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.
- PTLD = complication of immunosuppression after solid organ or hematopoietic stem cell transplant. Majority are associated with EBV infection.
- Solid organ transplantations with highest risk of developing PTLD: heart, lung, intestinal and multiorgan transplants
- 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.