OBSOLETE: Common variable immunodeficiency due to an intrinsic B cell defect

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ORPHA:77303
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Overview

This condition is listed in Orphanet under the code 77303 as 'OBSOLETE: Common variable immunodeficiency due to an intrinsic B cell defect.' The 'obsolete' label means that medical experts have reclassified or retired this specific diagnostic category. It was previously used to describe a subtype of Common Variable Immunodeficiency (CVID) where the problem originates inside the B cells themselves — the white blood cells responsible for making antibodies. Without enough working antibodies, the immune system cannot fight off infections properly. CVID as a broader condition causes the body to produce very low levels of protective antibodies (immunoglobulins). People with CVID-related conditions tend to get frequent, serious, or unusual infections — especially in the lungs, sinuses, and digestive system. Over time, repeated infections can damage organs, particularly the lungs. Because this specific subtype has been retired, patients who were previously given this diagnosis may now be classified under a more precise CVID subtype or a related primary immunodeficiency. If you have this diagnosis on your records, it is important to speak with an immunologist to confirm your current classification and ensure you are receiving the most up-to-date care. Treatment for CVID-related conditions typically involves regular immunoglobulin replacement therapy to boost the immune system.

Also known as:

Key symptoms:

Frequent or severe bacterial infections (such as pneumonia or sinus infections)Recurring ear infectionsChronic diarrhea or digestive problemsUnexplained weight lossSwollen lymph nodes or enlarged spleenFatigue and low energyAutoimmune problems (where the immune system attacks the body's own tissues)Inflammatory bowel-like symptomsIncreased risk of certain cancers, especially lymphomaSlow recovery from infections that most people shake off quickly

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 ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for OBSOLETE: Common variable immunodeficiency due to an intrinsic B cell defect.

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No actively recruiting trials found for OBSOLETE: Common variable immunodeficiency due to an intrinsic B cell defect at this time.

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No specialists are currently listed for OBSOLETE: Common variable immunodeficiency due to an intrinsic B cell defect.

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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 OBSOLETE: Common variable immunodeficiency due to an intrinsic B cell defect.

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Community

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Caregiver Resources

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Questions for your doctor

Bring these to your next appointment

  • Q1.Since this diagnosis is now listed as obsolete, what is my current correct diagnosis?,Which type of immunoglobulin replacement therapy is best for me — intravenous or subcutaneous?,How often do I need blood tests and lung function checks?,Are there any vaccines I should avoid or specifically get?,What signs of infection or complications should make me go to the emergency room?,Should I have genetic testing to find out if there is a specific gene causing my condition?,Are there any clinical trials I might be eligible for?

Common questions about OBSOLETE: Common variable immunodeficiency due to an intrinsic B cell defect

What is OBSOLETE: Common variable immunodeficiency due to an intrinsic B cell defect?

This condition is listed in Orphanet under the code 77303 as 'OBSOLETE: Common variable immunodeficiency due to an intrinsic B cell defect.' The 'obsolete' label means that medical experts have reclassified or retired this specific diagnostic category. It was previously used to describe a subtype of Common Variable Immunodeficiency (CVID) where the problem originates inside the B cells themselves — the white blood cells responsible for making antibodies. Without enough working antibodies, the immune system cannot fight off infections properly. CVID as a broader condition causes the body to pr