E70.331Hermansky-Pudlak syndrome

E70.331 is the ICD-10-CM diagnosis code for Hermansky-Pudlak syndrome. It belongs to category E70 (disorders of aromatic amino-acid metabolism), block E70-E88 (metabolic disorders) and chapter 4 (endocrine, nutritional and metabolic diseases). It is billable, so it can be reported on its own for dates of service from October 1, 2026 through September 30, 2027.

Within E70.33 (albinism with hematologic abnormality), E70.331 is specifically for Hermansky-Pudlak syndrome. Related codes cover Chediak-Higashi syndrome (E70.330), other albinism with hematologic abnormality (E70.338) and unspecified (E70.339).

As a secondary diagnosis it is a CC (complication or comorbidity), which can move an inpatient stay into the "with CC" MS-DRG of its family, unless the principal diagnosis excludes it.

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.

It has been part of ICD-10-CM since the code set took effect on October 1, 2015 and hasn't changed since.

Before ICD-10, this condition was coded in ICD-9-CM as 270.2 (other disturbances of aromatic amino-acid metabolism).