Neutropenia Severe Congenital 9 Autosomal Dominant C5676954
A genetic disorder causing dangerously low neutrophil counts, increasing susceptibility to severe bacterial infections. Treatment involves infection prevention and granulocyte colony-stimulating factor therapy.
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
Frequent bacterial infections
Fever episodes
Skin abscesses
Pneumonia
Generalized symptoms
(hematologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
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.
Granulocyte Colony-Stimulating Factor (G-CSF) Therapy
Growth factor therapy to stimulate neutrophil production and reduce infection risk. This represents the primary treatment approach for severe congenital neutropenia to maintain adequate white blood cell counts.
Antimicrobial Prophylaxis
Preventive antibiotic therapy to reduce bacterial infection risk during neutropenic periods. Prophylactic regimens should be tailored to individual patient history and local resistance patterns.
Additional treatment options
Hematologic Monitoring Program
Regular monitoring of complete blood counts and differential to track response to treatment and detect any changes in disease course. Frequency adjusted based on clinical stability.
monitoringInfection Prevention Strategies
Comprehensive infection prevention including meticulous hand hygiene, food safety practices, and avoidance of known infection sources. Family education is essential for young patients.
lifestyleBone Marrow Assessment
Diagnostic bone marrow evaluation to establish baseline characteristics and monitor for potential malignant transformation, which can occur in some cases of severe congenital neutropenia.
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 9 Autosomal Dominant C5676954?
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