National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Immunodeficiency with thymoma


Other Names:
Thymoma-immunodeficiency syndrome; Good syndrome
Categories:
Good syndrome is a rare, adult-onset primary immunodeficiency suspected in patients who exhibit hypogammaglobulinemia and low levels of B cells along with a benign thymic tumor (thymoma) on chest X-ray.[1][2][3] Symptoms include frequent opportunistic infections involving the sinuses and lungs, including severe CMV disease, P. carinii pneumonia, and mucocutaneous candidiasis.[2][3] While the cause of Good syndrome remains unknown, there is some evidence that a defect of the bone marrow is involved.[3] Treatment includes removal of the thymic tumor and immunoglobulin replacement.[1][2][3]
Last updated: 10/31/2012

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

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Medical Terms Other Names
Learn More:
HPO ID
80%-99% of people have these symptoms
Decreased circulating antibody level 0004313
Mediastinal lymphadenopathy
Swollen lymph nodes in center of chest
0100721
Thymoma 0100522
30%-79% of people have these symptoms
Abnormal leukocyte morphology 0001881
Bronchiectasis
Permanent enlargement of the airways of the lungs
0002110
Cough
Coughing
0012735
Dysphagia
Poor swallowing
Swallowing difficulties
Swallowing difficulty
[ more ] 		0002015
Dysphonia
Inability to produce voice sounds
0001618
Dyspnea
Trouble breathing
0002094
Fatigable weakness 0003473
Ptosis
Drooping upper eyelid
0000508
Recurrent skin infections
Skin infections, recurrent
0001581
Recurrent urinary tract infections
Frequent urinary tract infections
Repeated bladder infections
Repeated urinary tract infections
Urinary tract infections
Urinary tract infections, recurrent
[ more ] 		0000010
Sinusitis
Sinus inflammation
0000246
5%-29% of people have these symptoms
Anemia
Low number of red blood cells or hemoglobin
0001903
Aplasia/Hypoplasia of the thymus
Absent/small thymus
Absent/underdeveloped thymus
[ more ] 		0010515
Diabetes mellitus 0000819
Diarrhea
Watery stool
0002014
Recurrent respiratory infections
Frequent respiratory infections
Multiple respiratory infections
respiratory infections, recurrent
Susceptibility to respiratory infections
[ more ] 		0002205
Thrombocytopenia
Low platelet count
0001873
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Last updated: 7/1/2020
The long-term outlook (prognosis) for individuals with Good syndrome is believed to be worse than for those with other immunodeficiencies. The outlook is affected more by the severity of associated infections, hematologic complications, and autoimmune diseases, rather than by the behavior of the thymoma. In addition, the course of the condition may be more severe for individuals who require immunosuppressive drugs for associated autoimmune complications.

One single center review of individuals with primary antibody deficiency, spanning 20 years, reported that 70% of individuals with Good syndrome were still living 5 years after the diagnosis. At 10 years, only 33% were still living. Another review of 152 cases found significant overall mortality (46%) associated with the condition.[4]
Last updated: 10/31/2012

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • ClinicalTrials.gov lists trials that are related to Immunodeficiency with thymoma. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

    Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

Patient Registry

  • A registry supports research by collecting of information about patients that share something in common, such as being diagnosed with Immunodeficiency with thymoma. The type of data collected can vary from registry to registry and is based on the goals and purpose of that registry. Some registries collect contact information while others collect more detailed medical information. Learn more about registries.

    Registries for Immunodeficiency with thymoma:
    United States Immunodeficiency Network (USIDENT) Registry
     

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Immunodeficiency with thymoma. Click on the link to view a sample search on this topic.

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new question

  • I would like to know the incidence of Good syndrome. I find very limited on-line information and have never encountered a doctor with another patient with this disease, or generally even a doctor who has heard of it. I wonder if there is any possibility of a support community. I would also like to know the prognosis for this disease and its impact on life expectancy. See answer


  1. Other Important Primary Immunodeficiency Diseases. Immune Deficiency Foundation. 2011; http://primaryimmune.org/about-primary-immunodeficiencies/specific-disease-types/other-antibody-deficiency-disorders/. Accessed 12/6/2011.
  2. Tarr PE, Sneller MC, Mechanic LJ, Economides A, Eger CM, Strober W, Cunningham-Rundles C, Lucey DR. Infections in patients with immunodeficiency with thymoma (Good syndrome). Report of 5 cases and review of the literature. Medicine (Baltimore). 2001; http://www.ncbi.nlm.nih.gov/pubmed/11307588. Accessed 12/6/2011.
  3. P Kelleher, SA Misbah. What is Good’s syndrome? Immunological abnormalities in patients with thymoma. J Clin Pathol . 2003; http://jcp.bmj.com/content/56/1/12.full. Accessed 12/6/2011.
  4. Kelesidis T, Yang O. Good's syndrome remains a mystery after 55 years: A systematic review of the scientific evidence. Clin Immunol. June 2010; 135(3):347-363. https://www.ncbi.nlm.nih.gov/pubmed/20149753.