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Investigations

Investigations

Values Normal Limits
WBC 3.1 (diff 1.6 neutrophils and 2.8) (4-12×109/l)
HB 4.0999999999999996 (12.1-15.2g/l)
MCV 78.8 (80-96 fl)
Platelets 15 (140-45×109/l)
Reticulocyte count 5.6000000000000001E-2 (0.8-4% RBC)
Smear:
Normochormic, normocytic anaemia with spherocytes
Red cell fragments
Left shift in neutrophils
LDH 953 (70-250 u/l)
Haptoglobin (free serum) 0.45 (27-139 mg/dl)
Coombs direct Positive
INR 0.9 (1.0-1.3)
PTT 30 (23-31)
WR Negative
TPHA Negative
Toxoplasmosis Negative
Rubella Negative
Parvovirus B19 Negative
Malaria Negative
Herpes Negative
CMV Negative
Total bilirubin: 22 (3-17 umol/l)
Uncongugated bilirubin: 15 (0.0-0.3 mg/dl)
ANA Positive
Anti Ro: 104
Anti La: 156
ACLA: IgG 18
ACLA IgM 12
B2 glycoprotein Normal

Day 16 post admission infant developed a seizure

 

Mothers Results       
WR: negative
ANA: Positive, titre 1280 speckled pattern
dsDNA: negative
Anti Ro:  96
Anti La:  144

Abdominal ultrasound
Features of hepatomegaly
Splenomegaly with multiple microabscesses
Very thickened bladder with dilated post urethra

Bone marrow trephine
Reactive changes in RBC and megakaryopoeitic precursor
Focus granulation tissue secondary to previous haemorrhage
No granulomatous infiltration
No neoplastic infiltrate

29 days later ct scan shows decreased bleeding and oedemaCT  Brain
Day 16 post admission, infant developed a seizure.
CT scan shows multiple parenchymal bleeds and oedema

29 Days later CT scan shows decreased bleeding and oedema.