2024 ICD-10-CM Diagnosis Code D89.82
Autoimmune lymphoproliferative syndrome [ALPS]
The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:
- Autoimmune lymphoproliferative syndrome
- Autoimmune lymphoproliferative syndrome
- Autoimmune lymphoproliferative syndrome due to CTLA4 haploinsufficiency
- Autoimmune lymphoproliferative syndrome with recurrent viral infection
- Dianzani autoimmune lymphoproliferative disease
- RAS-associated autoimmune leukoproliferative disease
- STAT3-related early-onset multisystem autoimmune disease
Clinical Category is Immunity disorders
- CCSR Category Code: BLD008
- Inpatient Default CCSR: Y - Yes, default inpatient assignment for principal diagnosis or first-listed diagnosis.
- Outpatient Default CCSR: Y - Yes, default outpatient assignment for principal diagnosis or first-listed diagnosis.
Autoimmune Lymphoproliferative Syndromerare congenital lymphoid disorder due to mutations in certain fas-fas ligand pathway genes. known causes include mutations in fas, tnfsf6, nras, casp8, and casp10 proteins. clinical features include lymphadenopathy; splenomegaly; and autoimmunity.
Index to Diseases and Injuries References
The following annotation back-references for this diagnosis code are found in the injuries and diseases index. The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10-CM code(s).
What are autoimmune diseases?
Your immune system protects you from disease and infection by attacking germs that get into your body, such as viruses and bacteria. Your immune system can tell that the germs aren't part of you, so it destroys them. If you have an autoimmune disease, your immune system attacks the healthy cells of your organs and tissues by mistake.
There are more than 80 types of autoimmune diseases. They can affect almost any part of your body. For example, alopecia areata is an autoimmune disease of the skin that causes hair loss. Autoimmune hepatitis affects the liver. In type 1 diabetes, the immune system attacks the pancreas. And in rheumatoid arthritis, the immune system can attack many parts of the body, including the joints, lungs, and eyes.
What causes autoimmune diseases?
No one is sure why autoimmune diseases happen. But you can't catch them from other people.
Autoimmune diseases do tend to run in families, which means that certain genes may make some people more likely to develop a problem. Viruses, certain chemicals, and other things in the environment may trigger an autoimmune disease if you already have the genes for it.
Who is at risk for autoimmune diseases?
Millions of Americans of all ages have autoimmune diseases. Women develop many types of autoimmune diseases much more often than men. And if you have one autoimmune disease, you are more likely to get another.
What are the symptoms of autoimmune diseases?
The symptoms of an autoimmune disease depend on the part of your body that's affected. Many types of autoimmune diseases cause redness, swelling, heat, and pain, which are the signs and symptoms of inflammation. But other illnesses can cause the same symptoms.
The symptoms of autoimmune diseases can come and go. During a flare-up, your symptoms may get severe for a while. Later on, you may have a remission, which means that your symptoms get better or disappear for a period of time.
How are autoimmune diseases diagnosed?
Doctors often have a hard time diagnosing autoimmune diseases. There's usually not a specific test to show whether you have a certain autoimmune disease. And the symptoms can be confusing. That's because many autoimmune diseases have similar symptoms. And some symptoms, such as muscle aches, are common in many other illnesses. So it can take a long time and some visits to different types of doctors to get a diagnosis.
To help your doctor find out if an autoimmune disease is causing your symptoms,:
- Learn about the health conditions in your family history. What health problems did your grandparents, aunts, uncles, and cousins have? Write down what you learn and share it with your doctor.
- Keep track of your symptoms, including how long they last and what makes them better or worse. Share your notes with your doctor.
- See a specialist who deals with the symptoms that bother you most. For example, if you have rash, see a dermatologist (skin doctor).
What are the treatments for autoimmune diseases?
The treatment depends on the disease. In most cases, the goal of treatment is to suppress (slow down) your immune system, and ease swelling, redness, and pain from inflammation. Your doctor may give you corticosteroids or other medicines to help you feel better. For some diseases, you may need treatment for the rest of your life.
[Learn More in MedlinePlus]
Autoimmune lymphoproliferative syndrome
Autoimmune lymphoproliferative syndrome (ALPS) is an inherited disorder in which the body cannot properly regulate the number of immune system cells (lymphocytes). ALPS is characterized by the production of an abnormally large number of lymphocytes (lymphoproliferation). Accumulation of excess lymphocytes results in enlargement of the lymph nodes (lymphadenopathy), the liver (hepatomegaly), and the spleen (splenomegaly).
Autoimmune disorders are also common in ALPS. Autoimmune disorders occur when the immune system malfunctions and attacks the body's own tissues and organs. Most of the autoimmune disorders associated with ALPS target and damage blood cells. For example, the immune system may attack red blood cells (autoimmune hemolytic anemia), white blood cells (autoimmune neutropenia), or platelets (autoimmune thrombocytopenia). Less commonly, autoimmune disorders that affect other organs and tissues occur in people with ALPS. These disorders can damage the kidneys (glomerulonephritis), liver (autoimmune hepatitis), eyes (uveitis), or nerves (Guillain-Barre syndrome). Skin problems, usually rashes or hives (urticaria), can also occur in ALPS.
ALPS can have varying patterns of signs and symptoms. Most commonly, lymphoproliferation becomes apparent during childhood. Enlargement of the lymph nodes and spleen frequently occur in affected individuals. Autoimmune disorders typically develop several years later, most frequently as a combination of hemolytic anemia and thrombocytopenia, also called Evans syndrome. People with this classic form of ALPS generally have a near-normal lifespan, but have a greatly increased risk of developing cancer of the immune system cells (lymphoma) compared with the general population.
Some people have signs and symptoms that resemble those of ALPS, including lymphoproliferation, lymphadenopathy, splenomegaly, and low blood counts, but the specific pattern of these signs and symptoms or the genetic cause may be different. Researchers disagree whether individuals with these non-classic forms should be considered to have ALPS or a separate condition.
[Learn More in MedlinePlus]
- FY 2024 - No Change, effective from 10/1/2023 through 9/30/2024
- 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. This was the first year ICD-10-CM was implemented into the HIPAA code set.
 Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:
- The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
- The condition places limitations on self-care, independent living, and social interactions.