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Table 1 Laboratory values meeting HLH diagnostic criteria found prior to, during and after trial of anakinra

From: Presentation of hemophagocytic lymphohistiocytosis due to a novel MUNC 13–4 mutation masked by partial therapeutic immunosuppression

HLH diagnostic criteria

Patient laboratory values

Timing relative to anakinra trial

Admission to prior to anakinra

During anakinra (day from start of anakinra)

One week after anakinra

Cytopenia in ≥ 2 cell lines

 
 

Haemoglobin < 9g/dL (90g/L)

WNL

8.8 (d2); Nadir 5.2 (d16)

WNL

 

Platelet < 100 × 109/L

60

26 (d 16)

13-21

 

Absolute neutrophil count < 1000 / μL

924

700 (d17)

48-153

Hypertriglyceridemia and/or

Hypofibrinogenemia

 
 

Fasting triglyceride ≥ 265 mg/dL)

-

802 (d16)

423, 577, 726

 

Fibrinogen < 150 mg/dL

127

WNL

-

Ferritin ≥ 500 ng/mL (500 µg/L)

905

629 (d15)

4814, 7597

sCD25 ≥ 2400 μg/mL

-

42,140 (d17)

-

Decreased or absent NK cell activity

  
 

LU20 NK a

108

37.4 (d4)

-

 

CD107a b

-

7 % (d17)

-

Hemophagocytosis in bone marrow

“some”

 

“numerous”

  1. -: laboratory value not measured; d: day from start of anakinra; NK: natural killer; WNL: within normal limits.
  2. a LU20 NK: Patient NK cells were cultured with radioisotope-labeled tumor cells. Functional NK cell activity measured by quantifying released isotope after correction for patient peripheral blood mononuclear cell % NK.
  3. b CD107a: Mononuclear cells from peripheral blood were stimulated with K562 target cells to induce degranulation. Six hours post incubation, CD107a expression was detected by flow cytometry.