Primary Mediastinal Large B-Cell Lymphoma (PMBCL)

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This blog is for informational purposes only and should not be taken as medical advice. Content is sourced from third parties, and we do not guarantee accuracy or accept any liability for its use. Always consult a qualified healthcare professional for medical guidance.

What is Primary Mediastinal Large B-Cell Lymphoma (PMBCL)?

Primary Mediastinal Large B-Cell Lymphoma (PMBCL) is an aggressive non-Hodgkin B-cell lymphoma arising in the mediastinum (thymus-derived), comprising 2-4% of NHLs and 10% of large B-cell lymphomas. It’s genetically similar to Hodgkin lymphoma, often affecting young women (median age 35), presenting as a large anterior mediastinal mass.

Symptoms

Symptoms from mass compression include cough, chest pain, shortness of breath, superior vena cava syndrome (facial swelling, hoarseness), B symptoms (fever, night sweats, weight loss), and fatigue. Advanced causes pleural effusion or swallowing difficulties.

Causes

Causes involve genetic alterations (CIITA, PD-L1 amplifications for immune evasion), with no clear environmental links. Risk factors include young age (20-40), female gender, and autoimmune diseases. In 2025, JAK2/STAT pathway is key.

Diagnosis

Diagnosis uses CT/PET showing mediastinal mass, biopsy with immunohistochemistry (CD20+, CD30+), and molecular testing for PD-L1. Staging via Lugano. In 2025, NGS identifies immune signatures.

Treatment

Treatment uses R-CHOP or DA-EPOCH-R chemotherapy, with 80-90% cure. Radiation for bulky disease. Relapsed cases use CAR-T (axicabtagene ciloleucel, 70% response). In 2025, bispecific antibodies (glofitamab) improve outcomes.

Future Outlook

In 2025, 5-year survival is 85-95% with chemoimmunotherapy. CAR-T and bispecifics raise relapsed survival to 60%. By 2030, PD-1 inhibitors could achieve 95% cure.

Sources

The information is based on PMC’s “Primary Mediastinal Large B-cell Lymphoma” for overview; Macmillan’s “Primary mediastinal large B-cell lymphoma (PMBCL)” for symptoms; Together by St. Jude’s “Primary Mediastinal Large B-Cell Lymphoma: What It Is” for understanding; Medscape’s “Mediastinal Lymphoma: Overview” for overview; PMC’s “Primary mediastinal large B cell lymphoma” for symptoms; MyLymphomaTeam’s “Primary Mediastinal B-Cell Lymphoma: An Overview” for overview; Onclive’s “Latest Advances in the Management of Primary Mediastinal B-Cell Lymphoma” for treatment; ASH’s “Primary Mediastinal B-Cell Lymphoma: How I Treat In Brief” for treatment; Frontiers in Oncology’s “Primary Mediastinal B-Cell Lymphoma: Novel Precision Therapies” for therapies; AOL’s “Primary mediastinal large B-cell lymphoma: state of the art” for review.