Abstract: SA-PO942

A Rare PTLD Presenting as Autonomic Dystonia: Who Would Have Thought

Session Information

Category: Nephrology Education

  • 1302 Fellows and Residents Case Reports

Authors

  • Karmegam, Sathish, Methodist Dallas Medical Center, Dallas, Texas, United States
  • Fa, Kosunarty, Methodist Dallas Medical Center, Dallas, Texas, United States
  • Castillo-Lugo, Jose A., Methodist Dallas Medical Center, Dallas, Texas, United States
Background

Introduction: Post Transplant Lymphoproliferative Disorders (PTLD) are life threatening complications of transplantation. Chronic use of immunosuppressive agents to prevent allograft rejection increases the long-term risk of malignancy. This is a report of a rare case of Diffuse Large B Cell Lymphoma presenting with autonomic dysfunction as a paraneoplastic syndrome.

Methods

Case Description: A 40 y/o Caucasian man with PMH of Living Unrelated Kidney Transplant (about 15 months ago), acute vascular rejection 2B (within 6 months of transplant), and HTN presented with fatigue and bloody diarrhea. His medications were tacrolimus, MMF, prednisone and lisinopril. He had severe orthostatic hypotension with recurrent syncopal episodes on admission. Hgb and chemistries were normal. Lisinopril was discontinued. He received aggressive volume expansion, oral fludrocortisone, and midodrine. Infectious stool work up was negative. CMV viral load was weakly positive. He was started on IV ganciclovir empirically. His adrenal axis, cardiac function and CT of abdomen were normal. CMV adrenalitis was ruled out and ganciclovir discontinued. His EBV PCR turned out to be strongly positive. IV acyclovir was started. Chest CT showed no central lymphadenopathy but small left axillary lymphadenopathy. A colonoscopy showed ‘multiple shallow ulcers throughout the colon with heaped up margins’. Biopsy was negative for CMV inclusion bodies. Colonic mucosa showed atypical large cell lymphoid infiltrate in the lamina propria consistent with ‘Diffuse large B-Cell Lymphoma’ (DLBC). Axillary lymph node excision biopsy was consistent with ‘Diffuse large B-cell lymphoma and T-lymphocyctes with positive CD-20. MRI of the brain was negative. MMF was discontinued and tacrolimus dose was decreased. Hematology service recommended R-CHOP chemotherapy. His autonomic dysfunction improved with treatment.

Conclusion

Discussion: Lymphomas are among the most common complications of transplantation and many are related to EBV infection. The incidence is highest in the first post-transplant year due to high immunosuppression. Autodystonia is a well-known paraneoplastic syndrome of CNS and Hodgkin’s lymphomas. High level of suspicion is required to make diagnosis and initiate treatment early. DLBC lymphoma presenting with autodystonia is extremely rare and no other cases have been reported until now.