Hyper Igm Syndrome Type 3 C1720957
A primary immune disorder where infection-fighting cells cannot switch antibody types properly, leaving patients vulnerable to repeated infections. Treatment involves antibody replacement and infection prevention.
Key facts at a glance
- ICD-10
- D89.9
- ICD-11
- 3A00.0
- Body system
- immunologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Pneumocystis jirovecii pneumonia
Generalized symptoms
(immunologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Recurrent sinopulmonary infections
(immunologic)Chronic diarrhea due to Cryptosporidium infection
(immunologic)Autoimmune cytopenias
(immunologic)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Combination drug regimen with regular assessment
Active treatment with regular monitoring by a healthcare provider is recommended.
Immunoglobulin Replacement Therapy
Lifelong immunoglobulin replacement therapy via intravenous or subcutaneous routes to compensate for the class-switching defect and provide protective antibodies against encapsulated bacteria and other pathogens.
Pneumocystis jirovecii Prophylaxis
Trimethoprim-sulfamethoxazole as first-line prophylaxis against PCP, which is a major cause of morbidity in Hyper-IgM syndrome type 3 patients due to severely impaired T-cell function.
Additional treatment options
Hematopoietic Stem Cell Transplantation
Curative approach through allogeneic hematopoietic stem cell transplantation to replace the defective immune system with healthy donor cells capable of proper immunoglobulin class switching.
procedureComprehensive Immunologic Monitoring
Regular monitoring of immunoglobulin levels, lymphocyte subsets, and antibody responses to vaccines to assess disease progression and treatment efficacy, enabling timely therapeutic adjustments.
monitoringLive Vaccine Avoidance
Strict avoidance of live attenuated vaccines including MMR, varicella, and rotavirus in patients and household contacts to prevent vaccine-derived infections in immunocompromised individuals.
lifestyleWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Hyper Igm Syndrome Type 3 C1720957?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalMedication Comparisons
Compare medications used to treat Hyper Igm Syndrome Type 3 C1720957. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy