Anemia due to enzyme disorders (D55)
D55 codes classify anemia caused by specific enzyme defects affecting red blood cells, leading primarily to types of hemolytic anemia where red blood cells break down prematurely.
The ICD-10 code for anemia due to enzyme disorders covers a range of enzyme deficiencies. For example, D55.0 is used for anemia from glucose-6-phosphate dehydrogenase (G6PD) deficiency, which is also known as Favism or G-6-PD variant enzyme deficiency anemia. Codes like D55.1 address anemia from glutathione metabolism disorders, including hereditary nonspherocytic hemolytic anemia (HNSHA) variants. Other subcodes cover anemia from glycolytic enzyme problems (D55.2 and D55.21 for pyruvate kinase deficiency), nucleotide metabolism issues (D55.3), and less common enzyme-related anemias (D55.8). When the specific enzyme disorder is not identified, D55.9 applies.
Using these specific codes helps healthcare professionals document and track anemia forms linked to enzyme deficiencies, ensuring precise diagnosis and management. Recognizing synonyms like “chronic non-spherocytic hemolytic anemia” or “enzyme deficiency anemia” clarifies code selection when encountering multiple terminology forms in medical records.
Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50–D89)
Hemolytic anemias (D55-D59)
D55 Anemia due to enzyme disorders
- D55.0 Anemia due to glucose-6-phosphate dehydrogenase [G6PD] deficiency
- D55.1 Anemia due to other disorders of glutathione metabolism
D55.2 Anemia due to disorders of glycolytic enzymes
- D55.21 Anemia due to pyruvate kinase deficiency
- D55.29 Anemia due to other disorders of glycolytic enzymes
- D55.3 Anemia due to disorders of nucleotide metabolism
- D55.8 Other anemias due to enzyme disorders
- D55.9 Anemia due to enzyme disorder, unspecified
Anemia due to enzyme disorders (D55)
Instructional Notations
Type 1 Excludes
A type 1 excludes note is a pure excludes note. It means "NOT CODED HERE!" An Excludes1 note indicates that the code excluded should never be used at the same time as the code above the Excludes1 note. An Excludes1 is used when two conditions cannot occur together, such as a congenital form versus an acquired form of the same condition.
- drug-induced enzyme deficiency anemia D59.2
Clinical Terms
The following clinical terms provide additional context, helping users better understand the clinical background and common associations for each diagnosis listed in this section. Including related terms alongside ICD-10-CM codes supports coders, billers, and healthcare professionals in improving accuracy, enhancing documentation, and facilitating research or patient education.
Favism
Hemolytic anemia due to the ingestion of fava beans or after inhalation of pollen from the Vicia fava plant by persons with glucose-6-phosphate dehydrogenase deficient erythrocytes.
