Select Page

Primary mediastinal large B‐cell lymphoma

PMBCL

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. 

Disclaimer: The content of this non-commercial website is for information purposes only. Information provided here shouldn’t and mustn’t be used as a replacement or an alternative to an official, expert MD opinion. All the links are publicly available, unless specified differently.

PMBCL

Articles

All the recent articles (up to December 2019) that we had found on diagnosis, various treatment regimens and post-treatment. 

2005 Reiter et al.

NHL-BFM Leitfaden zur Diagnostik, Klassifizierung, Staging und Stratifizierung in Therapiegruppen (GERMAN)

2010 Bilic et al.

CD20 Positive Childhood B-non Hodgkin Lymphoma (B-NHL): Morphology, Immunophenotype and a Novel Treatment Approach: A Single Center Experience

2010 Algrin et al.

Interim-Positron Emission Tomography with [18F]Fluorodeoxyglucose (interim-PET) Evaluation In Mediastinal Lymphoma Including Hodgkin Lymphoma (HL) and Primary Mediastinal Large B-Cell Lymphoma (PMBL)

2011 Pohlen et al.

Efficacy and toxicity of a rituximab and methotrexate based regimen (GMALL B‐ALL/NHL 2002 protocol) in Burkitt's and primary mediastinal large B‐cell lymphoma

2012 Prahladan et al.

Interim PET Scan Does Not Predict Outcome in Primary Mediastinal B Cell Lymphoma Treated with R DA EPOCH

2013 Dunleavy et al.

Dose-Adjusted EPOCH-Rituximab Therapy in Primary Mediastinal B-Cell Lymphoma

2013 Woesmann et al.

Therapy in PMBCL - New England Journal of Medicine

2013 Itti et al.

An international confirmatory study of the prognostic value of early PET/CT in diffuse large B-cell lymphoma: comparison between Deauville criteria and SUVmax

2014 Dabrowska-Iwanicka et al.

PMBCL

2014 Avivi et al.

Autologous Stem Cell Transplantation for Primary Mediastinal B Cell Lymphoma in the Rituximab Era: A Retrospective Study By the EBMT Lymphoma Working Party

2014 Martelli et al.

FDG PET predicts survival after Chemoimmunotherapy for PMBCL - Results of the International Extranodal Lymphoma Study Group IELSG-26 Study

2014 Prochazka et al.

Prognostic Impact of Interim and Final PET in Primary Mediastinal Large B-Cell Lymphoma Treated with Intensive Etoposide-Based Therapy

2014 Romejko-Jarosinska et al.

Relapsed or refractory primary mediastinal B-cell lymphoma: Evaluation of outcome of a minority from 125 patients treated at a single institution

2014 Nagle et al.

The role of FDG-PET imaging as a prognostic marker of outcome in PMBCL

2015 El Mallawany et al.

Advances in the Diagnosis and Treatment of Childhood and Adolescent B-Cell Non-Hodgkin Lymphoma

2015 Tavares et al.

Treatment Outcomes of Patients with Primary Mediastinal Diffuse Large B-Cell Lymphoma: A Single-Center Experience

2015 Svoboda et al.

Brentuximab Vedotin in Combination with Multi-Agent Chemotherapy Is Well Tolerated and Shows Promising Activity As Frontline Treatment for Primary Mediastinal B-Cell Lymphoma

2015 Vassilakopoulos et al.

A 'PET' topic in primary mediastinal large Bcell lymphoma: positive or negative, and how to handle it in the end

2015 Dunleavy et al.

Primary mediastinal B-cell lymphoma and mediastinal gray zone lymphoma: do they require a unique therapeutic approach?

2015 Duehrsen et al.

The role of end-of-treatment PET in lymphoma management

2015 Pinnix et al.

Single-institution experience in the treatment of PMBCL tretaed with Immunochemotherapy in the Setting of response assessment by FDG PET

2015 Gleeson et al.

R-CHOP in Primary Mediastinal B-Cell Lymphoma (PMBL): Results from the UK NCRI R-CHOP 14 v 21 Trial

2015 Petkovic

Current trends in the treatment of primary mediastinal large B-cell lymphoma – an overview

2015 Burke et al.

Intergroup trial for children or adolescents with B-cell NHL or B-AL: evaluation of Rituximab efficacy and safety in high-risk patients

2015 Reiter et al.

Multicentre observational study for treatment of children and adolescents with mature B-cell Non-Hodgkin-Lymphoma/leukemia

2015 Aoki et al.

High-dose chemotherapy followed by autologous stem cell transplantation for relapsed/refractory primary mediastinal large B-cell lymphoma

2016 Hochberg et al.

Adolescent and young adult non‐Hodgkin lymphoma

2016 Kocurek et al.

Primary mediastinal B-cell lymphoma – metabolic and anatomical features in 18FDG-PET/CT and response to therapy

2016 Filippi et al.

Prognostic Role of Pre-Radiation Therapy (18)F-Fluorodeoxyglucose Positron Emission Tomography for Primary Mediastinal B-Cell Lymphomas Treated with R-CHOP or R-CHOP-Like Chemotherapy Plus Radiation.

