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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

Case Presentation ConferenceChildren’s Hospital of New Orleans

James M. Roth M.D.

Evelyn Kluka M.D.

history
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
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
Past Medical History
  • Esophageal Varices
  • Hematochezia
  • Jaundice
  • Cirrhotic liver disease
past surgical history
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
Medications
  • Bactrim- prophylaxis
  • Ganciclovir- prophylaxis
  • Procardia XL
  • Magnesium
  • Prednisone
  • Neoral- Cyclosporine anti-rejection drug
  • Cellcept-
allergies immunizations
Allergies/ Immunizations
  • No known drug allergies
  • No immunizations since liver transplant
  • Immunizations up to date till then
social history
Social History
  • Born in Mexico
  • Lives with mother currently in Dallas
physical exam
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 exam1
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 exam2
Physical Exam
  • Neurological: V2 and V3 with decreased sensation on the left side. Remaining cranial nerves grossly intact.
admission
Admission
  • Originally evaluated Dallas and CT scan showed a nasal mass
  • Admitted by GI/Transplant team and ENT service was consulted for biopsy
slide13
MRI
  • Mass filling the nasopharynx compressing or encompassing the left Eustachian tube with area of central necrosis
intraoperative findings
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
Lab Work
  • EBV titers IgM elevated
  • CBC wnl
  • Chem 7 wnl
  • PT/PTT wnl
surgical pathology
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
Diagnosis
  • Post Transplant Lymphoproliferative Disease (PTLD): Polyclonal Variant
slide18
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
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
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
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
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
Solid Organ Transplant
  • Renal- 1%
  • Liver- 2-3%
  • Heart- 4-10%
risk factors
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
Treatment
  • Decrease immunosuppression
  • Antivirals: acyclovir ganciclovir
  • Immunoglobulins: IVIG which helps to target CMV
  • Chemotherapuetics: Rituximab (CD20 ligand)
  • Radiotherapy
conclusions
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
slide27
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