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D45
ICD-10-CM
Polycythemia Vera

Find comprehensive information on Polycythemia Vera, including details on diagnosis, symptoms, treatment, and prognosis. Learn about relevant healthcare terminology for clinical documentation and medical coding related to Polycythemia Vera, such as ICD-10 codes (D45), JAK2 mutation testing, and bone marrow biopsy procedures. Explore resources for healthcare professionals, patients, and caregivers seeking information on managing and understanding Polycythemia Vera. This resource covers essential aspects of Polycythemia Vera diagnosis, including complete blood count (CBC) analysis, hematocrit levels, and red blood cell mass.

Also known as

Primary Polycythemia
Vaquez Disease
vaquez's disease
+3 more

Diagnosis Snapshot

Key Facts
  • Definition : Bone marrow disorder causing overproduction of red blood cells, thickening the blood.
  • Clinical Signs : Headache, dizziness, fatigue, itching, enlarged spleen, redness or bluish skin color.
  • Common Settings : Hematology clinic, primary care, cancer center for diagnosis and treatment monitoring.

Related ICD-10 Code Ranges

Complete code families applicable to AAPC D45 Coding
D45

Polycythemia vera

Primary polycythemia characterized by increased red blood cells.

D75.81

Other specified myeloproliferative disease

Includes myeloproliferative disorders not classified elsewhere.

D47.1

Essential thrombocythemia

Overproduction of platelets, sometimes associated with polycythemia vera.

D75.89

Other specified myelodysplastic disorders

Includes myelodysplastic disorders not elsewhere classified, which can overlap features.

Code-Specific Guidance

Decision Tree for

Follow this step-by-step guide to choose the correct ICD-10 code.

Is the polycythemia due to a known underlying cause?

  • Yes

    Due to altitude or hypoxia?

  • No

    Is it Polycythemia Vera (PV)?

Code Comparison

Related Codes Comparison

When to use each related code

Description
Increased red blood cells
Secondary erythrocytosis
Relative erythrocytosis

Documentation Best Practices

Documentation Checklist
  • Document JAK2 V617F mutation status
  • Hemoglobin >16.5 g/dL (men), >16 g/dL (women)
  • Bone marrow biopsy findings consistent with PV
  • Presence of splenomegaly
  • Exclude secondary causes of erythrocytosis

Coding and Audit Risks

Common Risks
  • Code Specificity

    Incorrect use of unspecified polycythemia vera code (D45.1) when a more specific code for primary or secondary polycythemia is applicable (D45.0, C94.4).

  • Underlying Cause

    Failure to code the underlying cause of secondary polycythemia, leading to inaccurate reporting and potential missed risk adjustment opportunities.

  • Complication Coding

    Missing documentation and coding of associated complications like thrombosis, myelofibrosis, or leukemia, impacting quality metrics and reimbursement.

Mitigation Tips

Best Practices
  • Document JAK2 mutation status for accurate PV diagnosis (ICD-10-CM C94.50).
  • Ensure thorough bloodwork for elevated RBC, Hgb, Hct (CDI best practice).
  • Rule out secondary polycythemia for correct coding and billing compliance.
  • Monitor and document splenomegaly for accurate PV staging and treatment (ICD-10-CM R78.89).
  • Regular phlebotomy documentation supports medical necessity and HCC risk adjustment.

Clinical Decision Support

Checklist
  • 1. Hgb/Hct elevated? Review CBC, check for spurious polycythemia.
  • 2. JAK2 mutation or other clonal marker assessed?
  • 3. Bone marrow biopsy showing hypercellularity?
  • 4. EPO level low? Rule out secondary causes.
  • 5. Document all findings for accurate ICD-10-CM (D45.X) coding.

