A 12-year-old female patient with a history of ESRD secondary to focal segmental glomerulonephritis on peritoneal dialysis since she was 5 years old.
He received a deceased donor renal transplant at 11 years of age, with no incidents.
Donor serology: IgG EBV (+) and IgG cytomegalovirus (CMV) (+); IgG and IgM EBV receptor serology (-), IgG CMV (+).
She received induction therapy with basilib (2 doses of 10 mg) and methylprednisolone (300 mg/m2) and adequate tacrolimus/ oxime treatment with tacrolimus (0.3 mg/kg/0.6 mg/d), mycophenolate.
She received prophylaxis of cystosis jiroveci with metronidazole and antifungal medication with nistatin.
Six months after transplantation the patient developed odynophagia and intermittent fever, associated with oral ulcers and submaxillary lymphadenopathy.
Laboratory tests showed no evidence of neutropenia or common respiratory virus infection.
EBV serology (VCA IgM), CMV antigenemia, and current pharyngeal cultures are negative.
Antibiotic and antifungal treatment was administered empirically and MMF was suspended without clinical improvement.
The patient developed mouth breathing and snoring at night, secondary to increased volume of the right amygdala, and diffuse abdominal pain.
The study highlights: increased LDH (505 U/L), renal function, liver tests, amylase, lipase and Widal test normal.
Abdominal ultrasound shows numerous hypoechogenic focal liver and spleen lesions. Cervical computed axial tomography (CAT) confirms the increase in right intestinal volume and multiple adenopathies.
Bartonetta henselae infection was ruled out by serology and Aspergittus by detection of antigen mannan.
Serology was repeated for EBV negative, but quantitative viral load by real-time polymerase chain reaction (PCR) resulted in 9000 copies/mL of plasma.
Tacrolimus dose was decreased in half and amygdala biopsy and cervical lymphadenopathy were performed.
The analysis of both samples was negative for bacteria, mycosis fungoides and herpes simplex virus; PCR for EBV in lymphoid tissue was positive.
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Histological analysis showed extensive lymphoid proliferation associated with CD20 infection + and positive in situ hybridization for EBV RNA (EBER-1), findings with polymorphous PTLD, EBV-type immunophenotype.
It was decided to observe response to decreased immunosuppression with clinical and imaging follow-up.
The patient presented oral ulcers and dysphagia and progressive regression of hepatosplenic lesions on serial ultrasound.
After 10 days of therapy modification, the viral load is 260 copies/mL of plasma.
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Due to the antitumor effect described in the literature, it was decided to change the treatment to sirolimus, MMF and prednisone and to suspend tacrolimus.
At ten months of follow-up, the patient is in excellent clinical condition, with no evidence of rejection and disappearance of intra-abdominal lesions.
