Contrast-enhanced ultrasound for multiple liver lesions after bone marrow transplant in a child with leukaemia: Multifocal focal nodular hyperplasia.

We present a case of a two-year-old girl in which liver lesions were characterised on contrast-enhanced ultrasound as multifocal focal nodular hyperplasia. This child had previously undergone haematopoietic stem cell transplantation for juvenile myelomonocytic leukaemia and was suspected to have hepatobiliary graft versus host disease. Liver biopsy was performed to confirm the unexpected focal nodular hyperplasia and look for concurrent graft versus host disease. Focal nodular hyperplasia was histologically confirmed on a background of diffuse liver damage in keeping with polypharmacotherapy, steatosis and sepsis. An element of graft versus host disease was not excluded but was not confidently shown in the sample of the lesion. This case report describes and illustrates how contrast-enhanced ultrasound may be of use to further assess hepatic lesions in a complex case of multifactorial hepatic pathology. Radiologists, haematologists and pathologists should be aware that multifocal focal nodular hyperplasia is part of the differential diagnosis of liver lesions in a child with liver damage due to complex disease and treatment. Biopsy remains the gold standard, if there is a concurrent clinical suspicion of graft versus host disease.

Dendritic cell reconstitution is associated with relapse-free survival and acute GVHD severity in children after allogeneic stem cell transplantation.

DCs are potent APCs and key regulators of innate and adaptive immunity. After allo-SCT, their reconstitution in the peripheral blood (PB) to levels similar to those in healthy individuals tends to be slow. We investigate the age- and sex-dependant immune reconstitution of myeloid (mDC) and plasmacytoid DC (pDC) in the PB of 45 children with leukaemia or myelodysplastic syndrome (aged 1-17 years, median 10) after allo-SCT with regard to relapse, acute GVHD (aGVHD) and relapse-free survival. Low pDC/µL PB up to day 60 post SCT are associated with higher incidence of moderate or severe aGVHD (P=0.035), whereas high pDC/µL PB up to day 60 are associated with higher risk of relapse (P<0.001). The time-trend of DCs/µL PB for days 0-200 is a significant predictor of relapse-free survival for both mDCs (P<0.001) and pDCs (P=0.020). Jointly modelling DC reconstitution and complications improves on these simple criteria. Compared with BM, PBSC transplants tend to show slower mDC/pDC reconstitution (P=0.001, 0.031, respectively), but have no direct effect on relapse-free survival. These results suggest an important role for both mDCs and pDCs in the reconstituting immune system. The inclusion of mDCs and pDCs may improve existing models for complication prediction following allo-SCT.

Myeloid and plasmacytoid dendritic cells: reference ranges in the peripheral blood of healthy children.

To date, few publications report on dendritic cells values in healthy children and mostly are found as control groups in studies focused on either allergic and autoimmune diseases or malignancies. This report provides an overview of 8 publications regarding absolute dendritic cells quantification in the peripheral blood of healthy children by using minimum manipulated samples processed within 24 hours.

Age-matched dendritic cell subpopulations reference values in childhood.

Dendritic cells (DCs) are the most potent antigen-presenting cells and are the key link between the innate and adaptive immune response. Only a few reports with study populations of up to 50 individuals have been published with age-based reference values for DC subpopulations in healthy children. Therefore, we aimed to establish reference ranges in a larger study population of 100 healthy children, which allowed age-matched subgroups. Most previous studies were performed using a dual-platform approach. In this study, a single-platform approach in a lyse no-wash procedure was used. DC subpopulations were defined as follows: CD45(+) CD85k(+) HLA-DR(+) CD14(-) CD16(-) CD33(+) cells as myeloid DCs (mDCs) and CD45(+) CD85k(+) HLA-DR(+) CD14(-) CD16(-) CD123(+) cells as plasmacytoid DCs (pDCs). Reference ranges were established using a semi-parametric regression of age-matched absolute and relative DC counts. We found a significant decline with increasing age in the medians of mDCs (P = 0.0003) and pDCs per µl peripheral blood (PB) (P = 0.004) and in the 50%, 90% and 95% reference ranges. We also identified significantly lower absolute cell counts of mDCs per µl PB in girls than in boys for all age groups (P = 0.0015). Due to the larger paediatric study population and single-platform approach, this study may give a more precise overview of the normal age-matched development of DC subpopulations and may provide a basis for analyzing abnormal DC counts in different illnesses or therapies such as post stem cell transplantation.