2026 ICD-10-CM Diagnosis Code D76.1Hemophagocytic lymphohistiocytosis

ICD-10-CM Codes›D50–D89›D70-D77›D76

ICD-10-CM D76.1
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

D76.1 is a billable ICD-10-CM diagnosis code for hemophagocytic lymphohistiocytosis. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026) and groups to MS-DRG 814 through 816. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Diseases of white blood cells.

Code Identity

ICD-10-CM Code
D76.1
Billable Status
Yes — Valid for Submission
Code Describes
Hemophagocytic lymphohistiocytosis
Short Description
Hemophagocytic lymphohistiocytosis
Same as the full description in the CMS dataset.
Parent Code
D76
Other specified diseases with participation of lymphoreticular and reticulohistiocytic tissue

Code Classification

ChapterD50–D89Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism
SectionD70-D77Other disorders of blood and blood-forming organs
CategoryD76Other specified diseases with participation of lymphoreticular and reticulohistiocytic tissue
This CodeD76.1Hemophagocytic lymphohistiocytosis

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Acquired hemophagocytic lymphohistiocytosis associated with malignant disease
  • Acquired pancytopenia
  • Disorder of hematopoietic system in newborn
  • Familial hemophagocytic lymphohistiocytosis
  • Hemophagocytic lymphohistiocytosis
  • Lipochrome histiocytosis - familial
  • Macrophage activation syndrome
  • Macrophage activation syndrome due to juvenile systemic onset arthritis
  • Malignant white blood cell disorder
  • Neonatal anemia
  • Neonatal thrombocytopenia
  • NOCARH syndrome
  • Secondary hemophagocytic lymphohistiocytosis

Tabular List NotesGuidance

Coding notes and annotation back-references applicable to this code.

Inclusion Terms

  • Familial hemophagocytic reticulosis
  • Histiocytoses of mononuclear phagocytes

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

    • Histiocytosis
      • mononuclear phagocytes NEC
    • Lymphohistiocytosis, hemophagocytic(familial)
    • Reticulosis(skin)
      • hemophagocytic, familial
    • Syndrome
      • macrophage activation

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR BLD007
Diseases of white blood cells
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • Macrophage Activation Syndrome

    a serious complication of childhood systemic inflammatory disorders that is thought to be caused by excessive activation and proliferation of t-lymphocytes and macrophages. it is seen predominantly in children with systemic onset juvenile idiopathic arthritis.

  • Grade 1 Secondary Hemophagocytic Lymphohistiocytosis, CTCAE

    asymptomatic or mild symptoms; requiring clinical and/or diagnostic evaluation; intervention not indicated

  • Grade 2 Secondary Hemophagocytic Lymphohistiocytosis, CTCAE

    moderate symptoms; intervention indicated (e.g., immunosuppressive agents)

  • Grade 3 Secondary Hemophagocytic Lymphohistiocytosis, CTCAE

    severe or medically significant but not immediately life threatening; hospitalization or prolongation of existing hospitalization indicated

  • Grade 4 Secondary Hemophagocytic Lymphohistiocytosis, CTCAE

    life-threatening consequences; urgent intervention indicated

  • Grade 5 Secondary Hemophagocytic Lymphohistiocytosis, CTCAE

    death

  • Secondary Hemophagocytic Lymphohistiocytosis, CTCAE

    a disorder characterized by the development of a pathological and biochemical hyperinflammatory syndrome characterized by fever, cytopenias, hyperferritinemia, coagulopathy, hypofibrinogenemia and/or transaminitis and is attributable to therapy.

  • Neonatal Alloimmune Thrombocytopenia|NAIT|Neonatal Thrombocytopenia due to Platelet Alloimmunization

    thrombocytopenia that occurs in neonates as a consequence of transplacental passage of maternal alloantibodies directed against fetal platelet antigens.

  • Neonatal Thrombocytopenia

    a condition characterized by a decrease in the number of platelets in the blood below established reference ranges in a newborn.

  • Secondary Hemophagocytic Lymphohistiocytosis

    hemophagocytic lymphohistiocytosis due to infections, autoimmune disorders, or underlying malignancies. signs and symptoms include fever, lymphadenopathy, hepatomegaly, splenomegaly, and pancytopenia.

  • Refractory Secondary Hemophagocytic Lymphohistiocytosis

    secondary hemophagocytic lymphohistiocytosis that is resistant to treatment.

Patient EducationClinical

Familial hemophagocytic lymphohistiocytosis

Familial hemophagocytic lymphohistiocytosis is a disorder in which the immune system produces too many activated immune cells (lymphocytes) called T cells, natural killer cells, B cells, and macrophages (histiocytes). Excessive amounts of immune system proteins called cytokines are also produced.

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Convert D76.1 to ICD-9-CMHistory

The closest ICD-9-CM equivalents under the General Equivalence Mappings.

ICD-9-CM
288.4 Hemophagocytic syndromes
Approximate The match is approximate rather than exact.

Code HistoryHistory

FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About D76.1Overview

Is D76.1 a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report hemophagocytic lymphohistiocytosis on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What MS-DRG does D76.1 group to?

When hemophagocytic lymphohistiocytosis is the principal diagnosis on an inpatient stay, it groups to MS-DRG 814, 815, 816, with relative weights from 0.6320 to 2.1267 depending on complications. Higher weights mean higher Medicare reimbursement.

What is the ICD-9 equivalent of D76.1?

Under the General Equivalence Mappings, hemophagocytic lymphohistiocytosis converts to ICD-9-CM 288.4 (hemophagocytic syndromes). The mapping is approximate, so confirm the match fits the documentation.

Footnotes

[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.