Survey
* Your assessment is very important for improving the workof artificial intelligence, which forms the content of this project
Download Case Presentation Conference Children`s Hospital of New Orleans
Document related concepts
Transcript
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 • 1 month history of progressive left sided facial pain and tingling • Recent stuffy nose with clear discharge • Odynophagia Past Medical History • • • • Esophageal Varices Hematochezia Jaundice Cirrhotic liver disease 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 Medications • • • • • • • Bactrim- prophylaxis Ganciclovir- prophylaxis Procardia XL Magnesium Prednisone Neoral- Cyclosporine anti-rejection drug Cellcept- Allergies/ Immunizations • No known drug allergies • No immunizations since liver transplant • Immunizations up to date till then Social History • Born in Mexico • Lives with mother currently in Dallas 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 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 Physical Exam • Neurological: V2 and V3 with decreased sensation on the left side. Remaining cranial nerves grossly intact. Admission • Originally evaluated Dallas and CT scan showed a nasal mass • Admitted by GI/Transplant team and ENT service was consulted for biopsy MRI • Mass filling the nasopharynx compressing or encompassing the left Eustachian tube with area of central necrosis Intraoperative Findings • Fungating gray mass filling most of the nasopharynx slight more on the left than the right • Very solid in nature and avascular Lab Work • • • • EBV titers IgM elevated CBC wnl Chem 7 wnl PT/PTT wnl 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 Diagnosis • Post Transplant Lymphoproliferative Disease (PTLD): Polyclonal Variant 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 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 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 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 Common Presentation • Mononucleosis type infection • Febrile illness with leukopenia • Focal organ system failure – GI tract: endoscopy, CT scans – CNS: lumbar puncture – Lymph node involvement Solid Organ Transplant • Renal- 1% • Liver- 2-3% • Heart- 4-10% 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. Treatment • Decrease immunosuppression • Antivirals: acyclovir ganciclovir • Immunoglobulins: IVIG which helps to target CMV • Chemotherapuetics: Rituximab (CD20 ligand) • Radiotherapy 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 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
