ICD-10-CM 2027
ICD-10 code for Acanthocytosis
From the official ICD-10-CM alphabetic index entry “Acanthocytosis”. Page updated September 29, 2026.
About coding Acanthocytosis
The ICD-10-CM code for Acanthocytosis is E78.6 (Lipoprotein deficiency).
Within E78 (disorders of lipoprotein metabolism and other lipidemias), E78.6 is specifically for lipoprotein deficiency. Related codes cover pure hypercholesterolemia (E78.0), pure hyperglyceridemia (E78.1), mixed hyperlipidemia (E78.2), Hyperchylomicronemia (E78.3), other hyperlipidemia (E78.4), hyperlipidemia, unspecified (E78.5), disorders of bile acid and cholesterol metabolism (E78.7), other disorders of lipoprotein metabolism (E78.8) and 1 more.
It is not a CC or MCC, so as a secondary diagnosis it doesn't change the MS-DRG severity level.
In MS-DRG v44.0, it is part of the grouping logic for DRG 642 (Inborn and Other Disorders of Metabolism, relative weight 1.3219), in MDC 10 (Endocrine, Nutritional and Metabolic Diseases and Disorders), as a principal or secondary diagnosis. The DRG a claim lands in depends on the principal diagnosis, procedures and any CC/MCC secondary diagnoses.
Common questions about Acanthocytosis ICD-10 codes
What is the ICD-10 code for Acanthocytosis?
- E78.6 — Lipoprotein deficiency.
Is E78.6 billable?
- Yes. E78.6 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can E78.6 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict E78.6 as a principal diagnosis.
Is E78.6 a CC or MCC?
- No. E78.6 is neither a CC nor an MCC under MS-DRG v44.0.