Autoimmune Lymphoproliferative Syndrome

Effective October 1, 2009, code 279.4, Autoimmune disease, not elsewhere classified, has been expanded to uniquely identify autoimmune lymphoproliferative syndrome (ALPS). Autoimmune lymphoproliferative syndrome (ALPS) is an inherited disorder of the immune system. It can affect both children and adults. In ALPS, unusually high numbers of lymphocytes accumulate in the lymph nodes, liver, and spleen, which can lead to enlargement of these organs. ALPS can cause numerous autoimmune problems. These problems include anemia, thrombocytopenia, and neutropenia. There is no cure for ALPS. However, most of its complications can be treated with splenectomy, steroids, or blood transfusions. 279 Disorders...

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Note:  The following article synopsis was NOT provided by the AHA. It was created by Find-A-Code/innoviHealth.

Article Overview

This article covers a coding update for autoimmune lymphoproliferative syndrome within the autoimmune disease category and explains the broader clinical context of the condition. It is relevant to coders, billers, and healthcare professionals working with immune-system disorders and diagnosis coding changes. The article also presents the affected diagnosis category and the newly identified subcategory in the code set.

Why This Topic Matters

It helps readers understand a diagnosis coding change tied to a specific immune disorder and its placement within the classification structure, which can affect accurate code selection and reporting.

Article Sections

  1. Clinical Overview

    Provides a brief overview of the disorder and its general effects on the immune system and lymphatic organs.

  2. Coding Update

    Summarizes the diagnosis classification change and the affected portion of the immune-mechanism chapter.

What You Will Learn

  • The general clinical context of autoimmune lymphoproliferative syndrome
  • That the article addresses a diagnosis coding update in an immune-system category
  • How the condition is positioned within a broader classification change
  • What kind of audience would find the update relevant

Who Should Read This

  • Medical coders
  • Medical billers
  • Coding educators
  • Healthcare documentation staff
  • Clinicians reviewing diagnosis coding updates

Codes Discussed

  • ICD-9-CM: 279.4
  • ICD-9-CM: 279.41
  • ICD-9-CM: 279.49

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