2024 ICD-10-CM Diagnosis Code D76.1

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 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.

D76.1 is grouped with Diagnostic Related Groups - MS-DRG Mapping

Diagnostic Related Groups (DRGs) are a classification system used to group together diagnosis codes for the purpose of reimbursement and healthcare management. DRGs are used to standardize the way hospitals and other providers are paid for inpatient services. In this system patients are grouped together based on their principal diagnosis in areas referred to as Major Diagnostic Categories (MDC). Once a patient is assigned a particular diagnostic category, providers receive a predetermined payment rate for the services rendered. This reimbursement rate is often fixed and is meant to cover the cost of care for that patient. Reimbursement is also affected by the relative weight of a diagnostic related group based on the severity of a patient's illness and the associated cost of care during hospitalization.

Diagnostic related groups encourage healthcare providers to focus on quality and efficiency in patient care while managing costs effectively. The diagnosis code is present in the following groups for version MS-DRG V41.0 applicable from 10/01/2023 through 09/30/2024.

The relative weight of a diagnostic related group determines the reimbursement rate based on the severity of a patient's illness and the associated cost of care during hospitalization.

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.

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.