Hemophagocytic Lymphohistiocytosis

Also known as: HLH, hemophagocytic syndromes

What is hemophagocytic lymphohistiocytosis?

Hemophagocytic lymphohistiocytosis is a condition related to the body’s immune system that can be dangerous and life-threatening. The disease is an extreme example of immune system dysfunction, where the body’s own immune system begins to attack important tissues and organs such as the brain, liver and bone marrow.

What causes hemophagocytic lymphohistiocytosis?
The condition often occurs when a person’s immune system isn’t doing a good job ridding the body of certain infections or damaged cells in the first place. As a reaction, the immune system becomes overstimulated and begins damaging important organs and tissues. The disease may be related to genetic mutations, and some other conditions can trigger hemophagocytic lymphohistiocytosis.

What are the symptoms of hemophagocytic lymphohistiocytosis?
Symptoms are severe and can include fevers, anemia, jaundice, hepatitis, enlarged organs, breathing problems, seizures, bleeding, rash and neurological disorders. Without treatment, the disease can be life-threatening.

What are hemophagocytic lymphohistiocytosis care options?
The medications for hemophagocytic lymphohistiocytosis suppress the immune system and include drugs such as chemotherapy. In many cases, such as in the genetic form of the disease, a bone marrow transplant is required in order to cure the disease.

Reviewed by: Kamar Godder, MD

This page was last updated on: 5/23/2018 2:19:57 PM


Upcoming Events

Camp UOTS

Camp U.O.T.S. is an annual weeklong, overnight camp for children with cancer and blood disorders who are treated at Nicklaus Children’s Hospital.

Learn more and register

From the Newsdesk

Siblings of Cancer Patients enjoy a Fun Filled Day
07/06/2018 — More than two dozen children attended the Bear Hug camp at Nicklaus Children's last week. This day camp is for siblings of pediatric cancer patients to encourage socialization among peers and help them gain insight on their siblings' care journey. 
Daniella Celebrates her Ninth Birthday by Advocating for Children’s Health
06/26/2018 — On this very same day nine years ago, Daniella Alvarez was diagnosed Teratoid Rhabdoid Tumor (ATRT), a rare and aggressive type of brain cancer. The news came on June 26, 2009, her second birthday. Daniella endured years of brain surgeries, aggressive chemotherapies, radiation, imaging scans, multiple visits to intensive care at Nicklaus Children’s Hospital. She is now cancer free thanks to a pediatric clinical trial made possible through research funding.

Video

video
When he and his family were still living in Venezuela, Juan Pablo was diagnosed with stage 4 neuroblastoma, an aggressive type of cancer. Watch Juan Pablo’s story and help us raise awareness throughout the month of September for Pediatric Cancer Awareness Month.