A Group of Rare Blood Disorders
Hypereosinophilicsyndromes are a varied group of rare blood diseases.
Most hypereosinophilic syndromes are not cancer.
But some people may develop blood cancer (leukemia) after diagnosis.
Westend61 / Getty Images
This article covers the signs, symptoms, causes, complications, and treatment options for hypereosinophilic syndromes.
It will discuss if hypereosinophilic syndromes can be cured and the link between these diseases and leukemia or cancer.
Westend61 / Getty Images
What Is Hypereosinophilic Syndrome?
Reproduced with permission from © DermNetdermnetnz.org2023.
Hypereosinophilic syndrome is when the body has too many eosinophils in the blood or tissues for a prolonged time.
As part of that role, they cause inflammation.
Having too many eosinophils in the bloodstream is calledeosinophilia.
It can be a symptom of many diseases and conditions.
In hypereosinophilic syndromes, the eosinophils arent functioning generally as immune system cells.
They build up in tissues and cause damage.
In these cases, healthcare providers cant find a cause for the high number of white blood cells.
They build up to high levels that can damage the bodys organs.
Studies have found that hypereosinophilic syndromes develop between 0.3 and 6.3 out of every 100,000 people.
What Causes Hypereosinophilic Syndrome?
There is no single cause of hypereosinophilic syndrome.
They may be genetic, an early sign of cancer, an unknown infection, or other health problems.
About 90% of HES cases are males in middle age.
Hypereosinophilic syndrome is not leukemia.
It may take several years after hypereosinophilic syndrome is first found for healthcare providers touncover leukemia.
Whats the Most Common Sign of Hypereosinophilic Syndrome?
Not all people with hypereosinophilic syndrome do.
It depends on what organs the eosinophils occupy.
The rash also looks different in different people.
This photo contains content that some people may find graphic or disturbing.
Reproduced with permission from A DermNetdermnetnz.org2023.
Complications Associated With Hypereosinophilic Syndrome
Hypereosinophilic syndromes affect organs other than the skin.
HES in the heart, lungs, stomach, intestines, and other organs can lead to organ dysfunction.
Eventually, about 20% develop heart issues.
An average blood eosinophil number is 350 to 500 per microliter.
A number anywhere between 500 and 1500 may be called mild eosinophilia.
Moderate eosinophilia may fall between 1,500 and 5,000 eosinophils per microliter.
Severe eosinophilia is above 5,000 eosinophils per microliter.
In tissues other than blood, an increased eosinophil number is determined by analyzing samples in the lab.
HES may be diagnosed if:
Genetic testing looks for changes associated with hypereosinophilic syndromes.
Imaging studies, like chest X-rays andechocardiograms, assess damage to the heart and lungs.
How Is Hypereosinophilic Syndrome Treated?
Treatment for hypereosinophilic syndrome depends on the cause of the disease.
It also aims to reduce inflammation directly and slow disease progression.
Treatments vary based on the organs that are affected.
Other health issues can also complicate treatment.
It can be hard to predict how serious the disease will be in a given person.
People who are not having symptoms do not need treatment.
A healthcare provider will closely monitor these people for organ damage and complications.
Corticosteroids
Treatment with corticosteroids like prednisone is often the first treatment choice.
This isnt a cure, though.
Eosinophilia and symptoms come back once steroids are stopped.
Chemotherapy
In advanced HES, chemotherapy drugs keep eosinophil numbers low by killing fast-growing cells.
They can also treat blood cancers causing eosinophilia.
IFNa is effective in HES by suppressing the symptoms related to the disease.
About 25% to 30% of people stop taking Interferon alpha due to side effects.
Monoclonal Antibodies
When HES does not react to traditional treatments, specific monoclonal antibody treatments may help.
These include:
Can I Prevent Hypereosinophilic Syndrome?
There is no way to prevent hypereosinophilic syndromes.
Whats the Outlook for Hypereosinophilic Syndrome?
HES prognosis depends on whats causing the disease.
It cannot be cured, but medications can control most types.
How the disease responds to these treatments plays a significant role in how the condition plays out.
Prognosis depends on what organs the eosinophils are attacking and how much damage theyve done.
If the damage is already severe, it cant be undone, and HES may be fatal.
The most common cause of death in cases of HES is heart failure.
Today, more than 80% of HES patients survive five years or more.
If you think you have signs and symptoms of HES, schedule an exam.
Summary
Hypereosinophilic syndromes are a group of rare blood diseases.
The organs affected include the skin, heart, lungs, and gastrointestinal tract.
The most common sign of hypereosinophilic syndrome is a skin rash or swelling.
Other symptoms include weakness, fatigue, cough, trouble breathing, muscle aches, and fever.
Complications include heart failure, kidney damage, and blood clots.
Healthcare providers diagnose hypereosinophilic syndrome after ruling out other potential causes.
The cause of hypereosinophilic syndromes is often unknown, but some have a genetic origin.
Treatment aims to reduce eosinophil levels and inflammation.
Typical treatments include corticosteroids, chemotherapy, and targeted therapies.
Monoclonal antibodies can help.
Hypereosinophilic syndrome outlook depends on organ damage and response to treatment.
While there is no cure, it can be managed with medications.
American Academy of Allergy Asthma & Immunology.Hypereosinophilic syndrome symptoms, diagnosis, treatment & management.
American Partnership for Eosinophilic Disorders.Hypereosinophilic syndromes.
Schuster B, Zink A, Eyerich K.Medical algorithm: diagnosis and treatment of hypereosinophilic syndrome.Allergy.
2020;75(11):3003-3006. doi:10.1111/all.14368
Klion AD.How I treat hypereosinophilic syndromes.Blood.
2018;2018(1):326-331. doi:10.1182/asheducation-2018.1.326
DermNet.Hypereosinophilic syndrome.
2013;37(4):392-395. doi:10.1016/j.leukres.2012.12.016
