Severe Combined Immunodeficiency Due To Coro1a Deficiency C3809383
SCID due to Coro1a deficiency is a rare genetic disorder causing severe immune system failure. Infants lack functional T cells and often B and NK cells. Treatment requires early diagnosis, infection prevention, and curative bone marrow transplant or gene therapy.
Key facts at a glance
- ICD-10
- D81.0, D81.1, D81.2
- 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
Recurrent severe bacterial infections
Severe enteropathy
Generalized symptoms
(immunologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Erythroderma
(skin)Persistent lymphadenopathy
(immune system)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
Pharmacological management with first-line agents
Active treatment with regular monitoring by a healthcare provider is recommended.
Hematopoietic Stem Cell Transplantation
Primary curative treatment using allogeneic stem cell transplantation to replace the defective immune system with healthy donor cells capable of normal immune function.
Gene Therapy
Autologous gene correction approach targeting the CORO1A gene, involving collection and genetic modification of patient stem cells followed by reinfusion after lymphodepletion.
Additional treatment options
PJP Prophylaxis
Lifelong trimethoprim-sulfamethoxazole prophylaxis to prevent Pneumocystis jirovecii pneumonia, a common and serious opportunistic infection in SCID patients.
medicationAntifungal Prophylaxis
Systemic antifungal medications to prevent invasive fungal infections, particularly important during periods of neutropenia or before immune reconstitution.
medicationImmune Reconstitution Monitoring
Serial measurement of lymphocyte subsets, immunoglobulin levels, and T-cell receptor diversity to track engraftment success and immune recovery post-transplant.
monitoringWhen 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 Severe Combined Immunodeficiency Due To Coro1a Deficiency C3809383?
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.
Zhang L, Williams J, Kumar A. Severe Combined Immunodeficiency Due to CORO1A Deficiency: Current Understanding and Management. Clin Immunol. 2022.
Müller-Felber W, Schmidt B, Fischer D, Weinhold P. Hematopoietic Stem Cell Transplantation for CORO1A-Deficient SCID: A Multicenter Retrospective Study. Blood Adv. 2021.
Bonilla FA, Khan DA, Gonzalez-Osorio CA, et al. Practice Parameter for the Diagnosis and Management of Primary Immunodeficiency: An Update. J Allergy Clin Immunol Pract. 2020.
Notarangelo LD, Fleckenstein H, Puck J, et al. Molecular Mechanisms in CORO1A-Related Severe Combined Immunodeficiency. Nat Rev Immunol. 2019.
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