D76.3Other histiocytosis syndromes

D76.3 is the ICD-10-CM diagnosis code for other histiocytosis syndromes. It belongs to category D76 (other specified diseases with participation of lymphoreticular and reticulohistiocytic tissue), block D70-D77 (other disorders of blood and blood-forming organs) and chapter 3 (diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism). It is billable, so it can be reported on its own for dates of service from October 1, 2026 through September 30, 2027.

Within D76, choose D76.3 (Other histiocytosis syndromes) only when documentation doesn't support a more specific option: Hemophagocytic lymphohistiocytosis (D76.1) and Hemophagocytic syndrome, infection-associated (D76.2).

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 814 (Reticuloendothelial and Immunity Disorders with MCC, relative weight 2.0729), DRG 815 (Reticuloendothelial and Immunity Disorders with CC, relative weight 1.0444) and DRG 816 (Reticuloendothelial and Immunity Disorders without CC/MCC, relative weight 0.7381), in MDC 16 (Diseases and Disorders of the Blood and Blood Forming Organs and Immunological 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 288.4 (Hemophagocytic syndromes).