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