Myeloproliferative Neoplasms ICD-10 Codes & Diagnosis Overview

What Causes MPNs?

Abnormal blood cell production in the bone marrow is commonly due to chromosomal mutations in the blood stem cells. Dr. Abu-Zeinah adds, "the overproduction of blood cells,  can reflect in a routine blood count that a patient has".  What causes this mutation is not well understood. Although MPN trends are sometimes seen in families, the condition is not considered to be hereditary. The mutations often appear at random or by-chance. 

Environmental toxins may also contribute to the genetic mutation of the stem cells, but toxin exposure is difficult to assess. 

Subtypes of MPN

There are several different MPN subtypes that are all the result of abnormal levels of blood cells and blood-forming tissue. However, the types differ in terms of which cells are affected. Most conditions can alter the levels of a few different blood cell types, but typically one type is more affected than the others. This influences the way each of these conditions is diagnosed. 

The six common categories include the following:

• Chronic myeloid leukemia results from abnormally high white blood cell production.
• Chronic eosinophilic leukemia is an excess of eosinophils, which are a specific type of white blood cell.
• Chronic neutrophilic leukemia refers to an over-supply of neutrophils, which are another specific type of white blood cell. 
• Essential thrombocythemia results from too many platelets.
• Polycythemia vera is an abnormally high production of red blood cells.
• Primary myelofibrosis results when the fibers that typically support blood cell development and immature blood cells collect in the bone marrow. 

Dr. Adrienne Philips, a Hematologist/Medical Oncologist, at Weill-Cornell Medicine, told SurvivorNet."Symptoms that are indicative of a myeloproliferative neoplasm can vary depending on when a patient is first diagnosed" 

How are Myeloproliferative Disorders Diagnosed?

This group of rare blood cancers is typically first diagnosed later in a patient's life, although they can also be found in the adolescent population. Diagnosis is typically done using diagnostic testing, including blood tests, bone marrow aspirations, and biopsies. Other tests may be recommended as well by your healthcare team.

The most commonly prescribed diagnostic tests for MPN include the following:

• Complete blood count
• Bone marrow biopsy and aspiration
• Peripheral blood smear
• Genetic testing
• Blood chemistry
• Physical exam and medical history

Complete Blood Count

Complete blood count (CBC) with differential is a common diagnostic test. It requires a simple blood draw, then the sample is sent to a laboratory for review. This sample gives your healthcare team an estimate of the quantities of red blood cells, white blood cells, and platelets. This test also provides insight into the percent of the blood that is made up of red blood cells and a measurement of the hemoglobin in red blood cells. Hemoglobin is responsible for carrying oxygen throughout the body. This is the simplest test to perform to quantify blood cell levels. 

Bone Marrow Biopsy and Aspiration

To perform this procedure, a hollow needle is inserted into the back of the hip and a small sample of the bone marrow is removed. A biopsy requires a small piece of the solid tissue whereas an aspiration is a fluid sample. For this procedure to be performed, there is a need for local anesthesia. Once the sample is collected, it will be sent to a laboratory where the abnormal cells can be evaluated under a microscope. 

Peripheral Blood Smear

A peripheral blood smear also requires a blood sample, but unlike a CBC, this test is used to examine the appearance and shape of abnormal cells. It can also help to identify immature cells, sometimes referred to as blast cells, that are not able to function properly due to being under-developed state.  

Genetic Tests

Genetic testing is used to identify chromosomal mutations (like JAK2, CALR, or MPL) in stem cells that cause this dysregulation of blood cell development. This is sometimes referred to as a cytogenetic analysis. Fully understanding the genetic component of MPN cancer types not only provides insight into the disease as a whole, but also helps to guide treatment options on an individualized basis. It can also help estimate a patient's prognosis. 

Blood Chemistry

Blood chemistry testing examines certain substances that are found in the body. This helps your healthcare team understand how various organs or organ systems are working overall. Abnormal levels would be expected with certain disease states, including MPNs. This type of test also requires a simple blood sample. 

Physical Exam and Comprehensive Medical History

An overall physical exam and medical history evaluation will help your healthcare team assess your health status and whether there are any other disease states to consider. Additionally, this type of work-up at initial diagnosis provides baseline health status prior to treatment or therapy initiation. 

ICD-10 Codes Associated with Myeloproliferative Neoplasms

International Classification of Diseases, or ICD codes, give healthcare professionals a way to accurately describe a disease state to communicate with other medical professionals and bill for services. The code consists of a certain standardized sequence of numbers and letters. 

How Do ICD-10 Codes Work?

In order for healthcare providers to receive reimbursement for a particular service, visit, or procedure, they must submit the ICD-10 code, which describes the patient's condition, along with a common procedural technology (CPT) code for the specific action performed. Insurance companies use this combination of codes to determine reimbursement. 

If the CPT code describes an action that is typical for a given disease state, or ICD code, they will send a payment according to the patient's medical plan. If the CPT is for a service that is not standard for the ICD code condition, the insurance company will likely request further information before sending payment to the healthcare provider. 

This communication would be between your physician's reimbursement team and insurance coverage company, but patients may see this code on bills, treatment summaries, or progress notes. Understanding how your condition is categorized by your healthcare team will help keep you informed. The Center for Disease Control and Prevention (CDC) has a searchable database of ICD-10 codes available to the general public. 

Note that ICD-10 codes are in the process of being updated to ICD-11, the next generation of disease classification codes. ICD codes are typically updated every 1-2 years since healthcare is continuously changing. 

ICD-10 Codes for MPNs

The ICD-10 code for myeloproliferative neoplasm is D47.1. What exactly is this code describing? The initial letter of the code gives information on the disease state in general terms. For example, all infectious or parasitic conditions start with either an 'A' or 'B.' 

All neoplasms, and blood-related organs and conditions start with a 'D.' MPNs are further characterized by numbers. The subtypes of MPNs also have their own ICD code, such as essential thrombocythemia, which has the associated code: D47.3. 

Moving Forward Questions to Ask your Doctor

• What type of myeloproliferative neoplasm do I have? 

• What tests need to be performed to diagnose my condition?

• What treatment options are available for me?
• What ICD-10 code is associated with my specific condition?

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