Lymphocytic hypereosinophilic syndrome

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Overview

Lymphocytic hypereosinophilic syndrome (L-HES) is a rare blood disorder in which a specific type of white blood cell called eosinophils builds up to very high levels in the blood and tissues. In this particular form, the problem starts with abnormal T-lymphocytes (a type of immune cell) that produce too much of certain chemical signals called cytokines, especially interleukin-5 (IL-5). These signals tell the body to make far too many eosinophils. When eosinophils accumulate in organs such as the skin, lungs, heart, and digestive tract, they can cause significant damage over time. Common symptoms include skin rashes, itching, fatigue, coughing, shortness of breath, abdominal pain, and sometimes swollen lymph nodes. The skin is one of the most frequently affected organs. Over time, if left untreated, the excess eosinophils can damage the heart and other vital organs, which can become life-threatening. Treatment typically involves corticosteroids to lower eosinophil counts, and in many cases additional medications are needed. Because the underlying problem involves abnormal T-cells, L-HES can sometimes progress to T-cell lymphoma, making long-term monitoring essential. Newer targeted therapies, including antibodies that block IL-5, have improved outcomes for many patients. The condition is chronic and usually requires ongoing management by a team of specialists.

Also known as:

Key symptoms:

Skin rashes or eczema-like patchesSevere itchingSwelling under the skin (angioedema)Fatigue and general weaknessCoughing or shortness of breathAbdominal pain or digestive problemsSwollen lymph nodesFeverMuscle or joint painWeight lossHeart problems such as chest pain or irregular heartbeatNumbness or tingling in hands or feetRecurrent infections

Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Adult

Begins in adulthood (age 18 or older)

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Lymphocytic hypereosinophilic syndrome.

View clinical trials →

No actively recruiting trials found for Lymphocytic hypereosinophilic syndrome at this time.

New trials open frequently. Follow this disease to get notified.

Search ClinicalTrials.gov ↗Join the Lymphocytic hypereosinophilic syndrome community →

No specialists are currently listed for Lymphocytic hypereosinophilic syndrome.

View NORD Rare Disease Centers ↗Undiagnosed Disease Network ↗

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Lymphocytic hypereosinophilic syndrome.

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Community

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Latest news about Lymphocytic hypereosinophilic syndrome

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Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

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Family & Caregiver Grants

Financial assistance programs specifically for caregivers of rare disease patients.

Social Security Disability

Learn how rare disease patients may qualify for SSDI/SSI benefits.

Questions for your doctor

Bring these to your next appointment

  • Q1.What is my current eosinophil level, and what is the target we are aiming for with treatment?,Do I have evidence of organ damage, especially to my heart, and how will you monitor for this?,What type of abnormal T-cells were found, and what does this mean for my risk of developing lymphoma?,What are the side effects of my current medications, and are there ways to reduce my steroid dose over time?,Am I a candidate for mepolizumab or other targeted biologic therapies?,How often will I need blood tests and follow-up visits?,Are there any clinical trials available for lymphocytic hypereosinophilic syndrome that I might be eligible for?

Common questions about Lymphocytic hypereosinophilic syndrome

What is Lymphocytic hypereosinophilic syndrome?

Lymphocytic hypereosinophilic syndrome (L-HES) is a rare blood disorder in which a specific type of white blood cell called eosinophils builds up to very high levels in the blood and tissues. In this particular form, the problem starts with abnormal T-lymphocytes (a type of immune cell) that produce too much of certain chemical signals called cytokines, especially interleukin-5 (IL-5). These signals tell the body to make far too many eosinophils. When eosinophils accumulate in organs such as the skin, lungs, heart, and digestive tract, they can cause significant damage over time. Common sympt

How is Lymphocytic hypereosinophilic syndrome inherited?

Lymphocytic hypereosinophilic syndrome follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Lymphocytic hypereosinophilic syndrome typically begin?

Typical onset of Lymphocytic hypereosinophilic syndrome is adult. Age of onset can vary across affected individuals.