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FY2027

ICD-10 Code for Histiocytosis

The ICD-10-CM code for histiocytosis is D76.3Other histiocytosis syndromes. This is the code the Alphabetic Index gives for histiocytosis with no further qualifier, valid for FY2027 (October 1, 2026September 30, 2027).

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Which histiocytosis code should you use?

The Alphabetic Index lists 8 codes under Histiocytosis. Which one applies depends on the qualifier documented in the record — the type, site, cause, or associated condition. Find the qualifier below, then verify the code in the Tabular List before using it.

By type

acute differentiated progressiveC96.0Multifocal and multisystemic (disseminated) Langerhans-cell histiocytosis
Langerhans' cell NECC96.6Unifocal Langerhans-cell histiocytosis
lipid, lipoidD76.3Other histiocytosis syndromes
malignantC96.AHistiocytic sarcoma
mononuclear phagocytes NECD76.1Hemophagocytic lymphohistiocytosis
non-Langerhans cellD76.3Other histiocytosis syndromes
polyostotic sclerosingD76.3Other histiocytosis syndromes
sinus, with massive lymphadenopathyD76.3Other histiocytosis syndromes
syndrome NECD76.3Other histiocytosis syndromes
X NECC96.6Unifocal Langerhans-cell histiocytosis

Histiocytosis Langerhans' cell NEC

pulmonary, adultJ84.82Adult pulmonary Langerhans cell histiocytosis
unifocalC96.6Unifocal Langerhans-cell histiocytosis
multifocal X › multisystemicC96.0Multifocal and multisystemic (disseminated) Langerhans-cell histiocytosis
multifocal X › unisystemicC96.5Multifocal and unisystemic Langerhans-cell histiocytosis

Histiocytosis lipid, lipoid

essentialE75.29Other sphingolipidosis

Histiocytosis mononuclear phagocytes NEC

Langerhans' cellsC96.6Unifocal Langerhans-cell histiocytosis

Histiocytosis X NEC

acuteC96.0Multifocal and multisystemic (disseminated) Langerhans-cell histiocytosis
chronicC96.6Unifocal Langerhans-cell histiocytosis
multifocalC96.5Multifocal and unisystemic Langerhans-cell histiocytosis
multisystemicC96.0Multifocal and multisystemic (disseminated) Langerhans-cell histiocytosis
unifocalC96.6Unifocal Langerhans-cell histiocytosis

Index terms and codes are reproduced from the official ICD-10-CM FY2027 release. The Alphabetic Index is a finding aid — a code must always be verified in the Tabular List before it is reported. Data sources