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Table 2 Laboratory test results

From: Rare and heterogeneous manifestations of leucocyte adhesion deficiency type 1: report of two cases with diagnostic dilemmas and novel ITGB2 mutation

Age: two years and eight months

Hematological Parameter

Reference range

Hemoglobin level

5.6 g/dl

11.1–16.3 g/dl

Total Leucocyte Count

142 x 103 cells/µl

4–10 x 103 cells/µl

Neutrophils

87% (124 x 103 cells / µl)

40–80% (2–7 x 103 cells/µl)

Lymphocytes

10% (14.2x103 cells / µl)

20–40% (1–3 x 103 cells/µl)

Monocytes

0

2–10% (0.2-1x 103 cells/µl)

Eosinophils

1% (1.4x103 cells / µl)

1–6% (0.02–0.5 x 103 cells/µl)

Platelets

94 x 103 cells / µl

150-410x 103 cells/µl

Peripheral film:

hypochromic, anisocytosis, leukocytosis with neutrophilia

Normochromic and normocytic

Blood group:

O positive

 

Immunological Work-up

Reference range

Serum Immunoglobulins (Ig):

  

IgG

9.5

2.3 to 14.1 g/l

IgA

0.9

0-0.83 g/l

IgM

1.5

0-1.45 g/l

IgE

476

< 100 IU/ml

Complements (C)

  

C3

0.8

0.8–1.73 g/l

C4

0.19

0.13–0.47 g/l

Lymphocyte subset analysis:

% Ratio

Cells/µl

% Ratio

Cells/µl

Total CD3+ T cells

57

8580

57 to 81

1000 to 4900

CD3+CD4+ (helper) T cells

36.7

5580

24 to 47

500 to 2700

CD3+CD8+ (cytotoxic) T cells

17.5

2670

17 to 37

300 to 2100

CD16+56+ (NK1) cells

10.1

1540

8 to 28

200 to 900

CD 19+ (B) cells

31.4

4780

10 to 27

200 to 2200

Flow cytometric analysis for CD11/CD18

expression of CD11c, and CD18 were less than 1% on the patient’s neutrophils, while expression of CD11b was 74%

Other tests:

  

C-reactive Protein-H (CRP-H)

35 mg/dl

< 05 mg/dl

Anti-HIV2-IgG

Negative

Negative

Anti-HCV3-IgG

Negative

Negative

Mutation analysis for beta 2 integrin gene

A homozygous splice-site variant (c.994–1G > C) was detected in the patient.

  1. Abbreviations: HCV = hepatitis C Virus, HIV = human immunodeficiency virus, NK = Natural killer, NBT = nitroblue tetrazolium
b. Case 2