ICD-10: D71

Functional disorders of polymorphonuclear neutrophils

Clinical Information

Inclusion Terms

  • Progressive septic granulomatosis
  • Chronic (childhood) granulomatous disease
  • Congenital dysphagocytosis
  • Cell membrane receptor complex [CR3] defect

Additional Information

Description

Overview of ICD-10 Code D71

ICD-10 code D71 refers to Functional disorders of polymorphonuclear neutrophils. This classification falls under the broader category of blood diseases, specifically focusing on disorders that affect the function of neutrophils, which are a type of white blood cell essential for the immune response.

Clinical Description

Polymorphonuclear neutrophils (PMNs), commonly known as neutrophils, play a critical role in the body's defense against infections, particularly bacterial and fungal pathogens. They are the most abundant type of white blood cells and are a key component of the innate immune system. Functional disorders of these cells can lead to increased susceptibility to infections, impaired inflammatory responses, and various clinical manifestations.

Types of Functional Disorders

  1. Neutrophil Adhesion Deficiency (NAD): This condition is characterized by the inability of neutrophils to adhere to blood vessel walls, which impairs their migration to sites of infection. Patients often experience recurrent bacterial infections.

  2. Chronic Granulomatous Disease (CGD): Although primarily a genetic disorder affecting the ability of neutrophils to produce reactive oxygen species, it can also be classified under functional disorders due to the resultant impaired microbial killing.

  3. Cyclic Neutropenia: This is a rare disorder where neutrophil counts fluctuate cyclically, leading to periods of neutropenia and increased risk of infections.

  4. Hyper-IgE Syndrome: This condition involves elevated levels of immunoglobulin E (IgE) and is associated with recurrent infections due to impaired neutrophil function.

Clinical Manifestations

Patients with functional disorders of PMNs may present with:

  • Recurrent Infections: Frequent bacterial infections, particularly skin and respiratory infections, are common due to the impaired ability of neutrophils to respond effectively.
  • Delayed Wound Healing: Infections can lead to chronic wounds that heal poorly.
  • Inflammatory Responses: Some patients may exhibit exaggerated inflammatory responses, leading to conditions such as abscess formation.

Diagnosis

Diagnosis typically involves:

  • Complete Blood Count (CBC): To assess neutrophil levels.
  • Neutrophil Function Tests: These tests evaluate the ability of neutrophils to migrate, adhere, and produce reactive oxygen species.
  • Genetic Testing: In cases of suspected hereditary conditions, genetic testing may be performed to identify specific mutations.

Treatment

Management of functional disorders of PMNs focuses on:

  • Preventive Antibiotics: To reduce the frequency of infections.
  • Granulocyte Colony-Stimulating Factor (G-CSF): This may be used to stimulate neutrophil production in certain conditions.
  • Immunotherapy: In some cases, therapies aimed at enhancing immune function may be considered.

Conclusion

ICD-10 code D71 encapsulates a range of disorders affecting the functionality of polymorphonuclear neutrophils, leading to significant clinical implications, particularly in terms of infection susceptibility. Understanding these disorders is crucial for timely diagnosis and effective management, ultimately improving patient outcomes. For healthcare providers, recognizing the signs and symptoms associated with these conditions can facilitate early intervention and tailored treatment strategies.

Clinical Information

The ICD-10 code D71 refers to "Functional disorders of polymorphonuclear neutrophils," which are a type of white blood cell crucial for the immune response. Understanding the clinical presentation, signs, symptoms, and patient characteristics associated with this condition is essential for accurate diagnosis and management.

Clinical Presentation

Patients with functional disorders of polymorphonuclear neutrophils typically present with recurrent infections, particularly bacterial infections. These infections may be severe and can occur in various locations, including the skin, respiratory tract, and gastrointestinal system. The clinical presentation can vary significantly depending on the severity of the neutrophil dysfunction and the patient's overall health.

Signs and Symptoms

  1. Recurrent Infections: Patients often experience frequent episodes of infections, which may be atypical in nature. Common infections include:
    - Skin infections (e.g., abscesses)
    - Respiratory infections (e.g., pneumonia)
    - Gastrointestinal infections (e.g., enteritis)

  2. Delayed Wound Healing: Individuals may notice that wounds take longer to heal due to impaired neutrophil function, which is critical for the inflammatory response and tissue repair.

  3. Fever and Inflammation: Patients may present with fever, chills, and signs of systemic inflammation during infection episodes.

  4. Fatigue and Malaise: Chronic infections can lead to general fatigue and a feeling of malaise, impacting the patient's quality of life.

  5. Laboratory Findings: Blood tests may reveal neutrophilia (increased neutrophil count) or neutropenia (decreased neutrophil count), depending on the specific disorder. Functional assays may show impaired neutrophil activity, such as reduced chemotaxis or phagocytosis.

Patient Characteristics

Patients with functional disorders of polymorphonuclear neutrophils may exhibit certain characteristics that can aid in diagnosis:

  • Age: These disorders can occur in individuals of any age, but they may be more commonly diagnosed in children or young adults due to the early onset of recurrent infections.

