Hemophagocytic syndrome, infection-associated
ICD-10 D76.2 is a billable code used to indicate a diagnosis of hemophagocytic syndrome, infection-associated.
Hemophagocytic syndrome (HPS) is a severe systemic inflammatory response characterized by excessive activation of the immune system, leading to hemophagocytosis, where macrophages engulf blood cells. Infection-associated HPS is often triggered by viral, bacterial, or parasitic infections, particularly in immunocompromised patients. Clinically, patients may present with fever, splenomegaly, cytopenias (including neutropenia), and elevated liver enzymes. The syndrome is associated with various white blood cell disorders, including lymphoproliferative diseases and autoimmune conditions. Neutropenia, a common finding, can exacerbate the risk of infections, complicating the clinical picture. The spleen plays a crucial role in filtering blood and managing immune responses, and its involvement in HPS can lead to significant hematological abnormalities. Diagnosis typically requires a combination of clinical evaluation, laboratory tests, and sometimes bone marrow biopsy to confirm hemophagocytosis. Early recognition and treatment are vital to prevent severe complications, including multi-organ failure.
Detailed blood work results, including complete blood count (CBC) and bone marrow findings.
Patients presenting with unexplained cytopenias and splenomegaly.
Need for thorough documentation of all hematological parameters and clinical findings.
Clear identification of the infectious agent and its relationship to HPS.
Patients with known infections presenting with systemic inflammatory responses.
Documentation must establish causality between the infection and the hemophagocytic syndrome.
Used to evaluate cytopenias in patients suspected of HPS.
Must include results showing neutropenia and other abnormalities.
Hematology specialists should ensure comprehensive lab results are documented.
Key indicators include fever, splenomegaly, cytopenias (especially neutropenia), elevated liver enzymes, and laboratory confirmation of hemophagocytosis.
