Adult idiopathic neutropenia

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ORPHA:2688OMIM:607847D70
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Overview

Adult idiopathic neutropenia (AIN) is a blood disorder in which adults have a persistently low number of neutrophils — a type of white blood cell that plays a key role in fighting bacterial infections. The word "idiopathic" means the cause is unknown; doctors cannot find an underlying disease, medication, or genetic reason for the low neutrophil count. It is sometimes also called chronic idiopathic neutropenia of adults or chronic benign neutropenia. Neutrophils are your body's first line of defense against bacteria and fungi. When their numbers drop below normal (a condition called neutropenia), you may become more vulnerable to infections, particularly of the skin, mouth, gums, and respiratory tract. However, many people with adult idiopathic neutropenia experience surprisingly few serious infections, and the condition often follows a relatively mild course. Some patients have no symptoms at all and are only diagnosed after a routine blood test reveals a low white blood cell count. Treatment depends on how low the neutrophil count is and how often infections occur. Many patients need no treatment and are simply monitored with regular blood tests. For those who develop frequent or severe infections, granulocyte colony-stimulating factor (G-CSF), such as filgrastim, can be used to boost neutrophil production. Antibiotics may be prescribed to treat or prevent infections. The condition is considered chronic, meaning it lasts for months to years, but it is generally not life-threatening.

Also known as:

Key symptoms:

Frequent bacterial infectionsRecurring mouth sores or ulcersGum disease or swollen gumsSkin infections or boilsSore throat or throat infectionsFeverFatigue or tirednessSinus infectionsLung infections or pneumoniaUrinary tract infectionsSlow wound healingNo symptoms at all in mild cases

Clinical phenotype terms (15)— hover any for plain English
Helicobacter pylori infectionHP:0005202Abnormal bone marrow cell morphologyHP:0005561Increased circulating IgM levelHP:0003496Recurrent aphthous stomatitisHP:0011107Granulocytic hypoplasiaHP:0012139Decreased total monocyte countHP:0012312Bone marrow hypercellularityHP:0031020Recurrent fungal infectionsHP:0002841Increased total monocyte countHP:0012311Antineutrophil antibody positivityHP:0003453
Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Adult

Begins in adulthood (age 18 or older)

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Adult idiopathic neutropenia.

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No actively recruiting trials found for Adult idiopathic neutropenia at this time.

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No specialists are currently listed for Adult idiopathic neutropenia.

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 Adult idiopathic neutropenia.

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Community

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Questions for your doctor

Bring these to your next appointment

  • Q1.How low is my neutrophil count, and what does that mean for my infection risk?,How often should I have blood tests to monitor my neutrophil levels?,What signs of infection should I watch for, and when should I go to the emergency room?,Do I need treatment with G-CSF, or can we safely monitor my condition?,Are there any medications or supplements I should avoid because they could lower my counts further?,Should I get any special vaccinations or avoid live vaccines?,Is there a chance my neutropenia could improve on its own or get worse over time?

Common questions about Adult idiopathic neutropenia

What is Adult idiopathic neutropenia?

Adult idiopathic neutropenia (AIN) is a blood disorder in which adults have a persistently low number of neutrophils — a type of white blood cell that plays a key role in fighting bacterial infections. The word "idiopathic" means the cause is unknown; doctors cannot find an underlying disease, medication, or genetic reason for the low neutrophil count. It is sometimes also called chronic idiopathic neutropenia of adults or chronic benign neutropenia. Neutrophils are your body's first line of defense against bacteria and fungi. When their numbers drop below normal (a condition called neutropen

How is Adult idiopathic neutropenia inherited?

Adult idiopathic neutropenia follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Adult idiopathic neutropenia typically begin?

Typical onset of Adult idiopathic neutropenia is adult. Age of onset can vary across affected individuals.