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
Referral may be required

ETSU Pediatrics- St. Jude's Clinic

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

Kim Nichols is a Hematologist and an Oncologist practicing medicine in Johnson City, Tennessee. 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, Reticulohistiocytoma, Histiocytosis, 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
Referral may be required

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), Reticulohistiocytoma, 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
Referral may be required

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?
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: June 10, 2026
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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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: May 26, 2026
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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Who are the sources who wrote this article ?

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