Discover free eBooks, guides and med spa templates on our new resources page

Book a demo
☰
Why us
ICD Hub / Diseases of the immune system (D80–D89) / 4A00.0Z - Functional neutrophil defects, unspecified
ICD-11

ICD-11 Codes
for 4A00.0Z - Functional neutrophil defects, unspecified
Teodor Jurukovski January 1, 1970
Fact checked by: Teodor Jurukovski
Use Code

Alternative Names for Functional Neutrophil Defects

Functional neutrophil defects, unspecified, may also be referred to by several other terms in clinical practice and literature. These include:
  • Functional neutrophil disorders
  • Severe recurrent infections with present neutrophils
  • Congenital dysphagocytosis
  • Disorders of polymorphonuclear neutrophils
  • Granulomatous disease or disorder

Understanding Functional Neutrophil Defects

Functional neutrophil defects refer to a group of inherited disorders affecting the innate immune system. Neutrophils are crucial white blood cells responsible for combating bacterial and fungal infections. When these cells have functional defects, their ability to migrate to infection sites, engulf pathogens (phagocytosis), or kill them effectively is impaired. This can lead to a significantly increased susceptibility to severe and recurrent infections. The ICD-11 classification system addresses these conditions under specific codes, with `4A00.0Z` representing unspecified functional neutrophil defects.

Diagnosing Functional Neutrophil Defects

Diagnosing functional neutrophil defects typically involves a comprehensive evaluation. This includes a detailed medical history focusing on recurrent infections and a physical examination. Laboratory investigations are key and may include:
  • Complete blood count (CBC) to assess neutrophil numbers.
  • Specialised neutrophil function tests, such as phagocytosis assays, oxidative burst tests (e.g., using nitroblue tetrazolium or dihydrorhodamine), and chemotaxis studies.
  • Genetic testing may be employed to identify specific gene mutations associated with certain inherited neutrophil disorders.

Signs and Symptoms of Neutrophil Dysfunction

Individuals with functional neutrophil defects often present with recurrent, severe, or life-threatening infections. These can manifest as:
  • Frequent skin infections
  • Persistent or recurrent respiratory tract infections, such as pneumonia or bronchitis
  • Oral ulcers or gum disease
  • Delayed healing of wounds or injuries
  • In some cases, symptoms may include granuloma formation.
The severity and type of infections can vary depending on the specific nature and extent of the neutrophil dysfunction.

ICD-11 Coding for Functional Neutrophil Defects

The official ICD-11 code for unspecified functional neutrophil defects is `4A00.0Z`. This code falls under the category of Primary immunodeficiencies due to disorders of innate immunity. Accurate coding is essential for medical billing, statistical reporting, and clinical documentation. When a specific functional neutrophil defect is identified, more precise codes may be applicable, such as `4A00.00` for Neutrophil immunodeficiency syndrome or `4A00.0Y` for Other specified functional neutrophil defects.

Managing Neutrophil Dysfunction

Management of functional neutrophil defects primarily focuses on preventing and treating infections. This may involve:
  • Prophylactic antibiotics or antifungals to reduce the risk of infection.
  • Prompt and aggressive treatment of active infections.
  • Granulocyte colony-stimulating factor (G-CSF) may be used in certain cases to increase neutrophil production.
  • In severe, refractory cases, hematopoietic stem cell transplantation (HSCT) might be considered.
The specific treatment plan is tailored to the individual patient's condition and the severity of their immune compromise.

Frequently asked questions

What is the ICD-11 classification meaning of 4A00.0Z "Functional neutrophil defects, unspecified"?
This code represents disorders affecting the function of neutrophils when the specific nature of the functional defect is not documented. It encompasses conditions where neutrophils do not perform their intended functions, such as phagocytosis or killing microorganisms, without further specification.
Under what circumstances is the ICD-11 code 4A00.0Z "Functional neutrophil defects, unspecified" appropriate for documentation?
This code is used when a patient has a documented functional defect of neutrophils, but the specific type of defect is not detailed in the clinical documentation, or when it does not meet the criteria for any other more specific functional neutrophil defect code.
How does ICD-11 differentiate between "Functional neutrophil defects, unspecified" (4A00.0Z) and other functional neutrophil defects?
ICD-11 provides specific codes for particular functional neutrophil defects, such as Neutrophil immunodeficiency syndrome (4A00.00) or Other specified functional neutrophil defects (4A00.0Y). The unspecified code (4A00.0Z) is used when the defect is identified but not precisely characterized by these more specific categories.
×