ICD-10: D72.118
Other hypereosinophilic syndrome
Clinical Information
Inclusion Terms
- Gleich's syndrome
- Episodic angioedema with eosinophilia
Additional Information
Description
The ICD-10 code D72.118 refers to "Other hypereosinophilic syndrome," a condition characterized by an elevated eosinophil count in the blood, which can lead to various clinical manifestations. Below is a detailed overview of this syndrome, including its clinical description, symptoms, diagnosis, and management.
Clinical Description
Hypereosinophilic syndrome (HES) is defined by the presence of persistent eosinophilia, which is an elevated level of eosinophils, a type of white blood cell involved in the body's immune response. Eosinophils play a crucial role in combating parasitic infections and are also involved in allergic reactions and asthma. In HES, the eosinophil count exceeds 1,500 cells per microliter of blood for an extended period, typically more than six months, without an identifiable cause.
Types of Hypereosinophilic Syndrome
HES can be classified into several categories:
- Primary HES: This form is often associated with a clonal proliferation of eosinophils, which may be linked to genetic mutations, such as those affecting the FIP1L1-PDGFRA fusion gene.
- Secondary HES: This occurs due to other underlying conditions, such as infections, allergies, autoimmune diseases, or malignancies.
- Idiopathic HES: In some cases, the cause remains unknown despite extensive investigation.
Symptoms
The symptoms of D72.118 can vary widely depending on the organs affected by the eosinophilia. Common manifestations include:
- Dermatological Symptoms: Rashes, pruritus (itching), and urticaria (hives).
- Respiratory Symptoms: Asthma-like symptoms, cough, and wheezing due to eosinophilic infiltration in the lungs.
- Gastrointestinal Symptoms: Abdominal pain, diarrhea, and weight loss, which may occur if the gastrointestinal tract is involved.
- Cardiac Symptoms: Eosinophilic myocarditis can lead to chest pain, palpitations, and heart failure in severe cases.
- Neurological Symptoms: Neuropathy or encephalitis may occur if the nervous system is affected.
Diagnosis
Diagnosing hypereosinophilic syndrome involves several steps:
- Complete Blood Count (CBC): A CBC will reveal elevated eosinophil levels.
- Exclusion of Secondary Causes: It is essential to rule out other conditions that can cause eosinophilia, such as infections, allergies, and malignancies. This may involve additional blood tests, imaging studies, and possibly bone marrow biopsy.
- Clinical Evaluation: A thorough clinical history and physical examination are crucial to assess symptoms and organ involvement.
Management
Management of D72.118 focuses on controlling eosinophil levels and addressing symptoms. Treatment options may include:
- Corticosteroids: These are often the first line of treatment to reduce eosinophil counts and manage inflammation.
- Immunosuppressive Agents: Medications such as azathioprine or methotrexate may be used in cases resistant to steroids.
- Targeted Therapies: For patients with specific genetic mutations, targeted therapies like imatinib may be effective.
- Symptomatic Treatment: This may include antihistamines for itching or bronchodilators for respiratory symptoms.
Conclusion
Other hypereosinophilic syndrome (ICD-10 code D72.118) is a complex condition that requires careful diagnosis and management. Understanding the underlying causes and potential complications is essential for effective treatment. Patients with this syndrome should be monitored regularly to manage symptoms and prevent complications effectively. If you suspect hypereosinophilic syndrome, it is crucial to consult a healthcare professional for a comprehensive evaluation and tailored treatment plan.
Clinical Information
Hypereosinophilic syndrome (HES) is a rare condition characterized by an elevated eosinophil count in the blood, which can lead to various clinical manifestations. The ICD-10 code D72.118 specifically refers to "Other hypereosinophilic syndrome," which encompasses cases that do not fit into the more defined categories of HES. Below is a detailed overview of the clinical presentation, signs, symptoms, and patient characteristics associated with this condition.
