D82.3 is a billable ICD-10 code used to specify a medical diagnosis of immunodeficiency following hereditary defective response to epstein-barr virus. The code is valid during the fiscal year 2023 from October 01, 2022 through September 30, 2023 for the submission of HIPAA-covered transactions.
The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:
- Immunodeficiency following hereditary defective response to Epstein-Barr virus
- X-linked immunodeficiency with magnesium defect, Epstein-Barr virus infection and neoplasia
- X-linked lymphoproliferative disease due to SH2D1A deficiency
- X-linked lymphoproliferative disease due to XIAP deficiency
- X-linked lymphoproliferative syndrome
Tabular List of Diseases and Injuries
The Tabular List of Diseases and Injuries is a list of ICD-10 codes, organized "head to toe" into chapters and sections with coding notes and guidance for inclusions, exclusions, descriptions and more. The following references are applicable to this diagnosis code:
Inclusion TermsInclusion Terms
These terms are the conditions for which that code is to be used. The terms may be synonyms of the code title, or, in the case of "other specified" codes, the terms are a list of the various conditions assigned to that code. The inclusion terms are not necessarily exhaustive. Additional terms found only in the Alphabetic Index may also be assigned to a code.
- X-linked lymphoproliferative disease
Index to Diseases and Injuries References
The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10 code(s). The following references for this diagnosis code are found in the injuries and diseases index:
- - Immunodeficiency - D84.9
Convert to ICD-9 Code
|Source ICD-10 Code||Target ICD-9 Code|
|D82.3||279.8 - Immune mechanism dis NEC|
|Approximate Flag - The approximate mapping means there is not an exact match between the ICD-10 and ICD-9 codes and the mapped code is not a precise representation of the original code.|
Immune System and Disorders
What is the immune system?
Your immune system is a complex network of cells, tissues, and organs. Together they help the body fight infections and other diseases.
When germs such as bacteria or viruses invade your body, they attack and multiply. This is called an infection. The infection causes the disease that makes you sick. Your immune system protects you from the disease by fighting off the germs.
What are the parts of the immune system?
The immune system has many different parts, including:
- Your skin, which can help prevent germs from getting into the body
- Mucous membranes, which are the moist, inner linings of some organs and body cavities. They make mucus and other substances which can trap and fight germs.
- White blood cells, which fight germs
- Organs and tissues of the lymph system, such as the thymus, spleen, tonsils, lymph nodes, lymph vessels, and bone marrow. They produce, store, and carry white blood cells.
How does the immune system work?
Your immune system defends your body against substances it sees as harmful or foreign. These substances are called antigens. They may be germs such as bacteria and viruses. They might be chemicals or toxins. They could also be cells that are damaged from things like cancer or sunburn.
When your immune system recognizes an antigen, it attacks it. This is called an immune response. Part of this response is to make antibodies. Antibodies are proteins that work to attack, weaken, and destroy antigens. Your body also makes other cells to fight the antigen.
Afterwards, your immune system remembers the antigen. If it sees the antigen again, it can recognize it. It will quickly send out the right antibodies, so in most cases, you don't get sick. This protection against a certain disease is called immunity.
What are the types of immunity?
There are three different types of immunity:
- Innate immunity is the protection that you are born with. It is your body's first line of defense. It includes barriers such as the skin and mucous membranes. They keep harmful substances from entering the body. It also includes some cells and chemicals which can attack foreign substances.
- Active immunity, also called adaptive immunity, develops when you are infected with or vaccinated against a foreign substance. Active immunity is usually long-lasting. For many diseases, it can last your entire life.
- Passive immunity happens when you receive antibodies to a disease instead of making them through your own immune system. For example, newborn babies have antibodies from their mothers. People can also get passive immunity through blood products that contain antibodies. This kind of immunity gives you protection right away. But it only lasts a few weeks or months.
What can go wrong with the immune system?
Sometimes a person may have an immune response even though there is no real threat. This can lead to problems such as allergies, asthma, and autoimmune diseases. If you have an autoimmune disease, your immune system attacks healthy cells in your body by mistake.
Other immune system problems happen when your immune system does not work correctly. These problems include immunodeficiency diseases. If you have an immunodeficiency disease, you get sick more often. Your infections may last longer and can be more serious and harder to treat. They are often genetic disorders.
There are other diseases that can affect your immune system. For example, HIV is a virus that harms your immune system by destroying your white blood cells. If HIV is not treated, it can lead to AIDS (acquired immunodeficiency syndrome). People with AIDS have badly damaged immune systems. They get an increasing number of severe illnesses.
[Learn More in MedlinePlus]
X-linked lymphoproliferative disease
X-linked lymphoproliferative disease (XLP) is a disorder of the immune system and blood-forming cells that is found almost exclusively in males. More than half of individuals with this disorder experience an exaggerated immune response to the Epstein-Barr virus (EBV). EBV is a very common virus that eventually infects most humans. In some people it causes infectious mononucleosis (commonly known as "mono"). Normally, after initial infection, EBV remains in certain immune system cells (lymphocytes) called B cells. However, the virus is generally inactive (latent) because it is controlled by other lymphocytes called T cells that specifically target EBV-infected B cells.
People with XLP may respond to EBV infection by producing abnormally large numbers of T cells, B cells, and other lymphocytes called macrophages. This proliferation of immune cells often causes a life-threatening reaction called hemophagocytic lymphohistiocytosis. Hemophagocytic lymphohistiocytosis causes fever, destroys blood-producing cells in the bone marrow, and damages the liver. The spleen, heart, kidneys, and other organs and tissues may also be affected. In some individuals with XLP, hemophagocytic lymphohistiocytosis or related symptoms may occur without EBV infection.
About one-third of people with XLP experience dysgammaglobulinemia, which means they have abnormal levels of some types of antibodies. Antibodies (also known as immunoglobulins) are proteins that attach to specific foreign particles and germs, marking them for destruction. Individuals with dysgammaglobulinemia are prone to recurrent infections.
Cancers of immune system cells (lymphomas) occur in about one-third of people with XLP.
Without treatment, most people with XLP survive only into childhood. Death usually results from hemophagocytic lymphohistiocytosis.
XLP can be divided into two types based on its genetic cause and pattern of signs and symptoms: XLP1 (also known as classic XLP) and XLP2. People with XLP2 have not been known to develop lymphoma, are more likely to develop hemophagocytic lymphohistiocytosis without EBV infection, usually have an enlarged spleen (splenomegaly), and may also have inflammation of the large intestine (colitis). Some researchers believe that these individuals should actually be considered to have a similar but separate disorder rather than a type of XLP.
[Learn More in MedlinePlus]
- FY 2023 - No Change, effective from 10/1/2022 through 9/30/2023
- FY 2022 - No Change, effective from 10/1/2021 through 9/30/2022
- FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021
- FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
- FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
- FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
- FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
- FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016 (First year ICD-10-CM implemented into the HIPAA code set)