Dr. Saara Kaviany and Dr. Blachy J. Dávila Saldaña discuss treating HLH patients, focusing on controlling inflammation, hematopoietic stem cell transplants, and the importance of early recognition and targeted therapies for improved outcomes.
Controlling inflammation is crucial for minimizing organ damage in HLH patients and improving treatment success post-transplant.
Advancements in targeted therapies for HLH aim to reduce broad-acting treatment toxicity while enhancing efficacy and patient outcomes.
Deep dives
Importance of Controlling Inflammation in HLH Treatment
The primary goal of treating HLH is to subdue life-threatening inflammation that drives the disease. By controlling inflammation, organ damage can be minimized, improving the patient's overall condition and enhancing the success of subsequent treatments like stem cell transplants. Controlling inflammation is crucial for long-term outcomes, including reducing the risk of organ damage and infections.
Hematopoietic Stem Cell Transplant (HSCT) in HLH Treatment
HSCT aims to replace a defective immune system in HLH patients by introducing donor cells to create a new functional immune system. The process involves chemotherapy to prepare the body for transplant, where donor cells find their place and start growing. While the transplant process is life-changing, it requires careful post-transplant care and long-term immune system management, akin to starting anew with a newborn baby's immune system.
Side Effects of Broad-spectrum Therapies in HLH Treatment
Traditional treatments like steroids and atoposide, while effective in controlling HLH inflammation, can cause significant toxicity, including liver damage and severe cytopenia. Prolonged use of these medications can lead to transfusion dependency and increased infection risks. Providers aim to minimize such toxicities and transition towards targeted therapies to achieve better outcomes.
Targeted Therapies for HLH Treatment
Advancements in understanding HLH mechanisms have led to a shift towards targeted therapies that address specific inflammatory pathways, reducing the need for broad-acting treatments that may cause substantial toxicity. By focusing on dampening harmful cytokine-mediated processes, healthcare providers can potentially improve treatment efficacy while minimizing overall treatment-related toxicity and enhancing patient outcomes.
Adam Narloch is joined by Dr. Saara Kaviany and Dr. Blachy J. Dávila Saldaña for a discussion about treating patients with HLH. Dr Kaviany is a pediatric hematologist-oncologist and an assistant professor of pediatrics at University of Chicago Medicine. Dr Saldaña is also a pediatric hematologist-oncologist and an associate professor of pediatrics at George Washington University. Hear from these 2 experts about the overall treatment goals and strategies for patients with HLH.