  • Family History: A family history of immunodeficiency or recurrent infections may be present, suggesting a genetic component to the disorder.

  • Underlying Conditions: Some patients may have associated conditions, such as autoimmune diseases or other immunodeficiencies, which can complicate the clinical picture.

  • Ethnicity and Geography: Certain genetic disorders affecting neutrophil function may be more prevalent in specific ethnic groups or geographic regions, which can influence the clinical approach.

Conclusion

Functional disorders of polymorphonuclear neutrophils, classified under ICD-10 code D71, are characterized by recurrent infections and impaired immune responses. Recognizing the signs and symptoms, along with understanding patient characteristics, is crucial for healthcare providers in diagnosing and managing this condition effectively. Early intervention and appropriate treatment can significantly improve patient outcomes and quality of life.

Approximate Synonyms

The ICD-10 code D71 refers specifically to "Functional disorders of polymorphonuclear neutrophils," which are a type of white blood cell crucial for the immune response. Understanding alternative names and related terms for this condition can enhance clarity in medical documentation and communication. Below are some alternative names and related terms associated with D71.

Alternative Names

  1. Neutrophil Dysfunction: This term broadly describes any impairment in the function of neutrophils, which can lead to increased susceptibility to infections.

  2. Polymorphonuclear Neutrophil Disorders: This phrase encompasses various disorders affecting neutrophils, including functional impairments.

  3. Neutrophil Functional Deficiency: This term highlights the inadequacy in the neutrophils' ability to respond to infections or inflammatory stimuli.

  4. Impaired Neutrophil Function: This is a descriptive term that indicates a reduction in the effectiveness of neutrophils in their immune roles.

  5. Neutrophil Activation Disorders: This term can refer to conditions where neutrophils are either overactive or underactive, affecting their response to pathogens.

  1. Immunodeficiency Disorders: While broader, this category includes conditions where the immune system, including neutrophils, is not functioning optimally, leading to increased infection risk.

  2. Chronic Granulomatous Disease (CGD): A specific genetic disorder that affects neutrophil function, leading to recurrent infections and granuloma formation.

  3. Cyclic Neutropenia: A rare blood disorder characterized by periodic decreases in neutrophil counts, which can affect immune response.

  4. Neutropenia: A condition characterized by low levels of neutrophils, which can be related to functional disorders.

  5. Myeloperoxidase Deficiency: A specific type of neutrophil dysfunction where the enzyme myeloperoxidase is absent, affecting the neutrophils' ability to kill certain bacteria.

  6. Leukocyte Adhesion Deficiency: A condition where neutrophils cannot adhere to blood vessel walls, impairing their ability to migrate to sites of infection.

Conclusion

Understanding the alternative names and related terms for ICD-10 code D71 is essential for healthcare professionals in accurately diagnosing and discussing conditions related to neutrophil dysfunction. These terms not only facilitate better communication among medical practitioners but also enhance patient understanding of their conditions. If you need further information on specific disorders or related ICD-10 codes, feel free to ask!

Diagnostic Criteria

The ICD-10 code D71 refers to "Functional disorders of polymorphonuclear neutrophils," which are a type of white blood cell crucial for the immune response. Diagnosing this condition involves a combination of clinical evaluation, laboratory tests, and specific criteria that help healthcare professionals determine the presence and severity of the disorder.

Diagnostic Criteria for D71

Clinical Evaluation

  1. Patient History: A thorough medical history is essential. This includes any recurrent infections, inflammatory conditions, or other immune-related issues that the patient may have experienced. A family history of similar disorders may also be relevant.

  2. Symptoms: Patients may present with symptoms such as recurrent bacterial infections, delayed wound healing, or other signs of immune dysfunction. These symptoms can guide the clinician in suspecting a functional disorder of neutrophils.

Laboratory Tests

  1. Neutrophil Function Tests: These tests assess the ability of neutrophils to respond to infections. Common tests include:
    - Chemotaxis Assays: Evaluating the movement of neutrophils towards infection sites.
    - Phagocytosis Tests: Measuring the ability of neutrophils to engulf and destroy pathogens.
    - Reactive Oxygen Species (ROS) Production: Assessing the oxidative burst response of neutrophils, which is critical for killing bacteria.

  2. Complete Blood Count (CBC): A CBC can help determine the overall health of the immune system and identify any abnormalities in white blood cell counts.

  3. Bone Marrow Examination: In some cases, a bone marrow biopsy may be performed to evaluate the production of neutrophils and rule out other hematological disorders.

Genetic Testing

In certain cases, genetic testing may be warranted to identify specific inherited conditions that affect neutrophil function. This is particularly relevant in patients with a family history of immune disorders.

Exclusion of Other Conditions

It is crucial to rule out other potential causes of neutrophil dysfunction, such as:
- Acquired Immunodeficiencies: Conditions like HIV/AIDS or the effects of chemotherapy.
- Autoimmune Disorders: Diseases that may affect neutrophil function indirectly.
- Chronic Inflammatory Conditions: Such as rheumatoid arthritis or systemic lupus erythematosus.

