Case 3
A previously healthy 7-year-old female underwent OHT after developing
viral induced cardiomyopathy leading to heart failure. Pre-transplant
serologies were: EBV donor positive/recipient unknown. She was started
tacrolimus, Azathioprine and steroids to prevent transplant rejection.
The rest of the immediate post-transplant history is limited given
incomplete chart.
Approximately seven months post-transplant she developed enlarged lymph
nodes of the neck, axilla and groin and was diagnosed with polymorphous
PTLD via biopsy. Her immunosuppression was decreased briefly, but due to
graft rejection she required re-escalation of her immunosuppressive
medications. One year following initial PTLD diagnosis, she again
developed diffuse palpable adenopathy of the groin and axilla that was
found to be recurrence of PTLD confirmed on biopsy. She concomitantly
had chronic EBV DNAemia with peak documented levels of 7,365 copies/mL
(presumed to be higher but records are incomplete). She underwent total
lymphoid irradiation to the periaortic, splenic and pelvic lymph nodes
delivering a total of 4.8 Gy in six fractions before therapy was
truncated and not resumed due to ongoing medical issues surrounding
chronic EBV infection. Despite radiation therapy, she developed two new
liver lesions with the largest lesion measuring appx 2 cm on CT. No
evidence of additional disease outside the liver was seen. Liver biopsy
was consistent with EBV SMT (Fig 3). At the same time, she underwent
scheduled cardiac catheterization which revealed severe coronary artery
disease of her graft. She was admitted to the hospital in heart failure
and re-listed for transplant. During this hospitalization, she went into
cardiac arrest and required brief placement on ECMO before being
re-transplanted 2 days later. After a prolonged four-month
hospitalization, she was discharged home on cyclosporine, MMF, and
prednisone.
Once recovered, she underwent planned resection of the known solitary
hepatic SMT which was successful. Surveillance CT following resection
showed recurrence of the hepatic SMT, in addition to the development of
a second hepatic tumor that was deemed unresectable at the time. With
increasing growth of the tumors now measuring 8.5 cm and 4.5 cm along
with chronic GI complaints of diarrhea and abdominal pain,
chemoembolization was briefly considered but dismissed after CT
angiography demonstrated the tumors were poorly vascularized. She
underwent palliative radiation to the liver and received a total tumor
dose of 3500 cGy. Given the positive response to radiotherapy, she was
evaluated for second resection at an outlying facility and underwent
full resection of now three liver tumors with subsequent remission.
Due to progressive coronary artery disease and continued heart failure
symptoms, she was re-listed for a third cardiac transplant in addition
to a kidney transplant due to ESRD. Shortly thereafter, she was admitted
to the hospital with new onset hematemesis secondary to new fungating
mass in the gastric antrum. Upper GI series showed diffuse thickening of
the esophagus thought to be secondary to recurrence of SMT although not
confirmed with biopsy given her tenuous clinical state. In the setting
of heart failure and ESRD, the mass was deemed inoperable. She was no
longer eligible for transplant and was placed on comfort care and passed
away in the hospital due to progressive disease.