Familial monosomy 7 syndrome

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ORPHA:495930OMIM:619041D46.7
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Overview

Familial monosomy 7 syndrome is a rare blood disorder where the bone marrow — the spongy tissue inside your bones that makes blood cells — loses one copy of chromosome 7 in some or all of its cells. Normally, people have two copies of chromosome 7 in every cell. When one copy is missing in bone marrow cells, it disrupts the normal production of blood cells and greatly increases the risk of developing serious blood cancers, especially a type of bone marrow failure called myelodysplastic syndrome (MDS) and acute myeloid leukemia (AML). This condition is also sometimes called familial MDS/AML with monosomy 7. The syndrome tends to run in families, meaning multiple relatives may be affected across generations. It can appear in children or adults, though it often shows up earlier in life compared to non-familial forms of MDS. Common symptoms include frequent infections, unusual bruising or bleeding, and persistent tiredness caused by low blood cell counts. Some people also develop an enlarged spleen. Treatment focuses on managing the blood disorder and reducing cancer risk. The only potentially curative option is a stem cell transplant (also called a bone marrow transplant), which replaces the faulty bone marrow with healthy donor cells. Regular monitoring by a blood specialist is essential for all people with this condition, even before symptoms appear.

Key symptoms:

Extreme tiredness and weakness due to low red blood cell counts (anemia)Frequent or severe infections due to low white blood cell countsEasy bruising or unusual bleeding due to low platelet countsEnlarged spleen causing discomfort or a feeling of fullness in the abdomenPale skinShortness of breath with normal activitiesFever without a clear causeBone marrow failureDevelopment of myelodysplastic syndrome (MDS)Risk of progressing to acute myeloid leukemia (AML)

Inheritance

Autosomal dominant

Passed on from just one parent; each child has about a 50% chance of inheriting it

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 Familial monosomy 7 syndrome.

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No actively recruiting trials found for Familial monosomy 7 syndrome at this time.

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No specialists are currently listed for Familial monosomy 7 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 Familial monosomy 7 syndrome.

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Community

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Latest news about Familial monosomy 7 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.Which gene mutation is causing this in our family, and what does that mean for other relatives?,Should other family members be tested, and how soon?,How often do I need blood tests and bone marrow checks?,At what point would you recommend a stem cell transplant, and how do we find a donor?,What warning signs should make me go to the emergency room right away?,Are there any clinical trials I or my family member might be eligible for?,What support services are available to help our family cope with this diagnosis?

Common questions about Familial monosomy 7 syndrome

What is Familial monosomy 7 syndrome?

Familial monosomy 7 syndrome is a rare blood disorder where the bone marrow — the spongy tissue inside your bones that makes blood cells — loses one copy of chromosome 7 in some or all of its cells. Normally, people have two copies of chromosome 7 in every cell. When one copy is missing in bone marrow cells, it disrupts the normal production of blood cells and greatly increases the risk of developing serious blood cancers, especially a type of bone marrow failure called myelodysplastic syndrome (MDS) and acute myeloid leukemia (AML). This condition is also sometimes called familial MDS/AML wit

How is Familial monosomy 7 syndrome inherited?

Familial monosomy 7 syndrome follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.