2024 ICD-10-CM Diagnosis Code D76.1
Hemophagocytic lymphohistiocytosis
- ICD-10-CM Code:
- D76.1
- ICD-10 Code for:
- Hemophagocytic lymphohistiocytosis
- Is Billable?
- Yes - Valid for Submission
- Chronic Condition Indicator: [1]
- Chronic
- Code Navigator:
D76.1 is a billable diagnosis code used to specify a medical diagnosis of hemophagocytic lymphohistiocytosis. The code is valid during the current fiscal year for the submission of HIPAA-covered transactions from October 01, 2023 through September 30, 2024.
Approximate Synonyms
The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:
- Acquired hemophagocytic lymphohistiocytosis associated with malignant disease
- Familial hemophagocytic lymphohistiocytosis
- Hemolytic erythrophagocytic syndrome
- Hemolytic erythrophagocytic syndrome
- Hemolytic erythrophagocytic syndrome
- Hemolytic erythrophagocytic syndrome
- Hemolytic erythrophagocytic syndrome
- Hemolytic erythrophagocytic syndrome
- Hemophagocytic lymphohistiocytosis
- Hemophagocytic lymphohistiocytosis
- Hemophagocytic lymphohistiocytosis
- Lipochrome histiocytosis - familial
- Macrophage activation syndrome
- Macrophage activation syndrome
- Macrophage activation syndrome due to juvenile systemic onset arthritis
- Malignant white blood cell disorder
- Secondary hemophagocytic lymphohistiocytosis
- Secondary hemophagocytic lymphohistiocytosis
Clinical Classification
Clinical Category is Diseases of white blood cells
- CCSR Category Code: BLD007
- Inpatient Default CCSR: Y - Yes, default inpatient assignment for principal diagnosis or first-listed diagnosis.
- Outpatient Default CCSR: Y - Yes, default outpatient assignment for principal diagnosis or first-listed diagnosis.
Clinical Information
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.Secondary Hemophagocytic Lymphohistiocytosis
hemophagocytic lymphohistiocytosis due to infections, autoimmune disorders, or underlying malignancies. signs and symptoms include fever, lymphadenopathy, hepatomegaly, splenomegaly, and pancytopenia.
Tabular List of Diseases and Injuries
The following annotation back-references are applicable to this diagnosis code. The Tabular List of Diseases and Injuries is a list of ICD-10-CM codes, organized "head to toe" into chapters and sections with coding notes and guidance for inclusions, exclusions, descriptions and more.
Inclusion Terms
Inclusion TermsThese terms are the conditions for which that code is to be used. The terms may be synonyms of the code title, or, in the case of "other specified" codes, the terms are a list of the various conditions assigned to that code. The inclusion terms are not necessarily exhaustive. Additional terms found only in the Alphabetic Index may also be assigned to a code.
- Familial hemophagocytic reticulosis
- Histiocytoses of mononuclear phagocytes
Index to Diseases and Injuries References
The following annotation back-references for this diagnosis code are found in the injuries and diseases index. The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10-CM code(s).
- - Histiocytosis - D76.3
- - mononuclear phagocytes NEC - D76.1
- - Reticulosis (skin)
- - hemophagocytic, familial - D76.1
- - Syndrome - See Also: Disease;
- - macrophage activation - D76.1
Convert D76.1 to ICD-9-CM
- ICD-9-CM Code: 288.4 - Hemophagocytic syndromes
Approximate Flag - The approximate mapping means there is not an exact match between the ICD-10 and ICD-9 codes and the mapped code is not a precise representation of the original code.
Patient Education
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. This overactivation of the immune system causes fever and damages the liver and spleen, resulting in enlargement of these organs.
Familial hemophagocytic lymphohistiocytosis also destroys blood-producing cells in the bone marrow, a process called hemophagocytosis. As a result, affected individuals have low numbers of red blood cells (anemia) and a reduction in the number of platelets, which are involved in clotting. A reduction in platelets may cause easy bruising and abnormal bleeding.
The brain may also be affected in familial hemophagocytic lymphohistiocytosis. As a result, affected individuals may experience irritability, delayed closure of the bones of the skull in infants, neck stiffness, abnormal muscle tone, impaired muscle coordination, paralysis, blindness, seizures, and coma. In addition to neurological problems, familial hemophagocytic lymphohistiocytosis can cause abnormalities of the heart, kidneys, and other organs and tissues. Affected individuals also have an increased risk of developing cancers of blood-forming cells (leukemia and lymphoma).
Signs and symptoms of familial hemophagocytic lymphohistiocytosis usually become apparent during infancy, although occasionally they appear later in life. They usually occur when the immune system launches an exaggerated response to an infection, but may also occur in the absence of infection. Without treatment, most people with familial hemophagocytic lymphohistiocytosis survive only a few months.
[Learn More in MedlinePlus]
Code History
- FY 2024 - No Change, effective from 10/1/2023 through 9/30/2024
- FY 2023 - No Change, effective from 10/1/2022 through 9/30/2023
- FY 2022 - No Change, effective from 10/1/2021 through 9/30/2022
- FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021
- FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
- FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
- FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
- FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
- FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016. This was the first year ICD-10-CM was implemented into the HIPAA code set.
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.