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Current (2008) diagnostic criteria for HLH from HLH-2004 protocol (Ref: MB Jordan, Blood, 2011)

1. Molecular diagnosis compatible with HLA. These include proven pathological mutation of PRF1, UNC13D or STX11

or

2. At least 5 of 8 criteria below:

  • (1) Fever. Temp. spiking over 38.5 ° C for at least 7 days
  • (2) Splenomegaly, Palpabel spleen larger than 3 cm below left costal arc
  • (3) Cytopenia in two or more cell lines: Hemoglobin less than 9.0 g / dL, or Platelets more than 100,000 / μL, or absolute neutrophil less than 1000 / μL
  • (4) Hypertriglyceridemia or hypofibrinogenemia: Fasting triglycerides above 2.0 mmol / L, or more than 3 standard deviations (SD) above the normal value for age, or Fibrinogen less than 1.5 g / L, or more than 3 SD below nominal value for age
  • (5) Hemophagocytosis: Proven in bone marrow, spleen, or lymphknute; No evidence of malignancy
  • (6) Low or absent natural killer cell activity
  • (7) Serum ferritin levels more than 500 μg / L (but more than 3000 μg / L is a more realistic cut-off to rule out infections and autoimmune diseases
  • (8) Soluble CD25 (IL-2 receptor) more than 2400 U / mL (2 SD over reference value: non-age-related reference values) (Soluble IL-2 receptor measurement can be obtained from the Immunological Laboratory of Oslo University Hospital)

Original Table here (Jordan MB, 2011)

Macrophage activation syndrome, BINDEVEVSSYKDOMMER.no

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