Learn About Hemophagocytic Lymphohistiocytosis

What is the definition of Hemophagocytic Lymphohistiocytosis?
Hemophagocytic lymphohistiocytosis (HLH) is a condition in which the body makes too many activated immune cells (macrophages and lymphocytes). Symptoms may include fever, enlarged liver or spleen, cytopenia (decreased number of blood cells), and neurological abnormalities. HLH may be inherited in an autosomal recessive manner or it can have non-genetic causes in which case it is called acquired HLH. Each subtype is caused by a change in a different gene. The genetic cause of type 1 is currently unknown. Types 2-5 are caused by genetic changes in the PRF1 gene, the UNC13D gene, the STX11 gene and the STXBP2 gene, respectively. When HLH results from an inappropriate immune response to Epstein-Barr virus or another viral illness, it may be due to a separate genetic condition called X-linked lymphoproliferative disease (XLP). XLP is caused by a genetic change in the SH2D1A or XIAP gene and is inherited in an X-linked manner.
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What are the alternative names for Hemophagocytic Lymphohistiocytosis?
  • Familial hemophagocytic lymphohistiocytosis
  • FHL
  • Familial HLH
  • Familial erythrophagocytic lymphohistiocytosis
  • Familial histiocytic reticulosis
  • HLH
Who are the top Hemophagocytic Lymphohistiocytosis Local Doctors?
KL
Kai Lehmberg
Elite
Highly rated in
12
conditions

Universitätsklinikum Eppendorf

Hamburg, DE 

Kai Lehmberg practices in Hamburg, Germany. Lehmberg is rated as an Elite expert by MediFind in the treatment of Hemophagocytic Lymphohistiocytosis. They are also highly rated in 12 other conditions, according to our data. Their top areas of expertise are Hemophagocytic Lymphohistiocytosis, Non-Langerhans-Cell Histiocytosis, Reticulohistiocytoma, Bone Marrow Aspiration, and Bone Marrow Transplant.

YW
Yini F. Wang
Elite
Highly rated in
12
conditions

Beijing Friendship Hospital, Capital Medical University

Beijing, CN 

Yini Wang practices in Beijing, China. Wang is rated as an Elite expert by MediFind in the treatment of Hemophagocytic Lymphohistiocytosis. They are also highly rated in 12 other conditions, according to our data. Their top areas of expertise are Hemophagocytic Lymphohistiocytosis, Non-Langerhans-Cell Histiocytosis, Reticulohistiocytoma, Histiocytosis, and Bone Marrow Transplant.

 
 
 
 
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ES
Elena Sieni
Elite
Highly rated in
6
conditions

Azienda Ospedaliero-Universitaria Meyer

Florence, IT 

Elena Sieni practices in Florence, Italy. Sieni is rated as an Elite expert by MediFind in the treatment of Hemophagocytic Lymphohistiocytosis. She is also highly rated in 6 other conditions, according to our data. Her top areas of expertise are Hemophagocytic Lymphohistiocytosis, Non-Langerhans-Cell Histiocytosis, Reticulohistiocytoma, Histiocytosis, and Bone Marrow Transplant.

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What are the latest Hemophagocytic Lymphohistiocytosis Clinical Trials?
Retrospective Study of Immunotherapy Related Toxicities in Children and Adults With Cancer
Enrollment Status: Active, not recruiting
Publish Date: February 10, 2023

Summary: Immunotherapy is changing the landscape of cancer therapy. Particularly unique to immunotherapy is the toxicity profile, which differs from chemotherapy-based strategies and can be associated with inflammatory responses and/or autoimmune type reactions resulting from activation of the immune system. Referred to as immune related adverse events (irAEs), these adverse events may require systemic imm...

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A Phase 1b, Open-label, Single-arm, Multicenter Study to Evaluate the Safety, Efficacy, Pharmacokinetics and Pharmacodynamics of Multiple Doses of ELA026 in Adults and Adolescents With Secondary Hemophagocytic Lymphohistiocytosis (sHLH)
Enrollment Status: Recruiting
Publish Date: February 08, 2023
Intervention Type: Drug
Study Phase: Phase 1

Summary: Hemophagocytic lymphohistiocytosis is a rare, aggressive and life-threatening syndrome of excessive immune activation. ELA026 is a fully human IgG1 SIRP-directed monoclonal antibody designed to reduce the myeloid and T cells driving the inflammation. The purpose of this study is to assess the safety, efficacy pharmacokinetics and pharmacodynamics of ELA026 in adult and adolescent patients with sec...

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Who are the sources who wrote this article ?

Published Date: May 02, 2022
Published By: Genetic and Rare Diseases Informnation Center

What are the Latest Advances for Hemophagocytic Lymphohistiocytosis?
Ruxolitinib as an effective treatment for panniculitis associated hemophagocytic syndrome: A report of 2 cases and literature review.
Condition: Panniculitis Associated Hemophagocytic Syndrome (HPS)
Journal: Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences
Treatment Used: Ruxolitinib
Number of Patients: 2
Published: December 19, 2022
A retrospective study of 18 children with subcutaneous panniculitis-like T-cell lymphoma: multidrug combination chemotherapy or immunomodulatory therapy?
Condition: Children with Subcutaneous Panniculitis T-Cell Lymphoma (SPTCL)
Journal: Orphanet journal of rare diseases
Treatment Used: Multidrug Combination Chemotherapy vs. Immunomodulatory Therapy
Number of Patients: 18
Published: December 12, 2022
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Hemophagocytic lymphohistocytosis in trisomy 21: successful treatment with interferon inhibition.
Condition: Trisomy 21/Hemophagocytic Lymphohistocytosis (HLH)
Journal: Pediatric rheumatology online journal
Treatment Used: Emapalumab Followed by Baricitinib
Number of Patients: 2
Published: November 19, 2022
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