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Table.1 Diagnostic criteria at admission in patients between 0 and 18 years with a diagnosis of HLH in the FVL between 2012 and 2019 (n = 21)

From: Pediatric hemophagocytic lymphohistiocytosis: A rarely diagnosed entity in a developing country

Criteria at admission

Frequency

%(n)

Median (IQR)

Fever ≥ 7 days

71.4(15)

17 days(6–30)

Splenomegaly > 3 cm below rib limit

61.9(13)

-

Bicytopenia

61.9(13)

-

Hyperferritinemia > 500 mg/dl

81.0(17)

1866.5 ng/ml (889-10286)

Hypertriglyceridemia (> 150 mg/dl) or hypofibrinogenemia (< 150 mg/dl)

76.2(16)

267 mg/dl (184–324)

167.5 mg/dl (94–315)

Hemophagocytosis in BM

57.1(12)

-

Decrease in NK cells or altered functionality

14.3(3)

-

No evidence of malignancy

100.0(21)

-

Genetic Testing for primary HLHa

14.3(3)

-

  1. aGenetic testing is not widely available and is only performed in select cases
  2. **In our institution we have no availability of soluble CD 25