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Case Presentation Conference Children’s Hospital of New Orleans

Case Presentation Conference Children’s Hospital of New Orleans. James M. Roth M.D. Evelyn Kluka M.D. History. 13 year-old Hispanic male R.G. Chief Complaint: Headache, Left Ear Pain with radiation of the pain to the cheek. History of Present Illness.

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Case Presentation Conference Children’s Hospital of New Orleans

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  1. Case Presentation ConferenceChildren’s Hospital of New Orleans James M. Roth M.D. Evelyn Kluka M.D.

  2. History • 13 year-old Hispanic male R.G. • Chief Complaint: Headache, Left Ear Pain with radiation of the pain to the cheek

  3. History of Present Illness • 1 month history of progressive left sided facial pain and tingling • Recent stuffy nose with clear discharge • Odynophagia

  4. Past Medical History • Esophageal Varices • Hematochezia • Jaundice • Cirrhotic liver disease

  5. Past Surgical History • Liver Transplant 6 months prior to admission • Left myringotomy by an ENT in Dallas secondary to disequilibrium, tinnitus, and serous fluid collection

  6. Medications • Bactrim- prophylaxis • Ganciclovir- prophylaxis • Procardia XL • Magnesium • Prednisone • Neoral- Cyclosporine anti-rejection drug • Cellcept-

  7. Allergies/ Immunizations • No known drug allergies • No immunizations since liver transplant • Immunizations up to date till then

  8. Social History • Born in Mexico • Lives with mother currently in Dallas

  9. Physical Exam • Vital Signs normal • General: Awake alert • Ears: Right TM clear; Left TM slightly reddened with some fluid present • Nose: Reddened inferior turbinates no drainage

  10. Physical Exam • Oropharynx: Tonsils 1-2+ symmetric, uvula midline normal tongue mobility tongue soft to palpation • Neck: Small < 1 cm nodes scattered throughout neck • Face: Slight swelling to the left midface

  11. Physical Exam • Neurological: V2 and V3 with decreased sensation on the left side. Remaining cranial nerves grossly intact.

  12. Admission • Originally evaluated Dallas and CT scan showed a nasal mass • Admitted by GI/Transplant team and ENT service was consulted for biopsy

  13. MRI • Mass filling the nasopharynx compressing or encompassing the left Eustachian tube with area of central necrosis

  14. Intraoperative Findings • Fungating gray mass filling most of the nasopharynx slight more on the left than the right • Very solid in nature and avascular

  15. Lab Work • EBV titers IgM elevated • CBC wnl • Chem 7 wnl • PT/PTT wnl

  16. Surgical Pathology • Large lesion 3.5x1.5x.5 cm • Lymphoid lesion • Polyclonal cells: small mature lymphocytes, large active immunoblast, T cells, B cells, Strongly EBV positive

  17. Diagnosis • Post Transplant Lymphoproliferative Disease (PTLD): Polyclonal Variant

  18. PTLD • The presence of an abnormal proliferation of lymphoid cells • Highly related to EBV infection • Related to the type of solid organ transplanted • More common in children • Originally described in 1969 in 5 renal transplant patients

  19. Pathology • Several variants from benign polyclonal B cell hyperplasia to malignant monoclonal lymphoma • The progression to a monoclonal population leads to a more aggressive and malignant tumor

  20. Why transplant patient’s? • Immunosuppression is targeted against T cells especially cytotoxic T cells • These cells help to self regulate the immune system • With certain viral infection you get B cell proliferation • These cells can progress in an unregulated manner

  21. EBV Infection • Causes an active B cell proliferation • Linked to Burkitt’s lymphoma and nasopharyngeal cancer • R.G. was originally seronegative prior to transplantation • His runny nose and sore throat may have represented a recent EBV infection

  22. Common Presentation • Mononucleosis type infection • Febrile illness with leukopenia • Focal organ system failure • GI tract: endoscopy, CT scans • CNS: lumbar puncture • Lymph node involvement

  23. Solid Organ Transplant • Renal- 1% • Liver- 2-3% • Heart- 4-10%

  24. Risk Factors • Young age: Increased risk of primary EBV infections in the early post transplant period • Agents: Not any single agent more responsible but the cumulative intensity of immunosuppression seems to be most important.

  25. Treatment • Decrease immunosuppression • Antivirals: acyclovir ganciclovir • Immunoglobulins: IVIG which helps to target CMV • Chemotherapuetics: Rituximab (CD20 ligand) • Radiotherapy

  26. Conclusions • PTLD is a rare complication of transplantation- 2% of all solid organ transplant recipients • More common in children secondary to primary exposure to EBV • May present in the head and neck especially do to the rich lymphatic system • Treatable as long as there is not monoclonal proliferation

  27. R.G. • Underwent treatment at a variety of levels • Immunosuppression was decreased • Given IVIG, Acyclovir, Ganciclovir • Started on Rituximab • Received radiation therapy treatments • Repeat MRI did eventually show regression of disease

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