Hemophagocytic Lymphohistiocytosis (HLH)

October 14, 2020 Dian Sukma Hanggara 0 Comments

Kriteria diagnostik Hemophagocytic Lymphohistiocytosis (HLH), 5 di antara 8 (Histiocyte Society, 2004):

  • demam
  • splenomegali
  • sitopenia (minimal 2 seri sel)
  • Hb <9 g/dL
  • trombosit <100.000/uL
  • netrofil <1.000/uL
  • hipertrigliseridemia (puasa >265 mg/dL) dan/atau hipofibrinogemia (<150 mg/dL)
  • hiperferitinemia (>500 ng/mL)
  • Hemofagositosis (sumsum tulang, limpa, limfonodi)
  • aktivitas sel NK turun atau tidak ada
  • peningkatan soluble IL-2 receptor (CD25) (> 2400 U/mL)

 

Kriteria morfologi sumsum tulang (Gars et al, 2018):

  • hemofagositosis granulosit (1 per 1.000 sel)
  • hemofagositosis normoblas (4 per 1.000 sel), dan
  • setidaknya 1 hemofagositosis multipel sel berinti

 

 

Sumber:

Contino et al. Hemophagocytic Lymphohistiocytosis in Pediatric Patients: A Review. J Blood Disord Symptoms Treat 2018. Volume 2 (1): 011.

Gars et al. Bone marrow histomorphological criteria can accurately diagnose hemophagocytic lymphohistiocytosis. Haematologica 2018. Volume 103(10):1635-1641.

 

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