MCMedicalCodingSoftware
E88.01

E88.01: Alpha-1-antitrypsin deficiency

Billable / specific codeICD-10-CM

E88.01 is a billable, specific ICD-10-CM code for alpha-1-antitrypsin deficiency. It is valid for submission on a claim.

Check E88.01 with the rest of the claim: free scrubber

Inclusion terms

Alternative wordings a physician may use in the chart for this same code.

  • AAT deficiency

Excludes1 inherited from the categories above E88.01

A category’s note binds every code beneath it.

Written on E88.0: Disorders of plasma-protein metabolism, not elsewhere classified

  • monoclonal gammopathy (of undetermined significance) (D47.2)
  • polyclonal hypergammaglobulinemia (D89.0)
  • Waldenström macroglobulinemia (C88.00)

Written on E88: Other and unspecified metabolic disorders

  • histiocytosis X (chronic) (C96.6)

Excludes2 inherited from the categories above E88.01

Not part of this code, but both may be coded when both are present.

Written on E88.0: Disorders of plasma-protein metabolism, not elsewhere classified

  • disorder of lipoprotein metabolism (E78.-)

Risk adjustment

E88.01 risk-adjusts. Under the CMS-HCC V28 model it maps to this category:

  • HCC 50 Amyloidosis, Porphyria, and Other Specified Metabolic Disorders0.648 RAF

Coefficient shown for the Community Non-dual Aged segment. A higher HCC in the same hierarchy will suppress this one, so capturing it does not always add score. Look up the whole chart to see which of its HCCs the hierarchy would zero.

Medicare coverage

E88.01 is named in 4 Medicare coverage policies: 4 listing it as supporting medical necessity. Which one applies depends on your Medicare contractor.

  • A56199Billing and Coding: Molecular Pathology Procedures
  • A56717Billing and Coding: Respiratory Therapy (Respiratory Care)
  • A57224Billing and Coding: Respiratory Care
  • A60409Billing and Coding: Molecular Pathology Procedures

Alphabetic index entries that lead here

  • Deficiency, deficient›alpha-1-antitrypsin
  • Deficiency, deficient›serum antitrypsin, familial

Code history

Unchanged in every release from FY2024 to FY2027.

Other codes under E88.0

ICD-10-CMFY2027· effective October 1, 2026

Loaded directly from the CMS/NCHS ICD-10-CM public-domain release: see data sources and our editorial policy. If this page disagrees with the CMS tabular list, this page is wrong. Reference information for professional coders; not medical or billing advice.