Case Presentation Conference Children’s Hospital of New Orleans James M. Roth M.D. Evelyn Kluka M.D.
Feb 08, 2016
Case Presentation ConferenceChildren’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