Post Transplant Lymphoproliferative Disorder (PTLD) is an uncommon complication after solid organ transplant, with several different monomorphic subtypes; Burkitt Lymphoma PTLD (B-PTLD) is particularly rare and aggressive.
A 30 year old female with a history of IgA nephropathy and two renal transplants presented to the emergency room with nausea, vomiting, and diffuse abdominal distention and pain that started one week prior to presentation. CT imaging showed significant mesenteric and pelvic lymphadenopathy with moderate ascites, paracentesis was performed. Her immunosuppressive therapy was adjusted: azathioprine was held, tacrolimus was decreased for lower target level, and steroid was continued. A lymph node biopsy was attempted but unable to be preformed due to significant edema. Patient left against medical advice but presented 24 hours later to an outside hospital where all immunosuppressive therapy was held, including steroids. Her condition rapidly deteriorated over the following few days she developed profound electrolyte abnormalities, hematemesis, and fever, shock requiring vasopressor support, intubation, and continuous renal replacement therapy. She was transferred back to our facility where ICU level care was continued with the addition of steroids. Ascitic fluid studies were consistent with monomorphic PTLD favoring Burkitt Lymphoma. Lumbar puncture was negative for malignancy. Family initially declined chemotherapy but the patient’s condition improved and she was able to be extubated and consented to chemotherapy without significant delay in care. Treatment was started with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) and malignant ascites resolved. CRRT was transitioned to intermittent hemodialysis and then discontinued after recovery of transplanted kidney. She was discharged home without further hospitalizations one month post discharge. Outpatient PET imaging after second round of R-CHOP showed significant improvement of disease.
Our case demonstrates B-PTLD without CNS involvement that responded well to R-CHOP therapy without significant complications.
Sager M. Aggarwal D. Late onset Burkitt transplant lymphoproliferative disorder (PTLD) following solid organ transplant. Oral presentation at: American College of Physicians-Michigan, 2021 Michigan Chapter Virtual Meeting. 2021 Oct 14-17;Virtual.