An 11-month-old girl was referred to our
centre for matched sibling allogeneic SCT. She was diagnosed
as a case of familial hemophagocytic lymphohistiocytosis
(HLH) at the age of three months and treated as per HLH-2004
protocol. Her elder brother was complete 6/6 match with her
on HLA typing. She received reduced intensity conditioning
regimen and her doner stem cells (CD34 positive cells) were
infused. GVHD prophylaxis consisted of cyclosporine and
methotrexate. She had neutrophil and platelets engraftment
on Day +22 and +26, respectively. FISH studies performed on
day +30 showed 97% XY (donor) cells and 3% XX (recipient)
cells.
On day +45, she developed high grade
fever and neck swelling with difficulty in swallowing.
Bilateral tonsils were enlarged. Hepatosplenomegaly was also
noted. A possibility of PTLD due to EBV was considered. Her
EBV DNA copy numbers were raised to 72700. She was started
on injection rituximab 375 mg/m
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