Syndromic constitutional thrombocytopenia

Last reviewed

🖨 Print for my doctorAdvocacy Hub →
ORPHA:477794
Who is this for?
Show terms as
8Treatment centers

Where are you in your journey?

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

Overview

Syndromic constitutional thrombocytopenia is a rare inherited blood disorder where a person is born with a low number of platelets — the tiny blood cells that help stop bleeding. Unlike thrombocytopenia that develops later in life due to illness or medication, this condition is present from birth and is caused by changes (mutations) in a person's genes. What makes it 'syndromic' is that the low platelet count comes along with other health problems affecting different parts of the body, such as the skeleton, kidneys, immune system, or development. Because platelets are essential for normal blood clotting, people with this condition often bruise easily, bleed longer than usual after cuts or injuries, and may experience nosebleeds or bleeding gums. In more serious cases, internal bleeding can occur. The additional features that come with the syndrome vary widely depending on which gene is affected, and can include birth defects, growth problems, or increased risk of certain cancers. Treatment is tailored to each person and depends on the specific genetic cause and associated features. Options may include platelet transfusions during bleeding episodes or surgery, medications to boost platelet production, and in some cases bone marrow transplantation. Regular monitoring by a team of specialists is an important part of managing this condition throughout life.

Key symptoms:

Low platelet count present from birthEasy or excessive bruisingProlonged bleeding from cuts or minor injuriesFrequent nosebleedsBleeding gumsSmall red or purple spots on the skin (petechiae)Heavy menstrual bleeding in femalesBone or skeletal abnormalities (depending on subtype)Kidney problems (depending on subtype)Immune system problems or frequent infections (depending on subtype)Hearing loss (in some subtypes)Developmental delays or intellectual disability (in some subtypes)Increased risk of blood cancers such as leukemia (in some subtypes)Abnormal facial features or birth defects (in some subtypes)

Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Syndromic constitutional thrombocytopenia.

View clinical trials →

No actively recruiting trials found for Syndromic constitutional thrombocytopenia at this time.

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

Search ClinicalTrials.gov ↗Join the Syndromic constitutional thrombocytopenia community →

No specialists are currently listed for Syndromic constitutional thrombocytopenia.

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 Syndromic constitutional thrombocytopenia.

Search all travel grants →NORD Financial Assistance ↗

Community

Open Syndromic constitutional thrombocytopeniaForum →

No community posts yet. Be the first to share your experience with Syndromic constitutional thrombocytopenia.

Start the conversation →

Latest news about Syndromic constitutional thrombocytopenia

No recent news articles for Syndromic constitutional thrombocytopenia.

Follow this condition to be notified when news becomes available.

Caregiver Resources

NORD Caregiver Resources

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

Mental Health Support

Rare disease caregiving can be isolating. Connect with counseling and peer support.

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.Which specific gene is causing my (or my child's) condition, and what does that mean for treatment and long-term health?,How often should platelet counts be monitored, and at what level should I seek emergency care?,Are there medications or activities I need to avoid because of the low platelet count?,Is bone marrow transplantation an option for my specific subtype, and what are the risks and benefits?,What is the risk of developing a blood cancer, and how will we monitor for that?,Should other family members be tested for this condition?,Are there any clinical trials or new treatments I should know about?

Common questions about Syndromic constitutional thrombocytopenia

What is Syndromic constitutional thrombocytopenia?

Syndromic constitutional thrombocytopenia is a rare inherited blood disorder where a person is born with a low number of platelets — the tiny blood cells that help stop bleeding. Unlike thrombocytopenia that develops later in life due to illness or medication, this condition is present from birth and is caused by changes (mutations) in a person's genes. What makes it 'syndromic' is that the low platelet count comes along with other health problems affecting different parts of the body, such as the skeleton, kidneys, immune system, or development. Because platelets are essential for normal blo

At what age does Syndromic constitutional thrombocytopenia typically begin?

Typical onset of Syndromic constitutional thrombocytopenia is neonatal. Age of onset can vary across affected individuals.