Absence Of Cd4 Positive Cd25 Positive Regulatory T Cells C4021039
A rare immune deficiency where regulatory T cells are absent, leading to severe autoimmunity and widespread inflammation. Treatment focuses on immunosuppression and managing autoimmune complications.
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.
Generalized symptoms
(immunologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Recurrent infections
(immunologic)Autoimmune enteropathy
(gastrointestinal)Type 1 diabetes mellitus
(endocrine)Severe eczema
(dermatologic)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
Drug therapy and clinical monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Hematopoietic Stem Cell Transplantation
HSCT can restore immune function by replacing the defective T-cell compartment with healthy donor cells. This is the primary curative approach for severe combined immunodeficiency involving regulatory T-cell defects.
Prophylactic Antimicrobial Therapy
Continuous prophylactic antibiotics and antivirals help prevent opportunistic infections while awaiting immune reconstitution. Typically includes TMP-SMX and acyclovir prophylaxis.
Additional treatment options
Immunoglobulin Replacement Therapy
Regular IV or subcutaneous immunoglobulin provides passive humoral immunity to compensate for B-cell dysfunction that may accompany T-cell defects.
medicationInfection Surveillance Protocol
Regular monitoring for viral, bacterial, and fungal infections with prompt laboratory evaluation. Includes periodic imaging and screening for opportunistic pathogens.
monitoringInfection Prevention Measures
Strict hand hygiene, food safety practices, and avoidance of crowds and sick individuals reduces exposure to pathogens. Environmental controls in living spaces are essential.
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 Absence Of Cd4 Positive Cd25 Positive Regulatory T Cells C4021039?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Sakaguchi S, Miyara M, Costantino CM, Hafler DA. FOXP3+ regulatory T cells in the human immune system. Nat Rev Immunol. 2010.
Bach JF. Regulatory T cells under scrutiny. Nat Rev Immunol. 2019.
Josefowicz SZ, Lu LF, Rudensky AY. Regulatory T cells: mechanisms of differentiation and function. Annu Rev Immunol. 2012.
Belkaid Y, Rouse BT. Natural regulatory T cells in infectious disease. Nat Immunol. 2015.
Medication Comparisons
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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