Overview
Thymoma-hypogammaglobulinemia syndrome, also known as Good syndrome, is a rare acquired immunodeficiency disorder characterized by the combination of a thymoma (a tumor of the thymus gland) and hypogammaglobulinemia (abnormally low levels of immunoglobulins/antibodies in the blood). This condition primarily affects the immune system, leading to both humoral and cell-mediated immune deficiency. It was first described by Robert Good in 1954 and accounts for approximately 6-11% of adult-onset hypogammaglobulinemia cases. Patients with Good syndrome typically present in middle-aged to older adulthood with recurrent and often severe infections, including sinopulmonary infections (affecting the sinuses and lungs), opportunistic infections (such as cytomegalovirus, mucocutaneous candidiasis, and Pneumocystis jirovecii pneumonia), and chronic diarrhea. Laboratory findings characteristically include low or absent B cells in peripheral blood, reduced serum immunoglobulin levels across all classes (IgG, IgA, and IgM), and abnormalities in T-cell subsets including an inverted CD4/CD8 ratio. Patients may also develop autoimmune manifestations such as pure red cell aplasia, myasthenia gravis, and oral lichen planus. Treatment involves surgical removal (thymectomy) of the thymoma, although this does not typically resolve the immunodeficiency. Lifelong immunoglobulin replacement therapy is the cornerstone of management for the hypogammaglobulinemia, along with prophylactic antibiotics and antifungal agents to prevent recurrent infections. Despite treatment, the prognosis of Good syndrome is generally poorer than that of other primary immunodeficiencies such as common variable immunodeficiency (CVID), largely due to the severity and frequency of opportunistic infections and associated autoimmune complications.
Also known as:
Clinical phenotype terms— hover any for plain English:
Sporadic
Usually appears on its own, not inherited from a parent
Adult
Begins in adulthood (age 18 or older)
FDA & Trial Timeline
10 eventsCOEN Matteo — NA
Trustees of Dartmouth College — NA
Necmettin Erbakan University — NA
International AIDS Vaccine Initiative — PHASE1
Universität Duisburg-Essen — NA
Northumbria University — NA
Northumbria University — NA
Massachusetts General Hospital — NA
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
No FDA-approved treatments are currently listed for Thymoma-hypogammaglobulinemia syndrome.
View clinical trials →Clinical Trials
View all trials with filters →No actively recruiting trials found for Thymoma-hypogammaglobulinemia syndrome at this time.
New trials open frequently. Follow this disease to get notified.
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Thymoma-hypogammaglobulinemia syndrome.
Community
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Start the conversation →Latest news about Thymoma-hypogammaglobulinemia syndrome
Disease timeline:
New recruiting trial: The Effect of Kinesio Taping and Manual Lymph Drainage on Pregnant Women With Restless Legs Syndrome
A new clinical trial is recruiting patients for Thymoma-hypogammaglobulinemia syndrome
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A new clinical trial is recruiting patients for Thymoma-hypogammaglobulinemia syndrome
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A new clinical trial is recruiting patients for Thymoma-hypogammaglobulinemia syndrome
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A new clinical trial is recruiting patients for Thymoma-hypogammaglobulinemia syndrome
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A new clinical trial is recruiting patients for Thymoma-hypogammaglobulinemia syndrome
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A new clinical trial is recruiting patients for Thymoma-hypogammaglobulinemia syndrome
New recruiting trial: Study of the Evolution of the Expression of the LAMP-2 Protein During the Advance in Age
A new clinical trial is recruiting patients for Thymoma-hypogammaglobulinemia syndrome
New recruiting trial: Strengthening Health Literacy in Healthcare Workers Through Mind Body Medicine and Nutrition
A new clinical trial is recruiting patients for Thymoma-hypogammaglobulinemia syndrome
New recruiting trial: Clinical Trial to Evaluate the Safety and Immunogenicity of a Priming Regimen of 426c.Mod.Core-C4b Followed by HxB2.WT.Core-C4b Boosts, Both Adjuvanted With 3M-052 AF + Alum, in Adult Participants Without HIV and in Overall Good Health
A new clinical trial is recruiting patients for Thymoma-hypogammaglobulinemia syndrome
New recruiting trial: Well-being in IBS: Strengths and Happiness (WISH) 2.0
A new clinical trial is recruiting patients for Thymoma-hypogammaglobulinemia syndrome
Caregiver Resources
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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.
Common questions about Thymoma-hypogammaglobulinemia syndrome
What is Thymoma-hypogammaglobulinemia syndrome?
Thymoma-hypogammaglobulinemia syndrome, also known as Good syndrome, is a rare acquired immunodeficiency disorder characterized by the combination of a thymoma (a tumor of the thymus gland) and hypogammaglobulinemia (abnormally low levels of immunoglobulins/antibodies in the blood). This condition primarily affects the immune system, leading to both humoral and cell-mediated immune deficiency. It was first described by Robert Good in 1954 and accounts for approximately 6-11% of adult-onset hypogammaglobulinemia cases. Patients with Good syndrome typically present in middle-aged to older adult
How is Thymoma-hypogammaglobulinemia syndrome inherited?
Thymoma-hypogammaglobulinemia syndrome follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Thymoma-hypogammaglobulinemia syndrome typically begin?
Typical onset of Thymoma-hypogammaglobulinemia syndrome is adult. Age of onset can vary across affected individuals.
Which specialists treat Thymoma-hypogammaglobulinemia syndrome?
2 specialists and care centers treating Thymoma-hypogammaglobulinemia syndrome are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.