C96.0Multifocal and multisystemic (disseminated) Langerhans-cell histiocytosis

C96.0 is the ICD-10-CM diagnosis code for multifocal and multisystemic (disseminated) Langerhans-cell histiocytosis. It belongs to category C96 (other and unspecified malignant neoplasms of lymphoid, hematopoietic and related tissue), block C81-C96 (malignant neoplasms of lymphoid, hematopoietic and related tissue) and chapter 2 (neoplasms). It is billable, so it can be reported on its own for dates of service from October 1, 2026 through September 30, 2027.

Within C96 (other and unspecified malignant neoplasms of lymphoid, hematopoietic and related tissue), C96.0 is specifically for multifocal and multisystemic (disseminated) Langerhans-cell histiocytosis. Related codes cover malignant mast cell neoplasm (C96.2), sarcoma of dendritic cells (accessory cells) (C96.4), multifocal and unisystemic Langerhans-cell histiocytosis (C96.5), Unifocal Langerhans-cell histiocytosis (C96.6), malignant neoplasm of lymphoid, hematopoietic and related tissue, unspecified (C96.9), histiocytic sarcoma (C96.A) and other specified malignant neoplasms of lymphoid, hematopoietic and related tissue (C96.Z).

Don't report C96.0 together with the conditions in its Excludes1 note: adult pulmonary Langerhans cell histiocytosis (J84.82), multifocal and unisystemic Langerhans-cell histiocytosis (C96.5) and unifocal Langerhans-cell histiocytosis (C96.6).

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 011 (Tracheostomy for Face, Mouth and Neck Diagnoses or Laryngectomy with MCC, relative weight 5.6154), DRG 012 (Tracheostomy for Face, Mouth and Neck Diagnoses or Laryngectomy with CC, relative weight 4.381), DRG 013 (Tracheostomy for Face, Mouth and Neck Diagnoses or Laryngectomy without CC/MCC, relative weight 2.7307), DRG 826 (Myeloproliferative Disorders or Poorly Differentiated Neoplasms with Major O.R. Procedures with MCC, relative weight 4.7581), DRG 827 (Myeloproliferative Disorders or Poorly Differentiated Neoplasms with Major O.R. Procedures with CC, relative weight 2.3896), DRG 828 (Myeloproliferative Disorders or Poorly Differentiated Neoplasms with Major O.R. Procedures without CC/MCC, relative weight 1.6955) and 5 more, in Pre-MDC and MDC 17 (Myeloproliferative Diseases and Disorders, Poorly Differentiated Neoplasms), 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 202.50 (Letterer-siwe disease, unspecified site, extranodal and solid organ sites).