Mediastinal gray-zone lymphoma

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2 regimens on this page 2 variants on this page

Note: Mediastinal gray-zone lymphome (MGZL) is considered intermediate between primary mediastinal B-cell lymphoma (PMBL) and nodular sclerosis Hodgkin lymphoma (NSHL). Given that it is treated more similarly to the non-Hodgkin lymphoma, it is included here.

Untreated

DA-EPOCH-R

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DA-R-EPOCH: Dose Adjusted Etoposide, Prednisone, Oncovin (Vincristine), Cyclophosphamide, Hydroxydaunorubicin (Doxorubicin), Rituximab

Synonyms: DA-R-EPOCH-R

Structured Concept: C63461 (NCI-T), C1882521 (NCI-MT/UMLS)

Regimen

Study	Evidence
Wilson et al. 2014	Phase II

Chemotherapy

• Rituximab (Rituxan) 375 mg/m² IV once per cycle or 1 day before the start of EPOCH (depending on reference)
• Etoposide (Vepesid) 50 mg/m²/day IV continuous infusion on days 1 to 4 (total dose of 200 mg/m²)
• Prednisone (Sterapred) 60 mg/m² PO BID on days 1 to 5
• Vincristine (Oncovin) 0.4 mg/m²/day IV continuous infusion on days 1 to 4 (total dose of 1.6 mg/m²)
• Cyclophosphamide (Cytoxan) 750 mg/m² IV over 15 minutes once on day 5
• Doxorubicin (Adriamycin) 10 mg/m²/day IV continuous infusion on days 1 to 4 (total dose of 40 mg/m²)

Supportive medications

• Filgrastim (Neupogen) 5 mcg/kg SC once per day, starting on day 6 and continuing until ANC >5,000/μL past nadir
• PCP prophylaxis with any one of the following:
  • Trimethoprim/Sulfamethoxazole (Bactrim DS) 160/800 mg PO BID 3 days per week
    • Alternative used only in García-Suárez et al. 2007: cotrimoxazole 480 mg PO BID 3 days per week
  • Atovaquone (Mepron) 1500 mg PO once per day
  • Pentamidine (Nebupent) 300 mg nebulized every 28 days
• Only in García-Suárez et al. 2007: Darbepoetin alfa (Aranesp) 2.25 μg/kg SC when hemoglobin concentration was ≤100 g/l.

21-day cycle for 6 to 8 cycles

Dose-adjustments

• Start cycle 1 as described above.
• Obtain CBCs twice per week for nadir measurements.
• If nadir ANC >500, increase etoposide, doxorubicin, and cyclophosphamide by 20% compared to previous cycle.
• If nadir ANC <500 on 1 or 2 measurements, use same doses as last cycle.
• If nadir ANC <500 on at least 3 measurements, decrease etoposide, doxorubicin, and cyclophosphamide by 20% compared to previous cycle.
• And/or if nadir platelet count <25 on at least 1 measurement, decrease etoposide, doxorubicin, and cyclophosphamide by 20% compared to previous cycle.
• Dose adjustments below the cycle 1 starting dose only applies to cyclophosphamide. The lowest etoposide and doxorubicin would be dosed at is the original cycle 1 dose.
• Can start new cycle every 21 days if ANC >1,000 and platelets >100. If counts are below those levels, check daily CBC and continue growth factor support until counts are adequate and next cycle can start.

References

1. Wilson WH, Pittaluga S, Nicolae A, Camphausen K, Shovlin M, Steinberg SM, Roschewski M, Staudt LM, Jaffe ES, Dunleavy K. A prospective study of mediastinal gray-zone lymphoma. Blood. 2014 Sep 4;124(10):1563-9. Epub 2014 Jul 14. link to original article contains protocol PubMed

Relapsed/Refractory

Brentuximab vedotin (Adcetris)

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Regimen

Study	Evidence
Bartlett et al. 2013	Phase II, <20 pts

This regimen was evaluated in patients with CD30+ non-Hodgkin lymphoma, as determined by immunohistochemistry.

Chemotherapy

• Brentuximab vedotin (Adcetris) 1.8 mg/kg IV over 30 minutes once on day 1

21-day cycles, given until progression or unacceptable toxicity

References

1. Abstract: Nancy L. Bartlett, MD, Jeff P. Sharman, MD, Yasuhiro Oki, MD, Ranjana H. Advani, MD, Celeste M. Bello, MD, Jane N. Winter, MD, Yin Yang, MS, Dana A. Kennedy, PharmD and Eric D. Jacobsen, MD. A Phase 2 Study Of Brentuximab Vedotin In Patients With Relapsed Or Refractory CD30-Positive Non-Hodgkin Lymphomas: Interim Results In Patients With DLBCL and Other B-Cell Lymphomas. ASH Abstract 848. link to abstract