Clinical Presentation
Definition and Overview
Hypereosinophilic syndrome is defined by a sustained eosinophil count greater than 1,500 eosinophils per microliter of blood, along with associated symptoms or organ damage. The "other" designation in D72.118 indicates that the syndrome may not be linked to a specific underlying cause, such as a known malignancy or autoimmune disorder.
Patient Characteristics
Patients with other hypereosinophilic syndrome may present with a variety of characteristics, including:
- Age: HES can occur in individuals of any age, but it is most commonly diagnosed in adults.
- Gender: There is a slight male predominance in cases of hypereosinophilia.
- Underlying Conditions: Some patients may have a history of allergies, asthma, or other atopic conditions, although this is not universally the case.
Signs and Symptoms
Common Symptoms
Patients with other hypereosinophilic syndrome may exhibit a range of symptoms, which can vary significantly based on the organs affected. Common symptoms include:
- Dermatological Manifestations: Skin rashes, pruritus (itching), and urticaria (hives) are frequently reported. Eosinophilic infiltration can lead to conditions such as eosinophilic fasciitis.
- Respiratory Symptoms: Patients may experience asthma-like symptoms, including wheezing, shortness of breath, and cough due to eosinophilic infiltration in the lungs.
- Gastrointestinal Symptoms: Abdominal pain, diarrhea, and eosinophilic esophagitis can occur, leading to dysphagia (difficulty swallowing) and other gastrointestinal disturbances.
- Cardiac Symptoms: Eosinophilic infiltration of the heart can lead to cardiomyopathy, arrhythmias, and heart failure, which may present as chest pain or palpitations.
- Neurological Symptoms: Neurological involvement can manifest as peripheral neuropathy, headaches, or seizures, depending on the extent of eosinophilic infiltration in the nervous system.
Systemic Effects
In severe cases, the systemic effects of eosinophilia can lead to complications such as:
- Organ Damage: Prolonged eosinophilia can result in damage to various organs, including the heart, lungs, liver, and kidneys.
- Thromboembolic Events: Increased eosinophil levels can lead to hypercoagulability, resulting in thromboembolic complications.
Diagnosis and Evaluation
Diagnosis of other hypereosinophilic syndrome typically involves:
- Complete Blood Count (CBC): To confirm elevated eosinophil levels.
- Bone Marrow Biopsy: May be performed to rule out hematological malignancies.
- Imaging Studies: Such as echocardiograms or CT scans, to assess organ involvement.
- Allergy Testing: To identify potential allergic triggers.
Conclusion
Other hypereosinophilic syndrome (ICD-10 code D72.118) presents a complex clinical picture characterized by elevated eosinophil levels and a diverse array of symptoms affecting multiple organ systems. Early recognition and management are crucial to prevent potential complications associated with prolonged eosinophilia. Given the variability in presentation, a thorough evaluation and tailored treatment plan are essential for optimal patient outcomes.
Approximate Synonyms
The ICD-10 code D72.118 refers to "Other hypereosinophilic syndrome," which is a classification used in the International Statistical Classification of Diseases and Related Health Problems (ICD). This code is part of a broader category of conditions characterized by elevated eosinophil levels in the blood, which can lead to various health complications.
Alternative Names for Other Hypereosinophilic Syndrome
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Hypereosinophilia: This term broadly describes the condition of having an elevated eosinophil count, which can be a feature of various underlying disorders, including the specific syndrome denoted by D72.118.
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Secondary Hypereosinophilia: This term may be used when the hypereosinophilia is a result of another condition, such as infections, allergies, or malignancies.
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Eosinophilic Syndrome: A more general term that can encompass various forms of hypereosinophilia, including those classified under D72.118.
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Idiopathic Hypereosinophilia: This term is often used when the cause of the elevated eosinophil count is unknown, which can sometimes overlap with the classification of "Other hypereosinophilic syndrome."
Related Terms and Conditions
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Chronic Eosinophilic Leukemia (CEL): A rare type of blood cancer characterized by the overproduction of eosinophils, which may be related to or confused with hypereosinophilic syndromes[6].
