ICD-10 Diagnosis Code D81.5

Purine nucleoside phosphorylase [PNP] deficiency

Diagnosis Code D81.5

ICD-10: D81.5
Short Description: Purine nucleoside phosphorylase [PNP] deficiency
Long Description: Purine nucleoside phosphorylase [PNP] deficiency
This is the 2017 version of the ICD-10-CM diagnosis code D81.5

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)
      • Combined immunodeficiencies (D81)

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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Purine nucleoside phosphorylase deficiency Purine nucleoside phosphorylase deficiency is one of several disorders that damage the immune system and cause severe combined immunodeficiency (SCID). People with SCID lack virtually all immune protection from foreign invaders such as bacteria, viruses, and fungi. Affected individuals are prone to repeated and persistent infections that can be very serious or life-threatening. These infections are often caused by "opportunistic" organisms that ordinarily do not cause illness in people with a normal immune system. Infants with SCID typically grow much more slowly than healthy children and experience pneumonia, chronic diarrhea, and widespread skin rashes. Without successful treatment to restore immune function, children with SCID usually do not survive past early childhood.About two-thirds of individuals with purine nucleoside phosphorylase deficiency have neurological problems, which may include developmental delay, intellectual disability, difficulties with balance and coordination (ataxia), and muscle stiffness (spasticity). People with purine nucleoside phosphorylase deficiency are also at increased risk of developing autoimmune disorders, which occur when the immune system malfunctions and attacks the body's tissues and organs.
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