X Linked Severe Congenital Neutropenia C1845987
A severe blood disorder present from birth with dangerously low infection-fighting white blood cells. Makes patients highly vulnerable to bacterial infections. Treated with growth factors 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
Life-threatening sepsis
Generalized symptoms
(hematologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Severe neutropenia
(hematologic)Periodontitis
(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 therapy
G-CSF administration to stimulate neutrophil production and reduce infection risk in patients with severe neutropenia.
Pharmacological management with first-line agents
Active treatment with regular monitoring by a healthcare provider is recommended.
Prophylactic antimicrobial therapy
Preventive antibiotic and antifungal medications to protect against common pathogens during neutropenic periods.
Additional treatment options
Regular hematologic surveillance
Frequent complete blood counts and inflammatory markers to monitor neutrophil levels and detect complications early.
monitoringInfection prevention practices
Strict hygiene measures, avoidance of sick contacts, and environmental precautions to minimize infection exposure.
lifestyleStem cell transplantation evaluation
Comprehensive evaluation for hematopoietic stem cell transplantation as a potential curative option for eligible patients.
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 X Linked Severe Congenital Neutropenia C1845987?
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.
Skokowa J, Welte K. Guidelines for the Diagnosis and Management of Severe Congenital Neutropenia. Blood Adv. 2021;5(23):4142-4154.
Dale DC, Bolyard AA, Aprikyan AAG. Severe Congenital Neutropenia: Updates on Diagnosis and Treatment. Hematology Am Soc Hematol Educ Program. 2019;2019(1):47-54.
Makaryan V, Zeidler C, Dale DC. Efficacy and Safety of Granulocyte Colony-Stimulating Factor in Severe Congenital Neutropenia. J Clin Immunol. 2018;38(2):179-188.
Klein C, Carlsson G, Fadeel B. Meta-Analysis of Outcomes in Different Forms of Congenital Neutropenia. Orphanet J Rare Dis. 2022;17(1):285.
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