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Eosinophilic Granulomatosis with Polyangiitis (EGPA): A condition that involves eosinophilia and can lead to systemic vasculitis, which may be relevant in discussions of hypereosinophilic syndromes[6].
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Allergic Eosinophilic Esophagitis: A condition characterized by eosinophilic infiltration of the esophagus, often related to food allergies, which can be a part of the broader spectrum of eosinophilic disorders.
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Eosinophilic Pneumonia: A condition where eosinophils accumulate in the lungs, leading to respiratory symptoms, which may also be associated with hypereosinophilia.
Conclusion
Understanding the alternative names and related terms for ICD-10 code D72.118 is crucial for accurate diagnosis and treatment. The terminology surrounding hypereosinophilic syndromes can vary, and recognizing these terms can aid healthcare professionals in identifying and managing the condition effectively. If you have further questions or need more specific information, feel free to ask!
Diagnostic Criteria
The diagnosis of hypereosinophilic syndrome (HES), specifically under the ICD-10 code D72.118 for "Other hypereosinophilic syndrome," involves a comprehensive evaluation based on clinical criteria, laboratory findings, and exclusion of other conditions. Here’s a detailed overview of the criteria used for diagnosis:
Clinical Criteria for Diagnosis
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Eosinophilia:
- A key feature of HES is the presence of eosinophilia, which is defined as an elevated eosinophil count in the blood. Typically, this is characterized by an eosinophil count greater than 1,500 cells per microliter of blood, sustained over a period of time[1]. -
Symptoms:
- Patients may present with a variety of symptoms that can include:- Skin rashes or lesions
- Respiratory symptoms such as asthma or cough
- Gastrointestinal symptoms like abdominal pain or diarrhea
- Neurological symptoms, which may include peripheral neuropathy or other neurological deficits[1][2].
-
Duration:
- The eosinophilia must be persistent, usually defined as lasting for more than six months, and not attributable to other known causes such as allergies, infections, or malignancies[1].
Laboratory Findings
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Bone Marrow Biopsy:
- A bone marrow biopsy may be performed to assess for eosinophil proliferation and to rule out other hematological disorders, such as myeloproliferative neoplasms[2]. -
Exclusion of Secondary Causes:
- It is crucial to exclude secondary causes of eosinophilia, which can include:- Allergic reactions
- Parasitic infections
- Autoimmune diseases
- Certain malignancies, particularly hematological cancers[1][2].
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Additional Testing:
- Further tests may include imaging studies to evaluate organ involvement, such as echocardiograms for cardiac involvement or CT scans for pulmonary assessment[2].
Diagnostic Criteria Summary
The diagnosis of D72.118 is made when:
- There is persistent eosinophilia (>1,500 cells/µL).
- Symptoms consistent with HES are present.
- Other potential causes of eosinophilia have been ruled out through clinical evaluation and laboratory testing.
Conclusion
Diagnosing other hypereosinophilic syndrome (ICD-10 code D72.118) requires a thorough clinical assessment, including the evaluation of eosinophil levels, symptomatology, and exclusion of secondary causes. This comprehensive approach ensures that patients receive an accurate diagnosis and appropriate management for their condition. If you have further questions or need more specific information, feel free to ask!
Treatment Guidelines
Hypereosinophilic syndrome (HES) is characterized by an elevated eosinophil count in the blood and associated symptoms or organ damage. The ICD-10 code D72.118 specifically refers to "Other hypereosinophilic syndrome," which encompasses various forms of HES that do not fit into more specific categories. Treatment approaches for HES can vary based on the underlying cause, severity of symptoms, and the presence of organ involvement. Below is a detailed overview of standard treatment approaches for this condition.
