Hereditary isolated aplastic anemia

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ORPHA:397692OMIM:616553D61.0
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3Specialists8Treatment centers

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UniteRare data is sourced from FDA.gov, ClinicalTrials.gov, Orphanet, OMIM, and NORD.
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Overview

Hereditary isolated aplastic anemia is a rare inherited blood disorder in which the bone marrow fails to produce enough blood cells. Unlike acquired aplastic anemia, which can be triggered by infections, medications, or autoimmune problems, this form is caused by genetic changes passed down through families. The word 'isolated' means that the bone marrow failure occurs without other birth defects or physical abnormalities that are seen in related conditions like Fanconi anemia or dyskeratosis congenita. When the bone marrow does not work properly, all three types of blood cells can be affected: red blood cells (which carry oxygen), white blood cells (which fight infection), and platelets (which help blood clot). This leads to symptoms such as extreme tiredness, frequent or severe infections, and easy bruising or bleeding. The severity can range from mild to life-threatening depending on how much the bone marrow is affected. Treatment options include blood transfusions to manage symptoms, medications that stimulate blood cell production, immunosuppressive therapy, and in severe cases, bone marrow (hematopoietic stem cell) transplantation, which is the only potentially curative treatment. Early diagnosis and close monitoring by a hematologist are essential for managing this condition effectively.

Key symptoms:

Extreme tiredness and fatiguePale skinShortness of breath during normal activitiesFrequent or severe infectionsFevers that keep coming backEasy bruisingProlonged bleeding from cuts or injuriesNosebleeds that are hard to stopTiny red or purple spots on the skin (petechiae)Heavy menstrual periods in womenFeeling dizzy or lightheadedRapid heartbeatBleeding gums

Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Hereditary isolated aplastic anemia.

View clinical trials →

No actively recruiting trials found for Hereditary isolated aplastic anemia at this time.

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Search ClinicalTrials.gov ↗Join the Hereditary isolated aplastic anemia community →

Specialists

3 foundView all specialists →
RN
Ryotaro Nakamura
Duarte, California
Specialist

Rare Disease Specialist

PI on 6 active trials
TD
Tamila Kindwall-Keller, DO
Specialist
PI on 1 active trial19 Hereditary isolated aplastic anemia publications
PM
Paul Armistead, MD
CHAPEL HILL, NC
Specialist
PI on 1 active trial

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 Hereditary isolated aplastic anemia.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Hereditary isolated aplastic anemia

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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.How severe is the bone marrow failure, and what does that mean for my (or my child's) daily life?,Should we pursue genetic testing to identify the specific cause, and what genes will be tested?,Is bone marrow transplantation recommended, and how do we find a suitable donor?,What are the risks and benefits of immunosuppressive therapy versus transplant?,How often will blood counts need to be monitored, and what levels would trigger a transfusion?,What is the long-term risk of developing leukemia or other blood cancers?,Should other family members be tested for this genetic condition?

Common questions about Hereditary isolated aplastic anemia

What is Hereditary isolated aplastic anemia?

Hereditary isolated aplastic anemia is a rare inherited blood disorder in which the bone marrow fails to produce enough blood cells. Unlike acquired aplastic anemia, which can be triggered by infections, medications, or autoimmune problems, this form is caused by genetic changes passed down through families. The word 'isolated' means that the bone marrow failure occurs without other birth defects or physical abnormalities that are seen in related conditions like Fanconi anemia or dyskeratosis congenita. When the bone marrow does not work properly, all three types of blood cells can be affecte

Which specialists treat Hereditary isolated aplastic anemia?

3 specialists and care centers treating Hereditary isolated aplastic anemia are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.