Hemophagocytic Lymphohistiocytosis Overview

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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.
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?
Naval G. Daver
Elite in Hemophagocytic Lymphohistiocytosis
Dr. Naval G. Daver
Hematology Oncology | Oncology | Hematology
Elite in Hemophagocytic Lymphohistiocytosis
Dr. Naval G. Daver
Hematology Oncology | Oncology | Hematology

MD Anderson

2130 W Holcombe Blvd, Univ Of Tx M D Ande Life, 
Houston, TX 
713-592-5433
Languages Spoken:
English
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Naval Daver is a Hematologist Oncology specialist and an Oncologist in Houston, Texas. Dr. Daver is rated as an Elite provider by MediFind in the treatment of Hemophagocytic Lymphohistiocytosis. His top areas of expertise are Acute Myeloid Leukemia (AML), Myelodysplastic Syndrome (MDS), Leukemia, Bone Marrow Aspiration, and Bone Marrow Transplant.

Franco M. Locatelli
Elite in Hemophagocytic Lymphohistiocytosis
Franco M. Locatelli
Elite in Hemophagocytic Lymphohistiocytosis
Franco M. Locatelli
Rome, IT 

Franco Locatelli practices in Rome, Italy. Mr. Locatelli is rated as an Elite expert by MediFind in the treatment of Hemophagocytic Lymphohistiocytosis. His top areas of expertise are Acute Lymphoblastic Leukemia (ALL), Leukemia, Graft Versus Host Disease (GvHD), Bone Marrow Transplant, and Splenectomy.

 
 
 
 
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Elite in Hemophagocytic Lymphohistiocytosis
Fabrizio De Benedetti
Elite in Hemophagocytic Lymphohistiocytosis
Fabrizio De Benedetti
Rome, IT 

Fabrizio De Benedetti practices in Rome, Italy. Mr. De Benedetti is rated as an Elite expert by MediFind in the treatment of Hemophagocytic Lymphohistiocytosis. His top areas of expertise are Juvenile Idiopathic Arthritis (JIA), Hemophagocytic Lymphohistiocytosis, Non-Langerhans-Cell Histiocytosis, and Histiocytosis.

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What are the latest Hemophagocytic Lymphohistiocytosis Clinical Trials?
A Multipart, Open-label, Single-arm, Multicenter Study to Evaluate the Safety, Efficacy and Pharmacokinetics of ELA026 in Participants With Secondary Hemophagocytic Lymphohistiocytosis (sHLH)
A Multipart, Open-label, Single-arm, Multicenter Study to Evaluate the Safety, Efficacy and Pharmacokinetics of ELA026 in Participants With Secondary Hemophagocytic Lymphohistiocytosis (sHLH)
Enrollment Status: Recruiting
Publish Date: October 14, 2025
Intervention Type: Drug
Study Phase: Phase 2/Phase 3

Summary: Hemophagocytic lymphohistiocytosis is a rare, aggressive and life-threatening syndrome of excessive immune activation. Secondary hemophagocytic lymphohistiocytosis (sHLH) is the most common form of this disease and is typically associated with several other clinical conditions (eg, malignancy associated HLH (mHLH), infection, or autoimmune disease). ELA026 is a fully human immunoglobulin G1 (IgG1)...

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A Phase 1/1b Study of ASP2138 as Monotherapy and in Combination With Pembrolizumab and mFOLFOX6 or Ramucirumab and Paclitaxel in Participants With Metastatic or Locally Advanced Unresectable Gastric or Gastroesophageal Junction (GEJ) Adenocarcinoma and in Combination With mFOLFIRINOX in Participants With Metastatic or Locally Advanced Unresectable Pancreatic Adenocarcinoma Whose Tumors Have Claudin (CLDN) 18.2 Expression
A Phase 1/1b Study of ASP2138 as Monotherapy and in Combination With Pembrolizumab and mFOLFOX6 or Ramucirumab and Paclitaxel in Participants With Metastatic or Locally Advanced Unresectable Gastric or Gastroesophageal Junction (GEJ) Adenocarcinoma and in Combination With mFOLFIRINOX in Participants With Metastatic or Locally Advanced Unresectable Pancreatic Adenocarcinoma Whose Tumors Have Claudin (CLDN) 18.2 Expression
Enrollment Status: Recruiting
Publish Date: September 15, 2025
Intervention Type: Drug
Study Phase: Phase 1

Summary: Claudin 18.2 protein, or CLDN18.2 is a protein found on cells in the digestive system. It is also found on some tumors. Researchers are looking at ways to attack CLDN18.2 to help control tumors. ASP2138 is thought to bind to CLDN18.2 and a protein on a type of immune cell called a T-cell. This tells the immune system to attack the tumor. ASP2138 is a potential treatment for people with stomach can...

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