Blog

At the age of 4 months, Nikolas Kontoyannis was diagnosed with histiocytosis-x (now known as Langerhans cell histiocytosis or LCH). Nikolas’ journey was very uncertain and faced many challenges, including central nervous system involvement which still impacts Nikolas today. His journey is the source of inspiration for continued research and an annual Symposium held in Athens for the last 33 years.  

Born out of the need for research into Langerhans cell histiocytosis (LCH) and through the impact of the Kontoyannis family’s own experience, the Nikolas Symposium has been the leading scientific think-tank focused on histiocytosis since 1989.  

“Established in 1989 and held annually in Greece, the Symposium welcomes scientists, researchers and medical professionals from all over the world to examine, deliberate and debate the pathogenesis and structure of the diseases. The goal of the Nikolas Symposium is to shed light on and spread awareness of these little-known diseases by engaging more frequently with patients, promoting a thorough understanding of the condition and ultimately aspiring to finding a cure. Each year, a steering committee composed of leading professionals proposes a particular scientific topic of relevance to the diseases to be discussed that year. The committee invites prominent experts to attend the Symposium, fostering a lively and informative debate over a three-day period. Clinicians, doctors and scientists from all over the world gather to discuss the relationship between the chosen topic for that year and the cause of Histiocytic diseases. Moreover, they get the chance to meet with patients and their families, an experience that not many medical practitioners are exposed to before the Symposium.” –https://www.niksym.org/  

The Histiocytosis Association has had the honor of attending the Symposium to offer additional patient-perspective and to help the Steering Committee with planning. This year’s Nikolas Symposium was focused on the topic of “Tissue and Cellular Metabolism and the Langerhans cell histiocytosis lesion”. Speakers from outside the histiocytosis clinical and scientific field were brought in to give presentations on sub-topics related to the main topic of tissue and cellular metabolism. Presentations on LCH and histiocytic neoplasms were given at the start of the meeting, to ensure those who were outside the histiocytosis field could begin to think about how their work could impact histiocytosis research.  

The energy was electric, with all the guests highly engaged in the presentations given by others, and the coffee breaks were filled with debate and knowledge-sharing, the benefit of the symposium’s format.  While the days were filled with science, professional connections were also made which will lead to partnerships in the future of research and patient support. 

The Histiocytosis Association was also thrilled for the inclusion of Cihan Atila, MD in this year’s program. Dr. Atila is a Doctor of Medicine in Endocrinology, diabetes, and metabolism at the University Hospital in Basal Switzerland. He has been instrumental in providing resources on AVP-D (arginine-vasopressin deficiency, formerly diabetes insipidus) for the histiocytosis community and is spearheading the development of clinical guidelines for AVP-D. We are truly grateful for his partnership and leadership in research on this condition.  

The main takeaways from this year’s symposium are as follows:  

• There were three main themes that emerged from the discussions – pathophysiology, immunometabolism, and immune environment and conversation arose focused on how they all interact with one another.  

• There were two Pritchard Scholars who received a scholarship award as young scientists, interested in histiocytic disorders. They spoke on mouse modeling for LCH and germline genetics.  

• Cihan Atila, MD, spoke on pituitary function; one of the key observations that was noted was individuals with AVP-D can have little oxytocin production which could explain the psychosocial issues and feelings of happiness that sometimes accompany histiocytic neoplasms. He also spoke about the risk of hypernatremia/hyponatremia (water balance) and the histiocytosis scientists were interested in exploring this as it is not good for neurons.  

• There was a presentation that provided a bird’s eye view of all the immune states that could exist in immune environment, as well as discussion on a concept of understanding these immune states and using various treatments to move an individual along to remission.  

• Some of the conversation opened eyes to new potential pathways that might be responsive to treatment for LCH, meaning that the number of available targeted therapies (inhibitors) for histiocytosis could potentially grow 

• Along this line, the group learned about new potential therapeutic drugs that targeted metabolites, which are a crucial signaling molecule and could be something to target inside of LCH.  

• Pavan Reddy, MD, gave a talk on the regulation of inflammation by dietary modification  

• There are many new discoveries and technologies that are helping research findings translate rapidly into the clinic in the form of clinical trials/treatments.  

Overall, it was an incredible meeting and over the course of the next year, the hope is that many if not all the participants will stay connected to explore new ideas, generate new research, and help us get closer to the cure.  

With tremendous gratitude for the Kontoyannis family, the Steering Commitee, and all those in attendance, thank you for driving forward momentum and science. We look forward to learning from future Symposia and helping in any way we can.  

Deanna Fournier & the Histiocytosis Association Team