Autosomal dominant aplasia and myelodysplasia

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ORPHA:314399OMIM:614675D61.0
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4Active trials8Treatment centers

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

Autosomal dominant aplasia and myelodysplasia is a rare inherited blood disorder in which the bone marrow fails to produce blood cells properly. The bone marrow is the spongy tissue inside bones where blood cells are made. In this condition, the bone marrow can become severely underactive (aplasia) or produce abnormal, poorly functioning blood cells (myelodysplasia). Because it follows an autosomal dominant inheritance pattern, only one copy of the altered gene from one parent is enough to cause the disease. People with this condition often develop low blood counts, which can include low red blood cells (anemia), low white blood cells (increasing infection risk), and low platelets (leading to easy bruising and bleeding). Symptoms may include persistent fatigue, frequent or severe infections, unexplained bruising, pale skin, and shortness of breath. Some individuals may progress to more serious blood cancers such as acute myeloid leukemia (AML). Treatment depends on the severity of the disease. Supportive care includes blood transfusions and antibiotics for infections. Growth factors may be used to stimulate blood cell production. In more severe cases, a bone marrow (stem cell) transplant may be considered, which is currently the only potentially curative option. Regular monitoring with blood tests and bone marrow biopsies is essential to track disease progression and watch for transformation to leukemia.

Also known as:

Key symptoms:

Persistent tiredness and fatiguePale skinFrequent or severe infectionsEasy bruisingUnusual or prolonged bleedingShortness of breathWeaknessRecurrent feversSmall red or purple spots on the skin (petechiae)Bone painEnlarged spleenUnexplained weight lossDizziness or lightheadedness

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 ↗

FDA & Trial Timeline

4 events
Dec 2023Imatinib to Increase RUNX1 Activity in Participants With Germline RUNX1 Deficiency

National Cancer Institute (NCI) — PHASE1

TrialRECRUITING
Feb 2017French National Registry of Bone Marrow Failures

Assistance Publique - Hôpitaux de Paris — NA

TrialRECRUITING
Jan 2017Pre-myeloid Cancer and Bone Marrow Failure Clinic Study

Mayo Clinic — NA

TrialRECRUITING
Aug 2013Natural History Study of GATA2 Deficiency and Related Disorders

National Institute of Allergy and Infectious Diseases (NIAID)

TrialRECRUITING

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Treatments

No FDA-approved treatments are currently listed for Autosomal dominant aplasia and myelodysplasia.

4 clinical trialsare actively recruiting — trials can provide access to cutting-edge therapies.

View clinical trials →

Clinical Trials

4 recruitingView all trials with filters →
Phase 11 trial
Imatinib to Increase RUNX1 Activity in Participants With Germline RUNX1 Deficiency
Phase 1
Actively Recruiting
PI: Lea C Cunningham, M.D. (National Cancer Institute (NCI)) · Sites: Bethesda, Maryland · Age: 18120 yrs
N/A2 trials
Pre-myeloid Cancer and Bone Marrow Failure Clinic Study
N/A
Actively Recruiting
PI: Mrinal S. Patnaik, MBBS (Mayo Clinic) · Sites: Scottsdale, Arizona; Jacksonville, Florida +1 more · Age: 1899 yrs
French National Registry of Bone Marrow Failures
N/A
Actively Recruiting
PI: Régis Peffault De Latour · Sites: Paris
Other1 trial
Natural History Study of GATA2 Deficiency and Related Disorders
Actively Recruiting
PI: Steven M Holland, M.D. (National Institute of Allergy and Infectious Disea) · Sites: Bethesda, Maryland · Age: 2100 yrs

No specialists are currently listed for Autosomal dominant aplasia and myelodysplasia.

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 Autosomal dominant aplasia and myelodysplasia.

Search all travel grants →NORD Financial Assistance ↗

Community

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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.How severe is my bone marrow failure, and what does my bone marrow biopsy show?,What is my risk of developing leukemia, and how will you monitor for this?,Should I or my family members undergo genetic testing?,Am I a candidate for a bone marrow transplant, and when should we consider it?,What symptoms should prompt me to seek emergency care?,Are there clinical trials or new treatments I should know about?,How often will I need blood tests and bone marrow biopsies?

Common questions about Autosomal dominant aplasia and myelodysplasia

What is Autosomal dominant aplasia and myelodysplasia?

Autosomal dominant aplasia and myelodysplasia is a rare inherited blood disorder in which the bone marrow fails to produce blood cells properly. The bone marrow is the spongy tissue inside bones where blood cells are made. In this condition, the bone marrow can become severely underactive (aplasia) or produce abnormal, poorly functioning blood cells (myelodysplasia). Because it follows an autosomal dominant inheritance pattern, only one copy of the altered gene from one parent is enough to cause the disease. People with this condition often develop low blood counts, which can include low red

How is Autosomal dominant aplasia and myelodysplasia inherited?

Autosomal dominant aplasia and myelodysplasia follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

Are there clinical trials for Autosomal dominant aplasia and myelodysplasia?

Yes — 4 recruiting clinical trials are currently listed for Autosomal dominant aplasia and myelodysplasia on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.