Understanding Hypereosinophilic Syndrome
Definition and Symptoms
Hypereosinophilic syndrome is defined by a persistent eosinophilia (elevated eosinophil levels) greater than 1,500 eosinophils per microliter of blood for six months or longer, accompanied by symptoms such as:
- Skin rashes or lesions
- Respiratory issues (e.g., asthma, cough)
- Gastrointestinal symptoms (e.g., abdominal pain, diarrhea)
- Cardiac complications (e.g., myocarditis, pericarditis)
Classification
HES can be classified into primary (idiopathic), secondary (due to other conditions), and familial forms. The treatment approach may differ based on the classification and the specific symptoms presented.
Standard Treatment Approaches
1. Corticosteroids
Corticosteroids are often the first line of treatment for HES. They help reduce eosinophil counts and alleviate symptoms. Commonly used corticosteroids include:
- Prednisone
- Methylprednisolone
The dosage and duration depend on the severity of the condition and the response to treatment. Long-term use may require careful monitoring due to potential side effects.
2. Cytotoxic Agents
In cases where corticosteroids are ineffective or if there is significant organ involvement, cytotoxic agents may be employed. These include:
- Hydroxyurea: This medication can help lower eosinophil counts and is often used in conjunction with corticosteroids.
- Interferon-alpha: This treatment can also reduce eosinophil levels and is particularly useful in patients with severe symptoms.
3. Targeted Therapies
For patients with specific genetic mutations or those who do not respond to traditional therapies, targeted therapies may be considered. These include:
- Imatinib: Particularly effective in cases associated with the FIP1L1-PDGFRα fusion gene.
- Mepolizumab: An anti-IL-5 monoclonal antibody that specifically targets eosinophils, reducing their production and survival.
4. Supportive Care
Supportive care is crucial in managing symptoms and preventing complications. This may involve:
- Management of asthma or other respiratory issues with bronchodilators or inhaled corticosteroids.
- Treatment of skin lesions with topical corticosteroids or other dermatological therapies.
- Monitoring and managing cardiac symptoms, which may require cardiology consultation.
5. Regular Monitoring
Patients with HES require regular follow-up to monitor eosinophil levels and assess organ function. This may include:
- Complete blood counts (CBC) to track eosinophil levels.
- Imaging studies (e.g., echocardiograms) to evaluate organ involvement.
Conclusion
The management of hypereosinophilic syndrome, particularly under the ICD-10 code D72.118, involves a multifaceted approach tailored to the individual patient's needs. Corticosteroids remain the cornerstone of treatment, while cytotoxic agents and targeted therapies provide options for more severe cases. Regular monitoring and supportive care are essential to optimize outcomes and manage complications effectively. As research continues, new therapies may emerge, offering hope for improved management of this complex condition.
Related Information
Description
- Elevated eosinophil count in the blood
- Typically exceeds 1,500 cells per microliter
- Persistent eosinophilia for more than six months
- No identifiable cause required
- Primary HES: clonal proliferation of eosinophils
- Secondary HES: due to underlying conditions
- Idiopathic HES: unknown cause
Clinical Information
- Elevated eosinophil count greater than 1500
- Sustained eosinophilia leads to organ damage
- Skin rashes and pruritus are common symptoms
- Respiratory symptoms include wheezing and shortness of breath
- Gastrointestinal symptoms include abdominal pain and diarrhea
- Cardiac symptoms include cardiomyopathy and arrhythmias
- Neurological symptoms include peripheral neuropathy and seizures
Approximate Synonyms
- Hypereosinophilia
- Secondary Hypereosinophilia
- Eosinophilic Syndrome
- Idiopathic Hypereosinophilia
Diagnostic Criteria
- Eosinophilia >1500 cells/µL
- Persistent symptoms (>6 months)
- Ruling out secondary causes
- Bone marrow biopsy for evaluation
- Exclusion of allergies and infections
- Autoimmune diseases excluded
- Malignancies ruled out
Treatment Guidelines
- Corticosteroids reduce eosinophil counts
- Hydroxyurea lowers eosinophil levels
- Imatinib targets FIP1L1-PDGFRα fusion gene
- Mepolizumab reduces eosinophil production
- Supportive care manages symptoms and complications
- Regular monitoring tracks eosinophil levels and organ function
Related Diseases
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