Neutropenia Severe Congenital 8 Autosomal Dominant C5203411
A genetic disorder causing dangerously low white blood cell counts. Patients experience frequent severe infections and are treated with medications to boost white blood cell production.
Key facts at a glance
- ICD-10
- D64.9
- ICD-11
- 3A00.0
- Body system
- hematologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Recurrent bacterial infections
Recurrent fever
Severe periodontitis
Generalized symptoms
(hematologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Oral ulcers
(hematologic)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
Granulocyte Colony-Stimulating Factor (G-CSF) Therapy
Growth factor therapy to stimulate neutrophil production and reduce infection risk. This is the standard first-line treatment for severe congenital neutropenia to maintain adequate white blood cell counts.
Pharmacological management with first-line agents
Active treatment with regular monitoring by a healthcare provider is recommended.
Antibiotic Prophylaxis
Preventive antibiotic therapy to reduce bacterial infection risk, particularly during periods of severe neutropenia. Choice of antibiotic should be individualized based on history and sensitivities.
Additional treatment options
Regular Complete Blood Count Monitoring
Frequent CBC monitoring to track neutrophil counts and adjust treatment accordingly. More intensive monitoring during illness or treatment changes.
monitoringInfection Prevention Measures
Strict hand hygiene, avoiding contact with sick individuals, and maintaining good general hygiene practices. Education for patient and family on infection recognition and prevention.
lifestyleBone Marrow Evaluation
Diagnostic bone marrow examination to confirm diagnosis, assess cellularity, and rule out other conditions. May be repeated if transformation is suspected.
procedureWhen 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 Neutropenia Severe Congenital 8 Autosomal Dominant C5203411?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalMedication 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