D81.2 - Severe combined immunodeficiency [SCID] with low or normal B-cell numbers

Version 2023

ICD-10:	D81.2
Short Description:	Severe combined immunodef w low or normal B-cell numbers
Long Description:	Severe combined immunodeficiency [SCID] with low or normal B-cell numbers
Status:	Valid for Submission
Version:	ICD-10-CM 2023
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) Combined immunodeficiencies (D81)

1. Approximate Synonyms
2. Tabular List of Diseases and Injuries
3. Index to Diseases and Injuries References
4. Diagnostic Related Groups - MS-DRG Mapping
5. Convert to ICD-9 Code
6. Patient Education
7. Related Codes Browser
8. Code History

D81.2 is a billable ICD-10 code used to specify a medical diagnosis of severe combined immunodeficiency [scid] with low or normal b-cell numbers. The code is valid during the fiscal year 2023 from October 01, 2022 through September 30, 2023 for the submission of HIPAA-covered transactions.

Approximate Synonyms

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

Severe combined immunodeficiency with low or normal B-cell numbers
T cell negative B cell positive severe combined immunodeficiency due to gamma chain deficiency

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
- - combined - D81.9
  - - severe (SCID) - D81.9
    - - with
      - - low or normal B-cell numbers - D81.2

Diagnostic Related Groups - MS-DRG Mapping

The is diagnosis code is grouped in the following groups for version MS-DRG V40.0 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 10/01/2022 through 09/30/2023.

MS-DRG	MS-DRG Title	MCD	Relative Weight
808	MAJOR HEMATOLOGICAL AND IMMUNOLOGICAL DIAGNOSES EXCEPT SICKLE CELL CRISIS AND COAGULATION DISORDERS WITH MCC	16	2.141
809	MAJOR HEMATOLOGICAL AND IMMUNOLOGICAL DIAGNOSES EXCEPT SICKLE CELL CRISIS AND COAGULATION DISORDERS WITH CC	16	1.2157
810	MAJOR HEMATOLOGICAL AND IMMUNOLOGICAL DIAGNOSES EXCEPT SICKLE CELL CRISIS AND COAGULATION DISORDERS WITHOUT CC/MCC	16	0.9388

The relative weight of a diagnostic related group determines the reimbursement rate based on the severity of a patient's illness and the associated cost of care during hospitalization.

Convert to ICD-9 Code

Source ICD-10 Code		Target ICD-9 Code
D81.2		279.2 - Combined immunity defic
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.

Patient Education

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]

Omenn syndrome

Omenn syndrome is an inherited disorder of the immune system (immunodeficiency). Omenn syndrome is one of several forms of severe combined immunodeficiency (SCID), a group of disorders that cause individuals to have virtually no immune protection from bacteria, viruses, and fungi. Individuals with SCID are prone to repeated and persistent infections that can be very serious or life-threatening. Infants with Omenn syndrome typically experience pneumonia and chronic diarrhea. Often the organisms that cause infection in people with this disorder are described as opportunistic because they ordinarily do not cause illness in healthy people.

In addition to immunodeficiency, children with Omenn syndrome develop autoimmunity, in which the immune system attacks the body's own tissues and organs. This abnormal immune reaction can cause very red skin (erythroderma), hair loss (alopecia), and an enlarged liver and spleen (hepatosplenomegaly). In addition, affected individuals have enlargement of tissues that produce infection-fighting white blood cells called lymphocytes. These include the thymus, which is a gland located behind the breastbone, and lymph nodes, which are found throughout the body.

If not treated in a way that restores immune function, children with Omenn syndrome usually survive only until age 1 or 2.