2017 Kahn et al.

Adolescent and young adult lymphoma: collaborative efforts toward optimizing care and improving outcomes

2017 Adams et al.

Interim FDG-PET in lymphoma, a questionable practice in hematology

2017 Dunleavy

Primary mediastinal B-cell lymphoma: biology and evolving therapeutic strategies

2017 Giulino-Roth et al.

Outcomes of adults and children with PMBCL treated with DA-EPOCH-R

2017 MD Anderson Center, Texas University

PMBCL Algorithm

2017 Ceriani et al.

Prognostic models for PMBCL derived from 18-FDG PET/CT quantitative parameters in the IELSG 26 study

2017 Zinzani et al.

Safety and tolerability of pembrolizumab in patients with relapsed/refractory primary mediastinal large B-cell lymphoma

2017 Younes et al.

The landscape of new drugs in lymphoma

2017 Barrington et al.

FDG PET for therapy monitoring in Hodgkin and non-Hodgkin lymphomas

2017 Shah et al.

R‐CHOP versus dose‐adjusted R‐EPOCH in frontline management of primary mediastinal B‐cell lymphoma: a multi‐centre analysis

2017 Savage et al.

PMBCL

2018 Pinnix et al.

Positron emission tomography–computed tomography predictors of progression after DA-R-EPOCH for PMBCL

2018 Melani et al.

End-of-treatment and serial PET imaging in PMBCL following DA/EPOCH-R, a paradigm shift in clinical decision making

2018 Dunleavy et al.

Management of aggressive B-cell NHLs in the AYA population: an adult vs pediatric perspective

2018 Broccoli et al.

The unique biology and treatment of primary mediastinal B-cell lymphoma

2018 Ceriani et al.

Metabolic heterogeneity on baseline 18FDG-PET/CT scan is a predictor of outcome in primary mediastinal B-cell lymphoma

2018 Giulino-Roth

How I Treat Primary Mediastinal B-cell Lymphoma

2018 Vardhana et al.

Outcomes of Relapsed and Refractory Primary Mediastinal (Thymic) Large B Cell Lymphoma Treated with Second-Line Therapy and Intent to Transplant

2018 Messmer et al.

R-CHOP without radiation in frontline management of primary mediastinal B-cell lymphoma

2018 Avivi

Ten things to know about PMBCL

2018 Michalarea et al.

Durable Remissions Achieved with R-CHOP Chemotherapy without Radiotherapy in Patients with Primary Mediastinal B-Cell Lymphoma - the Royal Marsden Experience

2018 Avivi et al.

Autologous stem cell transplantation for primary mediastinal B-cell lymphoma: long-term outcome and role of post-transplant radiotherapy. A report of the European Society for Blood and Marrow Transplantation.

2019 Lees et al.

Biology and therapy of PMBCL: current status and future directions

2019 Cwynarski et al.

The management of primary mediastinal B‐cell lymphoma: a British Society for Haematology Good Practice Paper

2019 Tomassetti et al.

The role of pembrolizumab in relapsed/refractory primary mediastinal large B-cell lymphoma

CATEGORIES

Our Story

Real-life case

^
March 2019

Diagnosis

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).

^
April 2019

Preparation

Before chemo, potential sterility side effects and sperm bank was discussed 

^
April 2019

Starting treatment

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…)

^
June 2019

Interim PET-CT

After 3 cycles, interim PET-CT was performed, results (dimensions and SUVmax) assessed according to Deauville scale

^
August 2019

Stem cells harvested

Between chemo cycle 5 and cycle 6, stem cells were harvested from peripheral blood

^
August 2019

Chest MRI

After 5 cycles, MR was performed to check on mediastinal mass regression

^
September 2019

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. 

^
September 2019

Chest MRI

In order to check the mediastinal mass regression, it was agreed to perform the control MR between Rituximab cycles 7 and 8.

^
November 2019

Control PET-CT

After 8 cycles, final PET-CT was performed, results (dimensions and SUVmax) assessed according to Deauville scale. Remission confirmed.