Info
🌱 來自: Huppert’s Notes
Other Malignant Hematologic Conditions🚧 施工中
Other Malignant Hematologic Conditions
Hemophagocytic lymphohistiocytosis (HLH)
• Pathophysiology: Excess immune activation, inflammation, and tissue destruction that can be life threatening. Can be idiopathic or provoked by a viral trigger or malignancy.
• Clinical features: Fever, splenomegaly, cytopenias, mental status changes
• Diagnosis: 5 of the 8 criteria: Fever ≥38.5°C, splenomegaly, bicytopenia, ↑triglycerides or ↓fibrinogen, hemophagocytosis seen on pathology review, ↓natural killer cell activity, ferritin >500 ng/mL, ↑soluble IL-2 receptor.
• Treatment: Treat underlying cause. Give steroids. If acutely ill or decompensating, consider HLH-specific therapy based on the HLH-94 regimen (etoposide, dexamethasone, +/- intrathecal therapy)