Hemophagocytic Lymphohistiocytosis
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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?
Elite in Hemophagocytic Lymphohistiocytosis
Dr. Kim E. Nichols
Hematology | Oncology
Elite in Hemophagocytic Lymphohistiocytosis
Dr. Kim E. Nichols
Hematology | Oncology

ETSU Pediatrics- St. Jude's Clinic

400 N State Of Franklin Rd, Fl 1, 
Johnson City, TN 
423-431-3950
Languages Spoken:
English
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Offers Telehealth

. Dr. Nichols is rated as an Elite provider by MediFind in the treatment of Hemophagocytic Lymphohistiocytosis. She is also highly rated in 11 other conditions, according to our data. Her clinical expertise encompasses Hemophagocytic Lymphohistiocytosis, Non-Langerhans-Cell Histiocytosis, Histiocytosis, Reticulohistiocytoma, and Bone Marrow Transplant. Dr. Nichols is board certified in American Board Of Pediatrics.

Elite in Hemophagocytic Lymphohistiocytosis
Dr. Sarah Nikiforow
Oncology
Elite in Hemophagocytic Lymphohistiocytosis
Dr. Sarah Nikiforow
Oncology

Dana-Farber Cancer Institute, Stem Cell Transplantation Program

450 Brookline Avenue, 
Boston, MA 
617-632-3470
Languages Spoken:
English
See accepted insurances

Sarah Nikiforow is an Oncologist practicing medicine in Boston, Massachusetts. Dr. Nikiforow is rated as an Elite provider by MediFind in the treatment of Hemophagocytic Lymphohistiocytosis. She is also highly rated in 12 other conditions, according to our data. Her clinical expertise encompasses Hemophagocytic Lymphohistiocytosis, Non-Langerhans-Cell Histiocytosis, Graft Versus Host Disease (GvHD), Histiocytosis, and Bone Marrow Transplant. Dr. Nikiforow is board certified in Hematology.

 
 
 
 
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Elite in Hemophagocytic Lymphohistiocytosis
Dr. Carl Allen
Hematology | Oncology
Elite in Hemophagocytic Lymphohistiocytosis
Dr. Carl Allen
Hematology | Oncology

Office

1102 Bates Ave Ste 750, 
Houston, TX 
832-824-4312
Languages Spoken:
English
See accepted insurances

Carl Allen is a Hematologist and an Oncologist practicing medicine in Houston, Texas. Dr. Allen is rated as an Elite provider by MediFind in the treatment of Hemophagocytic Lymphohistiocytosis. He is also highly rated in 10 other conditions, according to our data. His clinical expertise encompasses Langerhans Cell Histiocytosis, Reticulohistiocytoma, Histiocytosis, Hemophagocytic Lymphohistiocytosis, and Bone Marrow Transplant.

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What are the latest Hemophagocytic Lymphohistiocytosis Clinical Trials?
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: April 13, 2026
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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Combination of Mitoxantrone Liposome and Etoposide, Dexamethasone, Pegaspargase and Golidocitinib (MEPL-G) in the Treatment of NK/T-cell Lymphoma Associated Hemophagocytic Lymphohistiocytosis (NKTCL-HLH): a Prospective, Multi-center, Single Arm, Phase Ib/II Clinical Trial
Combination of Mitoxantrone Liposome and Etoposide, Dexamethasone, Pegaspargase and Golidocitinib (MEPL-G) in the Treatment of NK/T-cell Lymphoma Associated Hemophagocytic Lymphohistiocytosis (NKTCL-HLH): a Prospective, Multi-center, Single Arm, Phase Ib/II Clinical Trial
Enrollment Status: Recruiting
Publish Date: April 13, 2026
Intervention Type: Drug
Study Phase: Phase 1/Phase 2

Summary: Extranodal NK/T-cell lymphoma (NKTCL) is an aggressive EBV-associated lymphoma with poor prognosis, highly prevalent in China. Early-stage NKTCL achieves favorable long-term survival, while advanced disease shows dismal outcomes with no standard therapy. Notably, 10%-20% of patients develop secondary hemophagocytic lymphohistiocytosis (NKTCL-HLH), a life-threatening complication with median surviv...

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