Currative and Palliative Interface in Familial Hemophagocytic Lymphohistiocytosis: A Retrospective Study in IIIrd Pediatrics Clinic - EAP 2021 Virtual Congress and MasterCourse

E. Boeriu ^1,2 S. Arghirescu ^1,2 P. Urtila ^1,2 D. File ¹ A. I. Boeriu ³ L. Valumareanu ¹ M. Lelik ¹ A. Chirita-Emandi ^1,2 M. Bataneant ^1,2

¹IIIrd Pediatric Clinic, Oncology and Hematology Department, Children’s Emergency Clinical Hospital “Louis Țurcanu” Timisoara, Timisoara, Romania
²Pediatrics, University of Medicine and Pharmacy „V. Babes” Timisoara, Timisoara, Romania
³LMU, University „Loudwig Maximillian” Munchen, Munchen, Germany

Introduction: Familial hemophagocytic lymphohistiocytosis (FHL) are defined as genetically dysfunctions characterized by hyper-inflammation caused by the proliferation and activation of lymphocytes and macrophages, and depending on the genetic defect, they are classified in various clinical forms. Although allogeneic transplantation is required as soon as possible, the unfavorable evolution motivates the palliative approach.

Objectives: We proposed a retrospective analysis of FHL cases, 7 cases were included in the study, 2 cases of HCS, 1 case of FHL type V, 4 cases of GS, during a period of 4 years respectively 2 years regarding complex curative and palliative medical approach, analyzing the challenges of medical staff and families until the end of life.

Material and methods: These cases were selected from the oncological and hematological pathology of the IIIrd Pediatric Clinic between 2009-2019. The data were analyzed to allow the diagnostic classification and the evolution until the moment of death, as well as the psycho-emotional aspects and support of the family.

Results: In HCS, the diagnosis was established early in the first months of life, while in GS the diagnosis was established after the age of 1 month and after 1 year of life respectively. Clinical simptomatology added to progressive neurological degradation required palliative care. Allogeneic transplantation was performed in a single case of GS with good subsequent evolution. Concerning HCS, curative therapy was attempted in one case, according to the HLH Protocol, with partial response and evolution towards death. In other case of HCS, only palliative care was performed. The presence in the same family of HCS at an interval of 8 years and for GS at an interval of 2 years made the approach of the family more challenging.

Conclusions: FHL are rare diseases and the limits of curative therapy require a palliative approach from the beginning of the disease.