Hemophagocytic lymphohistiocytosis
ICD-10 D76.1 is a billable code used to indicate a diagnosis of hemophagocytic lymphohistiocytosis.
Hemophagocytic lymphohistiocytosis (HLH) is a severe systemic inflammatory syndrome characterized by excessive activation of the immune system, leading to hemophagocytosis, where macrophages engulf blood cells. This condition can be primary (genetic) or secondary to infections, malignancies, autoimmune diseases, or immunodeficiencies. Clinically, HLH presents with symptoms such as prolonged fever, splenomegaly, cytopenias (including neutropenia), and elevated liver enzymes. The pathophysiology involves a hyper-inflammatory response, often triggered by infections like Epstein-Barr virus (EBV) or autoimmune disorders. Diagnosis is based on clinical criteria, including fever, splenomegaly, cytopenias, and specific laboratory findings such as elevated ferritin and soluble CD25. Prompt recognition and treatment are crucial, as HLH can lead to multi-organ failure and is associated with high mortality if untreated.
Detailed lab results, clinical findings, and treatment plans must be documented.
Patients presenting with unexplained cytopenias, splenomegaly, or severe infections.
Hematologists must ensure that all criteria for HLH are met and documented to support coding.
Comprehensive history of immune function, previous infections, and autoimmune conditions.
Patients with recurrent infections or autoimmune disorders presenting with HLH symptoms.
Immunologists should document any immunodeficiencies that may contribute to HLH.
Used to evaluate cytopenias in patients suspected of HLH.
Document the indication for the CBC and any relevant clinical findings.
Hematologists should ensure that the CBC results are clearly linked to the HLH diagnosis.
The key clinical criteria for diagnosing HLH include prolonged fever, splenomegaly, cytopenias (including neutropenia), elevated ferritin levels, hypertriglyceridemia, and elevated soluble CD25. A thorough clinical evaluation and laboratory testing are essential for accurate diagnosis.
