ICD-10 Diagnosis Code D82.3

Immunodef fol heredit defctv response to Epstein-Barr virus

Diagnosis Code D82.3

ICD-10: D82.3
Short Description: Immunodef fol heredit defctv response to Epstein-Barr virus
Long Description: Immunodeficiency following hereditary defective response to Epstein-Barr virus
This is the 2017 version of the ICD-10-CM diagnosis code D82.3

Code Classification
  • Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism
    • Certain disorders involving the immune mechanism (D80-D89)
      • Immunodeficiency associated with other major defects (D82)

Information for Medical Professionals

Diagnostic Related Groups
The diagnosis code D82.3 is grouped in the following Diagnostic Related Group(s) (MS-DRG v33.0)


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The ICD-10 and ICD-9 GEMs are used to facilitate linking between the diagnosis codes in ICD-9-CM and the new ICD-10-CM code set. The GEMs are the raw material from which providers, health information vendors and payers can derive specific applied mappings to meet their needs.

  • Immunodeficiency following hereditary defective response to Epstein-Barr virus
  • Immunodeficiency with major anomalies
  • X-linked immunodeficiency with magnesium defect, Epstein-Barr virus infection and neoplasia
  • X-linked lymphoproliferative syndrome

Index of Diseases and Injuries
References found for the code D82.3 in the Index of Diseases and Injuries:

Information for Patients

Immune System and Disorders

Your immune system is a complex network of cells, tissues, and organs that work together to defend against germs. It helps your body to recognize these "foreign" invaders. Then its job is to keep them out, or if it can't, to find and destroy them.

If your immune system cannot do its job, the results can be serious. Disorders of the immune system include

  • Allergy and asthma - immune responses to substances that are usually not harmful
  • Immune deficiency diseases - disorders in which the immune system is missing one or more of its parts
  • Autoimmune diseases - diseases causing your immune system to attack your own body's cells and tissues by mistake

NIH: National Institute of Allergy and Infectious Diseases

  • Agammaglobulinemia
  • Aging changes in immunity
  • Chronic granulomatous disease
  • Graft-versus-host disease
  • Histiocytosis
  • Hyperimmunoglobulin E syndrome
  • Immune response
  • Immunodeficiency disorders
  • Selective deficiency of IgA

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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.
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