Table 1 Summary of laboratory findings on NM at presentation and after each course of Intravenous Immunoglobulin demonstrating transient improvement

 Haemoglobin

12.4–16.7 g/dL

3.5

14.7

13.1

12.7

 Haematocrit

0.35–0.49 UL

0.12

0.48

0.38

0.40

 Red cell Count

3.8–5.5 × 10⁹/L

1.29

5.56

4.88

4.43

 Mean Cell Volume

79–100 fL

89.90

85.60

85.60

89.40

 Mean Cell Haemoglobin

27–35 pg

27.10

26.50

26.80

28.70

 Mean Cell Haemoglobin concentration

29 - 37 g/dL

30.20

31.30

31.30

32.10

 White Cell Count

4-12 × 10⁹/l

4.52

5.14

4.70

2.62

 Platelet Count

150-450 × 10⁹/L

310

245

245

187

 Red cell distribution width

11.0–16.0

22.80

14.10

14.10

13.20

 Reticulocyte production index

1.00–1.25

2.2

0.9

 Ferritin

20-300 ng/mL

7

11

 INR

1.00–1.25

1.06

1.02

 Prothrombin Time

< 14 s

13.2

11.8

14.6

 Activated Partial Thromboplastin Time

27–43 s

52.7

35.4

 Fibrinogen

1.7–4.2 g/L

3.5

3.8

 Factor VIII

50–150%

6

48 (7^a)

78 (15^a)

88 (22^a)

 Factor VII inhibitor

Bethesda

0

0

 Von Willebrand Factor Activity

47.8–173.2%

15

32 (6^a)

 Von Willebrand Factor Antigen

50–160%

10

52 (10^a)

51 (14^a)

43 (10^a)

 Agonist - ristocetin 0.5μg/mL

No response

Normal

Normal

Normal

 Agonist - ristocetin 1.5μg/mL

60–110%

5

10

10

32 (10^a)

 ADP 5.0 uM

60–100%

17

 Epinephrine 10μg/mL

60–110%

14

 Collagen 2μg/mL

60–110%

3

 Arachidonic Acid

Decreased

 Collagen/Epinephrine

82–150

> 300

 Collagen /Adenosine diphosphate

62–100

> 300

 Albumin

35–50 g/L

28

 Bilirubin (unconjugated)

3-6umol/L

4

 Bilirubin (conjugated)

2–7 umol/L

3

 AST

13–35 IU/L

8

 ALT

< 35 IU/L

3

IgM paraprotein

g/L

3

4

4

 Sodium

136-145 mmol/L

136

 Potassium

3.5–5.1 mmol/L

4.5

 Chloride

98-107 mmol/L

108

 Total CO₂

21-29 mmol/L

23

 Urea

2.9–8.2 mmolL

2.5

 Creatinine

53–97 umol/L

36

Bone Marrow Aspirate