Case 1
An 8-year-old female patient with severe aplastic anaemia who failed
immunosuppressive therapy with horse antithymocyte globulin and
cyclosporin underwent 10/10 human leukocyte antigen-matched bone marrow
transplantation with half-sibling elder brother. Neutrophil and platelet
engrafted on D+23 and D+27 respectively. Regeneration marrow and full
donor chimerism were demonstrated on D+30. She was noted to have
progressive deranged liver function and cholestasis since
post-transplant 5 weeks with peak alanine aminotransferase (ALT) 878
IU/L (<35), aspartate aminotransferase (AST) 966 IU/L (10-40),
gamma glutamyltransferase (GGT) 2,065 IU/L (13-28), total bilirubin 445
μmol/L (10-24) and direct bilirubin 430 μmol/L (5-10) respectively. She
was noted to have elevated cholesterol levels with total cholesterol
(TC) 23.1 mmol/L (<5.2), high density lipoprotein-cholesterol
(HDL-C) 0.5 mmol/L (>1.6) and high triglycerides (TG) 11.8
mmol/L (<1.7). Low density lipoprotein-cholesterol (LDL-C)
could not be calculated based on indirect quantitation with Friedewald
equation as TG was >4.5mmol/L as per usual practice and
hence it was measured directly and found to be normal at 0.2 mmol/L
(<4.1).
Apolipoprotein (Apo) A1 was low at 0.38 g/L (1.2-2.0) and ApoB was
elevated 1.88 g/L (0.41-1.07). Lipoprotein electrophoresis showed
chylomicron and very low-density lipoprotein (VLDL) bands, as well as an
addition beta-lipoprotein band which migrated to cathode was detected,
which was compatible with Lp-X (Figure 1A) . There was no family
history of hypercholesterolemia and clinical exam did not show any
xanthoma. Liver enzymes, bilirubin level and dyslipidemia gradually
normalized by post-transplant 2 years by expectant management(Figure 1B and Table 1) .