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Table 2 Patient’s autoimmune panel results

From: A life-threatening case of pregnancy-related atypical Haemolytic uremic syndrome and successful treatment with Eculizumab

Investigation

Result

Autoimmune Screen

Complement 3

0.76 g/L (0.74–1.57) repeated 12 h later 0.62 g/L

Complement 4

0.08 g/L (0.13–0.41) initial, repeated after 2 days normal 0.17 g/L

Complement 9

500 mg/L (125–165)

Complement 5–8

Normal range

Complement Factor H antibody

< 8 (< 100 AU)

CD46

1.27 (1.75–4.65 MFI) Crossed checked twice with a health control

Complement Factor H

581 mg/L (345–590 mg/L)

Complement Factor B

450 mg/L (normal)

Anti-DsDNA Antibodies

Negative

Anti-Nuclear Antibodies

RNP Antibodies

Lupus Anticoagulant

B2-Glycoprotein

Anti-Smith Antibodies

SSA and SSB Antibodies

Ro 52 Antibodies

Scleroderma Ab

Jo-1 Antibodies

Haemolytic Screen

Reticulocytes

150 × 10^9/L (50–100 × 10^9/L)

Haptoglobin

< 0.15 (0.3–2.5 g/L)

ADAMTS13 Activity

70% (40–130%; normal)

Blood Film

Red cell fragments (1 per high power field). Mild polychromasia. Toxic granulation of neutrophils. Moderate thrombocytopenia.

Direct Antiglobulin Test

Negative

Methylenetetrahydrofolate reductase protein

Negative gene analysis

Factor V Leiden mutation

Not Detected

The Prothrombin gene mutation

Not Detected

Coagulopathy

INR

1.8 (< 1) Corrected with mixing study

PT

19 s (11–18 s) Corrected with mixing study

APTT

56 s (24–38 s) Corrected with mixing study

Protein C Activity

52% (70–180%)

Protein S Activity

39% (55–190%)

Infection Screen

Toxoplasmosis

IgM and IgG Negative

CMV and Parvovirus

IgG positive. IgM negative.

Hepatitis B and C

Negative HCV core antibody and RNA/ Immune HBV surface antibody positive antigens negative