Reimbursement and Quality Metrics

Impact Summary
  • Polycythemia Vera reimbursement hinges on accurate ICD-10-CM (D45.1) and CPT coding for phlebotomy, bone marrow biopsies, and JAK2 mutation testing. Appropriate documentation is crucial for optimal payer reimbursement.
  • Quality metrics impacted: Hemoglobin/hematocrit control, thrombosis incidence, and patient-reported quality of life measures. Accurate coding facilitates data abstraction for quality reporting and performance benchmarking.
  • Improved coding specificity for PV-related complications (e.g., myelofibrosis, thrombosis) enhances case-mix index and resource allocation.
  • Timely and accurate billing for hydroxyurea or interferon therapy, alongside appropriate modifier use, maximizes Polycythemia Vera reimbursement.

Streamline Your Medical Coding

Let S10.AI help you select the most accurate ICD-10 codes. Our AI-powered assistant ensures compliance and reduces coding errors.

Frequently Asked Questions

Common Questions and Answers

Q: How can I differentiate essential thrombocythemia (ET) from Polycythemia Vera (PV) in patients presenting with elevated hemoglobin and platelet count?

A: Differentiating essential thrombocythemia (ET) from Polycythemia Vera (PV) in patients with elevated hemoglobin and platelets can be challenging. Key distinguishing factors include the presence of JAK2V617F mutation, which is highly prevalent in PV but less common in ET. Bone marrow biopsy findings also play a crucial role, revealing erythroid hyperplasia in PV, while ET shows primarily megakaryocytic proliferation. Furthermore, PV often presents with splenomegaly, whereas splenomegaly is less frequent in ET. Serum erythropoietin (EPO) levels are typically suppressed in PV but normal or even elevated in ET. Consider implementing a diagnostic algorithm incorporating these factors to accurately differentiate between PV and ET. Explore how genetic testing and bone marrow biopsy analysis can contribute to a definitive diagnosis. Learn more about the specific diagnostic criteria outlined by the World Health Organization (WHO).

Q: What are the best management strategies for Polycythemia Vera (PV) beyond phlebotomy, and how can I personalize treatment for individual patients?

A: While phlebotomy remains a cornerstone of Polycythemia Vera (PV) management, additional therapeutic strategies are often necessary, especially for high-risk patients or those experiencing significant symptoms despite phlebotomy. Cytoreductive therapy with agents like hydroxyurea or ruxolitinib can help control blood cell counts and reduce thrombotic risk. Aspirin is often prescribed for low-risk patients to further mitigate thrombotic complications. Personalized treatment for PV involves considering individual patient factors such as age, disease burden, symptom profile, and the presence of cardiovascular risk factors. Explore how risk stratification tools can assist in tailoring treatment decisions and consider implementing a multidisciplinary approach involving hematologists, cardiologists, and other specialists to optimize patient outcomes. Learn more about the latest clinical trials investigating novel therapeutic agents for PV.

Quick Tips

Practical Coding Tips
  • Code D45.1 for PV diagnosis
  • Document JAK2 mutation status
  • Specify primary or secondary PV
  • Include Hgb, Hct, RBC values
  • Consider myeloproliferative neoplasm codes

Documentation Templates

Patient presents with symptoms suggestive of Polycythemia Vera (PV), including erythromelalgia, pruritus, splenomegaly, and generalized fatigue.  The patient reports experiencing burning pain in the hands and feet, exacerbated by heat and relieved by cooling.  Physical examination reveals palpable splenomegaly.  Complete blood count (CBC) demonstrates elevated hemoglobin, hematocrit, and red blood cell count.  White blood cell count and platelet count are also elevated.  The patient denies any history of chronic hypoxemia or other secondary causes of polycythemia.  JAK2 mutation analysis is pending.  Differential diagnosis includes other myeloproliferative neoplasms (MPNs) such as essential thrombocythemia (ET) and primary myelofibrosis (PMF).  Given the clinical presentation and laboratory findings, a preliminary diagnosis of Polycythemia Vera is made.  Phlebotomy is initiated to reduce hematocrit and manage symptoms.  Further evaluation, including bone marrow biopsy and genetic testing, will be performed to confirm the diagnosis and guide treatment decisions.  Patient education regarding the disease process, treatment options, and potential complications of PV was provided.  Follow-up appointment scheduled to review laboratory results and assess response to therapy.  ICD-10 code D45.1, Polycythemia Vera, is assigned.
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