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    OpenPayerBilling CodesHemolytic anemiasICD-10-CM d55-anemia-due-to-enzyme-disorders
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    ICD-10-CM Code For Anemia Due To Enzyme Disorders: Group Overview

    The International Classification of Diseases, Tenth Revision, Clinical Modification (ICD-10-CM) code group D55 includes inherited and acquired hemolytic anemias due to enzyme deficiencies such as glucose-6-phosphate dehydrogenase deficiency and other erythrocyte enzyme disorders that lead to hemolysis. These ICD-10-CM diagnosis codes are used to document episodes of hemolytic anemia, specify the underlying enzyme disorder, and capture associated severity or complications such as acute hemolytic crises or chronic hemolysis. Accurate coding within the ICD-10-CM D55 group supports proper claim adjudication and complete clinical documentation and appropriate reimbursement.

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    ICD-10-CM D55: Anemia Due To Enzyme Disorders Overview

    This group represents hemolytic anemias caused by inherited or acquired enzyme deficiencies that impair red blood cell metabolism and survival. These conditions affect the hematologic system and can present with varying severity of anemia and hemolysis. Accurate coding matters for appropriate clinical documentation and for correct reimbursement and claim processing.

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    When to Use

    Use a code from this group when documentation confirms an enzyme disorder as the cause of hemolytic anemia, such as laboratory identification of enzyme deficiency or genetic testing. Assign specific codes when the clinical record notes acute hemolytic episodes, chronic hemolysis, or complications attributable to the enzyme disorder.

    ICD-10-CM Codes

    7 rows

    ICD-10-CM CodeDescription
    D55.0Anemia due to glucose-6-phosphate dehydrogenase [G6PD] deficiency
    D55.1Anemia due to other disorders of glutathione metabolism
    D55.21Anemia due to pyruvate kinase deficiency
    D55.29Anemia due to other disorders of glycolytic enzymes
    D55.3Anemia due to disorders of nucleotide metabolism
    D55.8Other anemias due to enzyme disorders
    D55.9Anemia due to enzyme disorder, unspecified

    DRG Codes

    2 rows

    DRG CodeDescription
    811Red Blood Cell Disorders with MCC
    812Red Blood Cell Disorders without MCC

    Typical Clinical Scenarios

    • An adult of Mediterranean descent presents to the emergency department with sudden onset jaundice, dark urine, fatigue, and back pain after taking a sulfonamide antibiotic. Laboratory evaluation shows acute hemolytic anemia with elevated indirect bilirubin and low haptoglobin consistent with a drug-induced hemolysis due to a red cell enzyme sensitivity; a diagnosis from this group is assigned to capture the acute, exposure-related enzyme deficiency causing hemolysis. Typical codes: D55.0, D55.1
    • A middle-aged patient with known chronic glucose-6-phosphate dehydrogenase deficiency is seen in primary care for routine management after episodes of episodic hemolysis triggered by infections and certain foods; current exam shows mild pallor but stable vital signs. This visit documents the underlying hereditary enzyme disorder as a chronic condition that predisposes to recurrent hemolytic episodes and guides ongoing monitoring and counseling, so a code from this group is used to reflect the chronic enzyme-related anemia. Typical codes: D55.3, D55.9
    • A hospitalized patient with sickle cell trait is admitted for severe infection and develops persistent anemia requiring transfusion; workup identifies hemolysis attributable to an acquired enzyme inhibitor causing ongoing red cell destruction as a complication of the acute illness. Coding from this group is applied to indicate the enzyme-related hemolytic process as a comorbidity contributing to the patient’s anemia and need for higher level care. Typical codes: D55.21, D55.29

    Related Codes

    D56-THALASSEMIA

    ICD-10-CM Code For Thalassemia: Group Overview

    D57-SICKLE-CELL-DISORDERS

    ICD-10-CM Code For Sickle-Cell Disorders: Group Overview

    D58-OTHER-HEREDITARY-HEMOLYTIC-ANEMIAS

    ICD-10-CM Code For Other Hereditary Hemolytic Anemias: Group Overview

    D59-ACQUIRED-HEMOLYTIC-ANEMIA

    ICD-10-CM Code For Acquired Hemolytic Anemia: Group Overview

    Frequently Asked Questions

    References

    1. 1.CPT® Codes and Descriptions. AAPC. Retrieved Aug 11, 2026.Maintains the CPT code set's official descriptions and guidelines, licensed from the American Medical Association, used as the definitional source for CPT codes on this page.
    2. 2.HCPCS Quarterly Update. Centers for Medicare & Medicaid Services (CMS). Retrieved Aug 11, 2026.CMS's quarterly release of HCPCS Level II codes and short/long descriptors, the source for HCPCS code definitions and update history.
    3. 3.ICD-10-CM Full Code List. Centers for Medicare & Medicaid Services (CMS). Retrieved Aug 11, 2026.CMS's complete ICD-10-CM diagnosis code list, used to cross-reference diagnosis codes associated with this procedure or service.
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