1. ICD-10-CM Codes
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2. D50-D89 Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism
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3. D70-D77 Other disorders of blood and blood-forming organs
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4. D76- Other specified diseases with participation of lymphoreticular and reticulohistiocytic tissue
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5. 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.

• Familial hemophagocytic lymphohistiocytosis

• 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

• 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

• 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
  • mononuclear phagocytes NEC D76.1
• Lymphohistiocytosis, hemophagocytic D76.1 (familial)
• Reticulosis (skin)
  • hemophagocytic, familial D76.1
• Syndrome - see also Disease
  • macrophage activation D76.1

Reimbursement claims with a date of service on or after October 1, 2015 require the use of ICD-10-CM codes.