26 May 2022

11/19/20: Post-Transplant Lymphoproliferative Disease (PTLD)

PGY2 Young Hsu presented a case of a 68 year old female with history of NASH cirrhosis s/p orthotopic liver transplant in 9/2016, atrial fibrillation s/p cardioversion in 10/2018 who presented with forgetfulness for 3 weeks.  She has had nonspecific headaches for ~1 month and nausea.  Her family noted that she has become particularly forgetful.  She could not remember her friends visiting her only after 10minutes.  She has urinary incontinence for ~10 days, but denied dysuria or itchiness.  She was treated for Klebsiella UTI with Bactrim 1 week prior to presentation.  Home medications include tacrolimus 2mg qAM/1.5mg qPM, Mycophenolate mofetil 1g BID, Bactrim DS and omeprazole.  She lives with her husband, no pets a home.  No recent travel or sick contact.  Denies any tobacco/alcohol/substance use.

Vitals: T 36.5, HR 54, RR 15, BP 142/63, O2 sat 98% on RA

Physical Exam significant for female with large body habitus, normal cardiac and pulm exam, A&Ox1, mild tremor on end point, neck flexion soft.

CBC: WBC 4.89> Hb 12.9/Hct 38.3 <Plt 140 (MCV 86.5, RDW 13%)


Na 137/K 4.7/Cl 105/HCO3 21/BUN 27/Cr 1.64 <Glucose 93  (Baseline Cr: 1.1-1.2)

ALP 79>T.protein 7.2/Albumin 4.4/AST 26/ALT 21/Tbili 0.5

Ca 9.6, Mg 2.5, Phos 3.8

Coags: PT 16, INR 1.3

Infectious workup started:

  • BCx, UA, fungal Cx, COVID-19 negative
  • Serum viral panel including HIV, RPR, EBV, CMV all negative
  • Serum fungal studies negative
  • LP performed with the following findings:
    • RBC 36
    • WBC 127
    • 100 mononuclear cells
    • Glucose: 43 mg/dL (normal 40-70)
    • Protein: 233 mg/dL (normal 15-45)
    • CSF Culture: no growth
    • CSF AFB Culture: no growth
    • CSF Biofire Panel: EBV detected
    • CSF West Nile negative
    • CSF Cytology: atypical lymphocytosis consistent with B cell lymphoproliferative disorder. In the setting of transplant-related immunosuppression, findings are compatible with a PTLD.

MRI Brain w/ and w/o Contrast showed multifocal brain lesions with extensive adjacent vasogenic edema.

TEE without any valvular dysfunction or valvular masses.

Treatment: Immunosuppression was decreased by hepatology after diagnosis. She was started on dexamethasone for vasogenic edema and started on Rituxan, Methotrexate, Vincristine and Porcarbazine (R-MVP). Patient is now s/p 5 cycles of R-MVP, 6 doses of IT methotrexate and CNS disease clearance seen since the third dose of IT methotrexate.

Learning Points:

  1. PTLD = complication of immunosuppression after solid organ or hematopoietic stem cell transplant. Majority are associated with EBV infection.
  2. Solid organ transplantations with highest risk of developing PTLD: heart, lung, intestinal and multiorgan transplants
  3. T cell immunity plays a dominant role in PTLD pathogenesis. Certain T cell suppressive agents such as anti-CD3 Ab, anti-thymocyte globulin, tacrolimus and cyclosporine have been associated with increased risk of PTLD.

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