Paulo and chair of the ASH guideline panel—said: "It's critical to definitively diagnose individuals with aplastic anemia to ensure that they are promptly connected to appropriate treatment. We hope these guidelines will not only improve patient outcomes but also expand access to diagnostic testing and catalyze broader global access to transplant and immunosuppressive therapies."
