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ICD-10-CM Codes
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D50-D89
Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism
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D70-D77
Other disorders of blood and blood-forming organs
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D76-
Other specified diseases with participation of lymphoreticular and reticulohistiocytic tissue
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2021 ICD-10-CM Diagnosis Code D76.1
2021 ICD-10-CM Diagnosis Code D76.1
Hemophagocytic lymphohistiocytosis
2016 2017 2018 2019 2020 2021 Billable/Specific Code
- D76.1 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
- The 2021 edition of ICD-10-CM D76.1 became effective on October 1, 2020.
- This is the American ICD-10-CM version of D76.1 - other international versions of ICD-10 D76.1 may differ.
Applicable To- Familial hemophagocytic reticulosis
- Histiocytoses of mononuclear phagocytes
The following code(s) above
D76.1 contain annotation back-references
Annotation Back-References
In this context, annotation back-references refer to codes that contain:
- Applicable To annotations, or
- Code Also annotations, or
- Code First annotations, or
- Excludes1 annotations, or
- Excludes2 annotations, or
- Includes annotations, or
- Note annotations, or
- Use Additional annotations
that may be applicable to
D76.1:
Approximate Synonyms
- Familial hemophagocytic lymphohistiocytosis
Clinical Information
- A group of related disorders characterized by lymphocytosis; histiocytosis; and hemophagocytosis. The two major forms are familial and reactive.
- A rare but potentially life-threatening disorder characterized by the proliferation of histiocytes and macrophages and phagocytosis of red blood cells, white blood cells, and platelets. It may be inherited or secondary, due to infections, autoimmune disorders, or underlying malignancies. Signs and symptoms include fever, lymphadenopathy, hepatomegaly, splenomegaly, and pancytopenia.
- A rare disorder in which histiocytes and lymphocytes (types of white blood cells) build up in organs including the skin, spleen, and liver, and destroy other blood cells. Hemophagocytic lymphohistiocytosis may be inherited or caused by certain conditions or diseases, including infections, immunodeficiency (inability of the body to fight infections), and cancer.
- A rare, life-threatening disorder usually appearing during the first few months of life. It is caused by abnormalities in the prf1, unc13d, and stx11 genes. It is characterized by histiocytic proliferation and phagocytosis. Patients present with fever, lymphadenopathy, and hepatosplenomegaly.
- Rare form of hemophagocytic lymphohistiocytosis with multiorgan involvement seen in infants and young children
ICD-10-CM D76.1 is grouped within Diagnostic Related Group(s) (MS-DRG v38.0):
- 814 Reticuloendothelial and immunity disorders with mcc
- 815 Reticuloendothelial and immunity disorders with cc
- 816 Reticuloendothelial and immunity disorders without cc/mcc
Convert D76.1 to ICD-9-CM
Code History
- 2016 (effective 10/1/2015): New code (first year of non-draft ICD-10-CM)
- 2017 (effective 10/1/2016): No change
- 2018 (effective 10/1/2017): No change
- 2019 (effective 10/1/2018): No change
- 2020 (effective 10/1/2019): No change
- 2021 (effective 10/1/2020): No change
- Histiocytosis D76.3
ICD-10-CM Diagnosis Code D76.3
Other histiocytosis syndromes
2016 2017 2018 2019 2020 2021 Billable/Specific Code
Applicable To- Reticulohistiocytoma (giant-cell)
- Sinus histiocytosis with massive lymphadenopathy
- Xanthogranuloma
- Lymphohistiocytosis, hemophagocytic D76.1 (familial)
- Reticulosis (skin)
- hemophagocytic, familial D76.1
- Syndrome - see also Disease
ICD-10-CM Codes Adjacent To D76.1
D75 Other and unspecified diseases of blood and blood-forming organs
D75.0 Familial erythrocytosis
D75.1 Secondary polycythemia
D75.8 Other specified diseases of blood and blood-forming organs
D75.82 Heparin induced thrombocytopenia (HIT)
D75.89 Other specified diseases of blood and blood-forming organs
D75.9 Disease of blood and blood-forming organs, unspecified
D75.A Glucose-6-phosphate dehydrogenase (G6PD) deficiency without anemia
D76 Other specified diseases with participation of lymphoreticular and reticulohistiocytic tissue
D76.1
Hemophagocytic lymphohistiocytosis
D76.2 Hemophagocytic syndrome, infection-associated
D76.3 Other histiocytosis syndromes
D77 Other disorders of blood and blood-forming organs in diseases classified elsewhere
D78 Intraoperative and postprocedural complications of the spleen
D78.0 Intraoperative hemorrhage and hematoma of the spleen complicating a procedure
D78.02 Intraoperative hemorrhage and hematoma of the spleen complicating other procedure
D78.1 Accidental puncture and laceration of the spleen during a procedure
D78.12 Accidental puncture and laceration of the spleen during other procedure
Reimbursement claims with a date of service on or after October 1, 2015 require the use of ICD-10-CM codes.