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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Offers Telehealth

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
Dr. Rebecca A. Marsh
Pediatric Hematology Oncology
Elite in Hemophagocytic Lymphohistiocytosis
Dr. Rebecca A. Marsh
Pediatric Hematology Oncology

Children's Hospital Medical Center

3333 Burnet Ave, 
Cincinnati, OH 
513-636-4200
Languages Spoken:
English
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Rebecca Marsh is a Pediatric Hematologist Oncology provider in Cincinnati, Ohio. Dr. Marsh is rated as an Elite provider by MediFind in the treatment of Hemophagocytic Lymphohistiocytosis. Her top areas of expertise are Hemophagocytic Lymphohistiocytosis, Non-Langerhans-Cell Histiocytosis, Histiocytosis, Reticulohistiocytoma, and Bone Marrow Transplant.

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What are the latest Hemophagocytic Lymphohistiocytosis Clinical Trials?
A Phase II Study of Reduced Intensity Conditioning in Pediatric Patients and Young Adults ≤55 Years of Age With Non-Malignant Disorders Undergoing Umbilical Cord Blood, Bone Marrow, or Peripheral Blood Stem Cell Transplantation
A Phase II Study of Reduced Intensity Conditioning in Pediatric Patients and Young Adults ≤55 Years of Age With Non-Malignant Disorders Undergoing Umbilical Cord Blood, Bone Marrow, or Peripheral Blood Stem Cell Transplantation
Who is this study for: Patients with non-malignant disorders that are amenable to treatment with hematopoietic stem cell transplant
Enrollment Status: Recruiting
Publish Date: December 15, 2025
Intervention Type: Drug
Study Drugs: Hydroxyurea, Alemtuzumab, Fludarabine, Melphalan, Thiotepa
Study Phase: Phase 2

Summary: The objective of this study is to evaluate the efficacy of using a reduced-intensity condition (RIC) regimen with umbilical cord blood transplant (UCBT), double cord UCBT, matched unrelated donor (MUD) bone marrow transplant (BMT) or peripheral blood stem cell transplant (PBSCT) in patients with non-malignant disorders that are amenable to treatment with hematopoietic stem cell transplant (HSCT). ...

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Frontline Ruxolitinib With De-Intensified HLH-94 for Adult Hemophagocytic Lymphohistiocytosis (HLH): A Multicenter, Single-Arm Phase 2 Study
Frontline Ruxolitinib With De-Intensified HLH-94 for Adult Hemophagocytic Lymphohistiocytosis (HLH): A Multicenter, Single-Arm Phase 2 Study
Enrollment Status: Recruiting
Publish Date: December 12, 2025
Intervention Type: Drug, Procedure
Study Phase: Phase 2

Summary: This phase II trial tests the effects of ruxolitinib in combination with a de-intensified HLH-94 drug regimen has on patients with newly diagnosed hemophagocytic lymphohistiocytosis (HLH), a disorder caused by dysregulated immune responses (that is, immune responses that are too strong and cause inflammatory damage to normal tissues). The therapy used for HLH decreases the activity of the immune s...

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