We read the recent paper on profile of
EBV-associated infectious mononucleosis (IM) by
Balasubramanian, et al. [1] with interest. They
concluded that EBV associated IM is more common in preschool
children. Here we highlight another dreaded complication due
to reactivation of EBV post-allogeneic stem cell transplant
(SCT) leading to post-transplant lymphoproliferative
disorder (PTLD). Mortality rates due to PTLD are reported to
be as high as 50–90% [2]. Various therapeutic approaches are
suggested for EBV-associated PTLD including anti-B-cell
treatments such as rituximab
(Anti-CD20 monoclonal antibody) [3].
Rituximab alone or combined with low-dose chemotherapy is an
effective therapy for EBV-associated PTLD [4,5]. We describe
here successful treatment of EBV induced PTLD with rituximab
in an infant post-allogeneic SCT.
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/m2
IV weekly × 4 doses. Her fever disappeared 48 hours later
and tonsils gradually became normal size by day +52. Her
repeat EBV DNA copy numbers were 1900 after 3 weeks. She was
discharged on day +81 post-transplant and is doing well till
date (18 months post-transplant). Early detection of EBV
induced PTLD and aggressive treatment with Rituximab is a
key to survival in patients who have undergone allogeneic
SCT.
References
1. Balasubramanian S, Ganesh R, Kumar JR.
Profile of EBV- associated infectious mononucleosis. Indian
Pediatr. 2012;49:837-8.
2. Ocheni S, Kroeger N, Zabelina T,
Sobottka I, Ayuk F, Wolschke C, et al. EBV
reactivation and post transplant lymphoproliferative
disorders following allogeneic SCT. Bone Marrow Transplant.
2008;42:181-6.
3. Yadav SP, Chinnabhandar V. Rituximab
usage in children: a double edged sword. Indian Pediatr.
2012;49:335-6.
4. Serinet MO, Jacquemin E, Habes D,
Debray D, Fabre M, Bernard O. Anti-CD20 monoclonal antibody
(Rituximab) treatment for Epstein-Barr virus-associated,
B-cell lymphoproliferative disease in pediatric liver
transplant recipients. J Pediatr Gastroenterol Nutr.
2002;34:389-93.
5. Gross TG, Orjuela MA, Perkins SL, Park JR, Lynch JC,
Cairo MS, et al. Low Dose Chemotherapy and Rituximab
for Post transplant Lymphoproliferative Disease (PTLD): A
Children’s Oncology Group Report. Am J Transplant. 2012;
12:3069-75.
