Polycythemia vera is a rare disease of the bone marrow in which there is uncontrolled production of the blood cells (mostly red blood cells), which thickens the blood and can lead to blood clots causing a heart attack or stroke.
ICD-10-CM Code D45: Neoplasms > Neoplasms of uncertain behavior, polycythemia vera and myelodysplastic syndromes
Excludes1: Familial polycythemia (D75.0) Secondary polycythemia (D75.1)
Clinical Manifestations:
Polycythemia vera usually develops slowly, and patients may be asymptomatic for years. It’s often discovered on blood tests performed for other conditions. A patient suffering from polycythemia vera can experience: Headache Dizziness Ringing in the ears Chest pain Itchiness Blurred vision Fatigue Excessive sweating Shortness of breath Numbness, tingling Unintentional weight loss Fever A painful big toe Enlarged spleen Bleeding complications such as easy bruising, bleeding from the gums and/or nose, and internal bleeding.
Diagnosis:
Providers diagnose the disease based on history, symptoms, signs, and physical examination. Laboratory studies include CBC, coagulation studies, and blood for oxygen saturation, vitamin B12 levels, uric acid, and erythropoietin. Bone marrow aspiration and/or biopsy will be performed. The provider may order genetic tests on the blood or bone marrow to identify a mutation in the protein Janus kinase 2 (JAK2), which is present in most patients with polycythemia vera.
Treatment:
There is no cure for polycythemia vera. Treatment consists of: Drawing excess blood out of veins (phlebotomy) to keep the hematocrit below 45% Low-dose aspirin Hydroxyurea or other drugs to reduce the number of red blood cells Antihistamines to reduce itching Splenectomy (removal of the spleen) may be performed.
Untreated, this disease can be fatal.
Clinical Responsibility:
Clinicians have the responsibility to diagnose polycythemia vera, monitor patients for complications, and manage their symptoms through treatment and lifestyle changes.
Code Usage Examples:
Example 1: A patient presents with fatigue, dizziness, and itchy skin. Blood tests reveal an elevated hematocrit and a JAK2 mutation, leading to a diagnosis of polycythemia vera. ICD-10-CM Code: D45
Example 2: A patient with a known history of polycythemia vera presents for a follow-up visit. The provider monitors the patient's hematocrit levels, assesses for symptoms, and discusses medication management. ICD-10-CM Code: D45
Example 3: A patient undergoing a routine blood test is found to have a high hematocrit. Further testing reveals a JAK2 mutation, leading to the diagnosis of polycythemia vera. ICD-10-CM Code: D45
Example 4: A patient is hospitalized with complications from polycythemia vera, including shortness of breath, headache, and a risk of stroke. ICD-10-CM Code: D45
Note: It is essential to assign the correct ICD-10-CM code for polycythemia vera based on the patient's specific clinical presentation and documented findings.