Conclusion

The diagnosis of functional disorders of polymorphonuclear neutrophils (ICD-10 code D71) is multifaceted, involving a detailed clinical assessment, specific laboratory tests, and the exclusion of other potential causes. Accurate diagnosis is essential for effective management and treatment of the condition, which may include antibiotics for infections, immunomodulatory therapies, or supportive care depending on the severity and underlying causes of the disorder.

Treatment Guidelines

Functional disorders of polymorphonuclear neutrophils, classified under ICD-10 code D71, encompass a range of conditions characterized by impaired neutrophil function. These disorders can lead to increased susceptibility to infections and other complications due to the neutrophils' critical role in the immune response. Understanding the standard treatment approaches for these disorders is essential for managing patient care effectively.

Overview of Functional Disorders of Polymorphonuclear Neutrophils

Polymorphonuclear neutrophils (PMNs) are a type of white blood cell vital for the body's defense against infections. Functional disorders can arise from genetic defects, autoimmune conditions, or other underlying health issues, leading to neutrophil dysfunction. Symptoms may include recurrent infections, delayed wound healing, and inflammatory responses.

Standard Treatment Approaches

1. Antibiotic Therapy

Patients with functional disorders of neutrophils often experience recurrent infections. Therefore, prompt and appropriate antibiotic therapy is crucial. The choice of antibiotics should be guided by the specific pathogens involved and their susceptibility profiles. Prophylactic antibiotics may also be considered in patients with a history of frequent infections to prevent complications.

2. Granulocyte Colony-Stimulating Factor (G-CSF)

G-CSF is a growth factor that stimulates the production and function of neutrophils. In cases where neutrophil counts are low or their function is impaired, G-CSF can be administered to enhance neutrophil production and improve immune response. This treatment is particularly beneficial in patients with congenital neutropenia or those undergoing chemotherapy.

3. Immunomodulatory Therapies

For patients with autoimmune-related neutrophil dysfunction, immunomodulatory therapies may be indicated. These can include corticosteroids or other immunosuppressive agents to reduce inflammation and modulate the immune response. The specific choice of therapy will depend on the underlying cause of the neutrophil dysfunction.

4. Supportive Care

Supportive care is essential in managing patients with functional disorders of neutrophils. This includes:

  • Infection Prevention: Educating patients on hygiene practices and avoiding exposure to infectious agents.
  • Nutritional Support: Ensuring adequate nutrition to support overall health and immune function.
  • Monitoring and Management of Complications: Regular follow-up to monitor for infections and other complications, adjusting treatment as necessary.

5. Gene Therapy and Experimental Treatments

In cases of inherited forms of neutrophil dysfunction, gene therapy is an emerging area of research. While still largely experimental, these therapies aim to correct the underlying genetic defects causing the functional disorders. Clinical trials may be available for eligible patients.

Conclusion

The management of functional disorders of polymorphonuclear neutrophils requires a comprehensive approach tailored to the individual patient's needs. Standard treatment strategies include antibiotic therapy, G-CSF administration, immunomodulatory therapies, and supportive care. Ongoing research into gene therapy offers hope for more definitive treatments in the future. Regular monitoring and a multidisciplinary approach are essential to optimize patient outcomes and enhance quality of life.

Related Information

Description

Clinical Information

  • Recurrent bacterial infections
  • Severe skin, respiratory, and gastrointestinal infections
  • Delayed wound healing
  • Fever and inflammation during infection episodes
  • Fatigue and malaise due to chronic infections
  • Neutrophilia or neutropenia in blood tests
  • Impaired neutrophil activity in functional assays

Approximate Synonyms

  • Neutrophil Dysfunction
  • Polymorphonuclear Neutrophil Disorders
  • Neutrophil Functional Deficiency
  • Impaired Neutrophil Function
  • Neutrophil Activation Disorders
  • Immunodeficiency Disorders
  • Chronic Granulomatous Disease (CGD)
  • Cyclic Neutropenia
  • Neutropenia
  • Myeloperoxidase Deficiency
  • Leukocyte Adhesion Deficiency

Diagnostic Criteria

  • Thorough medical history required
  • Recurrent infections symptom
  • Delayed wound healing symptom
  • Chemotaxis assays for neutrophil function
  • Phagocytosis tests for pathogen destruction
  • ROS production assessment crucial
  • CBC to evaluate white blood cell counts
  • Bone marrow examination in severe cases
  • Genetic testing for inherited conditions
  • Exclusion of other immune disorders

Treatment Guidelines

  • Prompt antibiotic therapy
  • G-CSF administration for neutrophil enhancement
  • Immunomodulatory therapies for autoimmune causes
  • Supportive care for infection prevention
  • Nutritional support for immune function
  • Monitoring and management of complications
  • Gene therapy and experimental treatments

Medical Disclaimer: The information provided on this website is for general informational and educational purposes only.

It is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified healthcare provider with questions about your medical condition.