ICD-10 Diagnosis Code D80.0

Hereditary hypogammaglobulinemia

Diagnosis Code D80.0

ICD-10: D80.0
Short Description: Hereditary hypogammaglobulinemia
Long Description: Hereditary hypogammaglobulinemia
This is the 2017 version of the ICD-10-CM diagnosis code D80.0

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 with predominantly antibody defects (D80)

Information for Medical Professionals

Diagnostic Related Groups
The diagnosis code D80.0 is grouped in the following Diagnostic Related Group(s) (MS-DRG v33.0)


Convert to ICD-9 Additional informationCallout TooltipGeneral Equivalence Map
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.
  • 279.04 - Cong hypogammaglobulinem

  • Agammaglobulinemia
  • Autosomal agammaglobulinemia with absent B-cells
  • Autosomal recessive hyperimmunoglobulin M syndrome
  • Congenital agammaglobulinemia
  • Congenital hypogammaglobulinemia
  • Humoral immune defect
  • Humoral immune defect
  • Humoral immune defect
  • Selective immunoglobulin dysfunction
  • Selective immunoglobulin dysfunction
  • X-linked agammaglobulinemia
  • X-linked agammaglobulinemia with growth hormone deficiency
  • X-linked hyperimmunoglobulin M syndrome
  • X-linked hypogammaglobulinemia

Index of Diseases and Injuries
References found for the code D80.0 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 agammaglobulinemia X-linked agammaglobulinemia (XLA) is a condition that affects the immune system and occurs almost exclusively in males. People with XLA have very few B cells, which are specialized white blood cells that help protect the body against infection. B cells can mature into the cells that produce special proteins called antibodies or immunoglobulins. Antibodies attach to specific foreign particles and germs, marking them for destruction. Individuals with XLA are more susceptible to infections because their body makes very few antibodies.Children with XLA are usually healthy for the first 1 or 2 months of life because they are protected by antibodies acquired before birth from their mother. After this time, the maternal antibodies are cleared from the body, and the affected child begins to develop recurrent infections. In children with XLA, infections generally take longer to get better and then they come back again, even with antibiotic medications. The most common bacterial infections that occur in people with XLA are lung infections (pneumonia and bronchitis), ear infections (otitis), pink eye (conjunctivitis), and sinus infections (sinusitis). Infections that cause chronic diarrhea are also common. Recurrent infections can lead to organ damage. People with XLA can develop severe, life-threatening bacterial infections; however, affected individuals are not particularly vulnerable to infections caused by viruses. With treatment to replace antibodies, infections can usually be prevented, improving the quality of life for people with XLA.
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Isolated growth hormone deficiency Isolated growth hormone deficiency is a condition caused by a severe shortage or absence of growth hormone. Growth hormone is a protein that is necessary for the normal growth of the body's bones and tissues. Because they do not have enough of this hormone, people with isolated growth hormone deficiency commonly experience a failure to grow at the expected rate and have unusually short stature. This condition is usually apparent by early childhood.There are four types of isolated growth hormone deficiency differentiated by the severity of the condition, the gene involved, and the inheritance pattern.Isolated growth hormone deficiency type IA is caused by an absence of growth hormone and is the most severe of all the types. In people with type IA, growth failure is evident in infancy as affected babies are shorter than normal at birth.People with isolated growth hormone deficiency type IB produce very low levels of growth hormone. As a result, type IB is characterized by short stature, but this growth failure is typically not as severe as in type IA. Growth failure in people with type IB is usually apparent in early to mid-childhood.Individuals with isolated growth hormone deficiency type II have very low levels of growth hormone and short stature that varies in severity. Growth failure in these individuals is usually evident in early to mid-childhood. It is estimated that nearly half of the individuals with type II have underdevelopment of the pituitary gland (pituitary hypoplasia). The pituitary gland is located at the base of the brain and produces many hormones, including growth hormone.Isolated growth hormone deficiency type III is similar to type II in that affected individuals have very low levels of growth hormone and short stature that varies in severity. Growth failure in type III is usually evident in early to mid-childhood. People with type III may also have a weakened immune system and are prone to frequent infections. They produce very few B cells, which are specialized white blood cells that help protect the body against infection (agammaglobulinemia).
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