In our institution, hepatic veno‐occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) has been treated with N‐acetylcysteine (NAC) since 2008—a loading dose of 150 mg/kg, followed by 12 doses ...of 70 mg/kg 6 hourly. Nine children were diagnosed with hepatic VOD/SOS. Hepatic VOD/SOS occurred in seven children after stem cell transplantation and two were receiving chemotherapy for Wilms tumor. Their clinical severity was classified as moderate in two and severe in seven by the European Society for Blood and Marrow Transplantation criteria. All children recovered and were discharged from 4 to 16 days after diagnosis. No side effects were observed. Intravenous NAC is an effective treatment for hepatic VOD/SOS.
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BFBNIB, FZAB, GIS, IJS, KILJ, NLZOH, NUK, OILJ, SAZU, SBCE, SBMB, UL, UM, UPUK
Background
Pleraxifor for peripheral blood stem cell (PBSC) mobilization in children with malignancies is often given following failure of standard mobilization (SM) rather than as a primary ...mobilizing agent.
Study Design and Methods
In this retrospective multicenter study, we report the safety of plerixafor‐based PBSC mobilization in children with malignancies and compare outcomes between patients who received plerixafor upfront with SM (Group A) with those who received plerixafor following failure of SM (Group B). In the latter pleraxifor was given either following a low peripheral blood (PB) CD34 (<20 cells/cu.mm) (Group B1) or as a second collection process due to an unsuccessful yield (CD34 + < 2 × 106/kg) (Group B2) following failed SM and first apheresis attempts.
Results
The study cohort (n = 47) with a median age of 8 (range 0.6‐21) year, comprised 19 (40%) Group A and 28 (60%) Group B patients (B1 = 12 and B2 = 16). Pleraxifor mobilization was successful in 87.2% of patients, similar between Groups A and B (84.2% vs 89.2%) and resulted in a median 4‐fold increase in PB CD34. Median number of apheresis attempts was 2 in Groups A and B1 but 4 in Group B2. In Group B2, median total CD34+ yield post‐plerixafor was 9‐fold higher than after SM (P = .0013). Mild to moderate transient adverse events affected 8.5% of patients. Among patients who proceeded to autologous transplant (n = 39), all but one engrafted.
Conclusion
Plerixafor‐based PBSC collection was safe and effective in our cohort and supports consideration as a primary mobilizing agent in children with malignancies.
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BFBNIB, FZAB, GIS, IJS, KILJ, NLZOH, NUK, OILJ, SAZU, SBCE, SBMB, UL, UM, UPUK
Acute pancreatitis is a rare presentation in Burkitt lymphoma (BL) and may lead to delayed medical or unnecessary surgical treatment. Three cases of BL presenting as acute pancreatitis in the ...authors' institutions were described. Similar cases reported in the medical literature were collected and described along with the authors' cases. There were 12 cases described in the medical literature and hence a total of 15 cases of BL presenting as acute pancreatitis. Fourteen cases were the first diagnosis, and the other presented at lymphoma relapse. Twelve cases occurred in children under 15 years. Twelve patients had extrapancreatic disease. Three children were treated with surgery before diagnosis. Two patients died. Six of the remaining had adequate follow-up and were surviving in remission 8 months to 16 years after diagnosis. Lymphoma should be included in the differential diagnosis of acute pancreatitis in children. Acute pancreatitis in combination with malignant infiltration on imaging is highly suggestive of BL, especially in the jaundiced child.
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