D76.1

Applicable To

• Familial hemophagocytic reticulosis

• Histiocytoses of mononuclear phagocytes

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

Details

• D76.1 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.

• The 2023 edition of ICD-10-CM D76.1 became effective on October 1, 2022.

• This is the American ICD-10-CM version of D76.1 – other international versions of ICD-10 D76.1 may differ.