ICD-10-CM Code D80.0

Hereditary hypogammaglobulinemia

Version 2020 Billable Code

Valid for Submission

D80.0 is a billable code used to specify a medical diagnosis of hereditary hypogammaglobulinemia. The code is valid for the year 2020 for the submission of HIPAA-covered transactions. The ICD-10-CM code D80.0 might also be used to specify conditions or terms like abnormal gamma globulin level, abnormal gamma globulin level, agammaglobulinemia, agammaglobulinemia, microcephaly, craniosynostosis, severe dermatitis syndrome, autosomal agammaglobulinemia with absent b-cells, congenital agammaglobulinemia, etc

ICD-10:D80.0
Short Description:Hereditary hypogammaglobulinemia
Long Description:Hereditary hypogammaglobulinemia

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 guidance for inclusions, exclusions, descriptions and more. The following references are applicable to the code D80.0:

Inclusion Terms

Inclusion 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.
  • Autosomal recessive agammaglobulinemia (Swiss type)
  • X-linked agammaglobulinemia Bruton (with growth hormone deficiency)

Index to Diseases and Injuries

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 the code D80.0 are found in the index:


Synonyms

The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

  • Abnormal gamma globulin level
  • Abnormal gamma globulin level
  • Agammaglobulinemia
  • Agammaglobulinemia, microcephaly, craniosynostosis, severe dermatitis syndrome
  • Autosomal agammaglobulinemia with absent B-cells
  • Congenital agammaglobulinemia
  • Congenital hypogammaglobulinemia
  • Humoral immune defect
  • Hypogammaglobulinemia
  • Isolated agammaglobulinemia
  • Microcephaly, hypogammaglobulinemia, abnormal immunity syndrome
  • Osteopetrosis hypogammaglobulinemia syndrome
  • Specific antibody deficiency
  • X-linked agammaglobulinemia
  • X-linked agammaglobulinemia with growth hormone deficiency
  • X-linked hypogammaglobulinemia
  • X-linked intellectual disability with hypogammaglobulinemia and progressive neurological deterioration syndrome

Diagnostic Related Groups

The ICD-10 code D80.0 is grouped in the following groups for version MS-DRG V37.0 What are Diagnostic Related Groups?
The Diagnostic Related Groups (DRGs) are a patient classification scheme which provides a means of relating the type of patients a hospital treats. The DRGs divides all possible principal diagnoses into mutually exclusive principal diagnosis areas referred to as Major Diagnostic Categories (MDC).
applicable from 10/01/2019 through 09/30/2020.

  • 814 - RETICULOENDOTHELIAL AND IMMUNITY DISORDERS WITH MCC
  • 815 - RETICULOENDOTHELIAL AND IMMUNITY DISORDERS WITH CC
  • 816 - RETICULOENDOTHELIAL AND IMMUNITY DISORDERS WITHOUT CC/MCC

Convert D80.0 to ICD-9

  • 279.04 - Cong hypogammaglobulinem

Code Classification

  • Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50–D89)
    • Certain disorders involving the immune mechanism (D80-D89)
      • Immunodeficiency with predominantly antibody defects (D80)

Code History

  • FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016
    (First year ICD-10-CM implemented into the HIPAA code set)
  • FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
  • FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
  • FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
  • FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020

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

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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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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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