Immunodeficiency 63 With Lymphoproliferation And Autoimmunity C5193126
A rare genetic immune disorder causing recurrent infections along with overgrowth of immune cells and autoimmune problems. Usually diagnosed in childhood. Requires comprehensive management addressing all aspects.
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
Severe opportunistic infection (e.g., Pneumocystis pneumonia)
Generalized symptoms
(immunologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Recurrent sinopulmonary infections
(respiratory)Autoimmune cytopenias
(hematologic)Lymphadenopathy
(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
IVIG provides essential immune support while also potentially modulating the autoimmune and lymphoproliferative components. Dosing may be adjusted based on infection frequency and autoimmune activity.
Autoimmune Disease Management
Targeted immunosuppressive therapy using corticosteroids, rituximab, or other agents to control autoimmune manifestations while balancing infection risk from immune suppression.
Additional treatment options
Lymphoproliferation Monitoring
Regular imaging studies including CT or PET scans and periodic malignancy screening to detect malignant transformation of proliferating lymphocytes early.
monitoringHematopoietic Stem Cell Transplantation
Definitive curative approach for eligible patients, particularly those with progressive disease or malignancy. Requires careful balancing of timing before organ damage occurs.
procedureChronic Illness Psychological Support
Counseling and support services address the psychological burden of managing a rare immunodeficiency with autoimmune features and potential malignancy concerns.
psychologicalWhen 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 Immunodeficiency 63 With Lymphoproliferation And Autoimmunity C5193126?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
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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