Case presentation conference children s hospital of new orleans
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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

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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


Case presentation conference children s hospital of new orleans

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


Case presentation conference children s hospital of new orleans

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


Case presentation conference children s hospital of new orleans

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


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