D76.2
Hemophagocytic syndrome, infection-associated
Clinical Classification Guidelines
Use Additional Code
- code to identify infectious agent or disease.
Medical Intelligence & Overview
Hemophagocytic syndrome, infection-associated (ICD-10 code D76.2), is a rare but serious condition where the immune system becomes overactive in response to an infection. This overactivation leads to damage of the body's own tissues and organs. Often caused by infections such as viruses, bacteria, or fungi, this syndrome disrupts normal immune regulation, resulting in widespread inflammation and cell destruction.
Causes & Symptoms
Clinical Causes: Viral infections, such as Epstein-Barr virus (EBV) and cytomegalovirus (CMV) Bacterial infections, including tuberculosis and leprosy Fungal infections like histoplasmosis and candidiasis Other infectious agents that stimulate an abnormal immune response
Key Symptoms: High fever that persists or fluctuates Enlargement of spleen and liver (splenomegaly and hepatomegaly) Cytopenias, leading to anemia, leukopenia, and thrombocytopenia Fatigue and weakness Swollen lymph nodes Rashes or skin lesions Bleeding or bruising due to low platelets Liver dysfunction, resulting in jaundice Neurological symptoms like confusion or seizures in severe cases
Diagnostic & Treatment
Diagnosis Path: Persistent fever and splenomegaly Cytopenias affecting at least two blood cell lines Elevated levels of ferritin, triglycerides, and liver enzymes Low or absent natural killer (NK) cell activity Presence of hemophagocytosis in bone marrow, lymph nodes, or spleen Identification of the underlying infectious agent through laboratory testing
Treatment Protocols: Treating the underlying infection with appropriate antimicrobial therapies Immunosuppressive medications such as corticosteroids to reduce inflammation Chemotherapy agents like etoposide, if the syndrome is severe or persistent Supportive care including blood transfusions and management of organ failure Use of therapies to modulate immune responses, such as intravenous immunoglobulin (IVIG) In some cases, hematopoietic stem cell transplantation may be considered
Clinical Advice & FAQs
Billing Guidance
Is D76.2 a billable ICD-10 code?
Yes, D76.2 is a specific, billable code that can be used to indicate a diagnosis for reimbursement purposes.
Documentation
How do I report D76.2?
Clinical documentation must specify the nature of Hemophagocytic syndrome, infection-associated and any associated comorbidities for accurate reporting.
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