Primary mediastinal large B‐cell lymphoma
Primary mediastinal B‐cell lymphoma (PMBL) is a distinct subtype of diffuse large B‐cell lymphoma (DLBCL) arising from putative thymic B‐cells in the mediastinum and comprises 2–4% of all non‐Hodgkin lymphomas (NHLs) (Gaulard et al, 2008) – therefore it’s relatively rare, and has only recently (since Oct 2015) been treated as a separate entity, both diagnostically and therapeutically; concise information is scattered around and limited to very specific communities, hematology magazines and websites.
As the parents from Croatia whose teenage son was diagnosed with PMBCL, we wanted to make a single point of reference with all the recent articles (up to 2019) that we had found on diagnosis, various treatment regimens and post-treatment.
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All the recent articles (up to December 2019) that we had found on diagnosis, various treatment regimens and post-treatment.
16yr old male, accidental finding of mediastinal mass (X-rayed, and then confirmed by MR). Pre-therapy PET-CT provided baseline data: mass dimensions (i.e. volume), disease spread and SUVmax. Thoracic biopsy has confirmed PMBCL (including the 2nd opinion).
Before chemo, potential sterility side effects and sperm bank was discussed
Treatment was performed based on B-NHL BFM-04 protocol – 6 chemotherapy cycles + Rituximab (MabThera) with intrathecal methotrexate in each cycle, with 21-26 days between cycles (1st day to 1st day)
Various side effects were expected and observed during and after each chemotherapy cycle (high blood pressure, fever, liver issues, changes in blood parameters…)
After 3 cycles, interim PET-CT was performed, results (dimensions and SUVmax) assessed according to Deauville scale
Stem cells harvested
Between chemo cycle 5 and cycle 6, stem cells were harvested from peripheral blood
After 5 cycles, MR was performed to check on mediastinal mass regression
Additional 2 doses of Rituximab
After 6 cycles and the multidisciplinary assessment, it was agreed to continue with 2 additional cycles of Rituximab (21-26 days between cycles), and PET/CT is to be performed 4-6 weeks after the last dose of Rituximab.
In order to check the mediastinal mass regression, it was agreed to perform the control MR between Rituximab cycles 7 and 8.
After 8 cycles, final PET-CT was performed, results (dimensions and SUVmax) assessed according to Deauville scale. Remission confirmed.