1 / 27

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.

bishop
Télécharger la présentation

Case Presentation Conference Children’s Hospital of New Orleans

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  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

More Related