PMC6930172_01
Computed tomography (CT), chest. (A) Disseminated atypical pulmonary infiltrates (day +145 after alloHSCT), (B) Chest CT following antimycotic therapy (day +170), (C) Disease progression after three courses of rituximab and two courses R-CHOP (day +228), (D) Complete remission (CR) after five courses of brentuximab vedotin and three courses of third-party EBV-specific T-cells.
fmed-06-00295-g0001.jpg
PMC6930172_01_fmed-06-00295-g0001.jpg
F1
A computed tomography (CT)-scan of the chest revealed disseminated pulmonary lesions (Figure 1A). However, no clinical improvement was observed, and CT-scan revealed rapid enlargement of the lesions (Figure 1B). Summarizing the results of histopathology, PET-CT staging (Figure 1A), and the history of EBV-reactivation (EBV-PCR negative at this time point) following alloHSCT, the diagnosis was monomorphic EBV-associated PTLD presenting as DLBCL in Ann Arbor stage IVB with multiple pulmonary lymphomas. However, along with deteriorating symptoms, another CT-scan documented further lymphoma progression (Figure 1C). Interpreting the agonizing cough and persistent dyspnea to be primarily caused by a left pulmonary lymphoma mass (Figure 1C), an individualized decision for surgery was made, and the patient underwent thoracotomy with left lower lobectomy, resulting in symptom relief. One year following the initial PTLD diagnosis, staging by PET-CT and cMRI demonstrated CR of all disease manifestations (Figure 1D, Supplementary Figure 1B).
Histopathology and immunohistochemistry of pulmonary tumor biopsies. (A) alphaCD20, (B) MiB1, (C) alphaLMP1, (D) alphaCD30.
fmed-06-00295-g0002.jpg
PMC6930172_01_fmed-06-00295-g0002.jpg
F2
Subsequent biopsy and histopathology gave the diagnosis of diffuse large B-cell lymphoma (DLBCL), displaying high cellular proliferation (MiB1 80%) and co-expression of EBV-derived proteins (LMP1), as well as CD20 and CD30 (Figure 2). Demonstration of a single enhancing lesion on brain MRI (Supplementary Figure 2) together with PCR-detection of EBV-DNA in the CSF was highly suggestive of a cerebral PTLD manifestation (biopsy refused by patient).