What are the causes of HLH syndrome

Hemophagocytosis Syndrome (Hemophagocytic Lymphohistiocytosis, HLH)

This text provides information on the clinical picture of hemophagocytic syndrome (hemophagocytic lymphohistiocytosis, HLH), its frequency, causes, symptoms, diagnosis, treatment and prognosis.

Author: Prof. Dr. med. S. Ehl, Henrike Ritterbusch, Julia Dobke, created on: 25.09.2019, editing: Ingrid Gr√ľneberg, approval:, Prof. Dr. med. U. Creutzig, last changed: January 31, 2020

Notes on the text

The following information text is aimed at patients who are affected by haemophagocytosis syndrome and their relatives, friends, teachers and other caregivers as well as the interested public. It is intended to contribute to a better understanding of this disease, the options for treating it and the problems and special needs of the patients concerned.

Our information does not replace the necessary clarifying discussions with the treating physicians and other employees of the treatment team; however, they can help to prepare these conversations and to understand them better.

The information contained in this patient text has been created primarily on the basis of the literature given below and taking into account the current guidelines for the treatment of children and adolescents with haemophagocytosis syndrome. The text was checked by the editors specified above in August 2019 and approved for posting on the Internet for a period of five years. It should be checked and updated again at the latest after this period has expired.

Please note that the following are general information and recommendations that - given the complex situation - do not necessarily apply to every patient in their entirety. Many therapy recommendations have to be decided on an individual basis and on an interdisciplinary basis. Your treatment team will inform you of the measures that apply to you.

Basic literature

  1. Lehmberg K, Ehl S: Haemophagocytic Lymphohistiocytosis. Pediatric hematology and oncology, eds: A. Eggert, C. Niemeyer, Springer Verlag 2nd edition 2018 [ISSN: 978-3-662-43686-8] LEH2018
  2. Pachlopnik Schmid, J: Hemophagocytosis Syndrome. PAEDIATRICA 2011, Vol. 22 No. 4, p.13 PAC2011
  3. Ritterbusch H, Ehl, S: Hemophagocytosis Syndrome. Information sheets on primary immunodeficiency RIT2009