Mediastinal gray-zone lymphoma

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Last updated on 2024-07-23:2 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-R-EPOCH==
DA-R-EPOCH: Dose Adjusted Rituximab, Etoposide, Prednisone, Oncovin (Vincristine), Cyclophosphamide, Hydroxydaunorubicin (Doxorubicin)
DA-EPOCH-R
===Regimen===
Study 	Dates of enrollment	Evidence
Wilson et al. 2014 (NCI 93-C-0133MGZL)	Not reported 	Phase 2
Note: this should be considered a substudy under the master protocol NCI 93-C-0133.
====Targeted therapy====
*Rituximab (Rituxan) 375 mg/m2 IV once per cycle or 1 day before the start of EPOCH (depending on reference)
====Chemotherapy====
*Etoposide (Vepesid) 50 mg/m2/day IV continuous infusion over 96 hours, started on day 1 (total dose per cycle: 200 mg/m2)
*Vincristine (Oncovin) 0.4 mg/m2/day IV continuous infusion over 96 hours, started on day 1 (total dose per cycle: 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 over 96 hours, started on day 1 (total dose per cycle: 40 mg/m2)
====Glucocorticoid therapy====
*Prednisone (Sterapred) 60 mg/m2 PO twice per day on days 1 to 5
====Supportive therapy====
*Filgrastim (Neupogen) 5 mcg/kg SC once per day, starting on day 6 and continuing until ANC greater than 5000/μL past nadir
*PCP prophylaxis with ONE of the following:
**Trimethoprim-Sulfamethoxazole (Bactrim DS) 160/800 mg PO twice per day 3 days per week 
***Alternative used only in García-Suárez et al. 2007: cotrimoxazole 480 mg PO twice per day 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 mcg/kg SC when hemoglobin concentration was less than or equal to 10 g/dL.
21-day cycle for 6 to 8 cycles
====Dose and schedule modifications====
*Start cycle 1 as described above.
*Obtain CBCs twice per week for nadir measurements.
*If nadir ANC greater than 500/μL, increase etoposide, doxorubicin, and cyclophosphamide by 20% compared to previous cycle.
*If nadir ANC less than 500/μL on 1 or 2 measurements, use same doses as last cycle.
*If nadir ANC less than 500/μL on at least 3 measurements, decrease etoposide, doxorubicin, and cyclophosphamide by 20% compared to previous cycle.
*And/or if nadir platelet count less than 25 x 109/L 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 greater than 1000/μL and platelets greater than 100 x 109/L. If counts are below those levels, check daily CBC and continue growth factor support until counts are adequate and next cycle can start.
===References===
# NCI 93-C-0133MGZL: 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 dosing details in abstract have been reviewed by our editors link to PMC article EuroPMC
=Relapsed or refractory=
==Brentuximab vedotin monotherapy==
===Regimen===
Study 	Dates of enrollment	Evidence
Jacobsen et al. 2015 (SGN35-012CD30+B-NHL)	2011-08 to 2013-08 	Phase 2, fewer than 20 pts in this subgroup
Note: This regimen was evaluated in patients with CD30+ non-Hodgkin lymphoma, as determined by immunohistochemistry.
====Antibody-drug conjugate therapy====
*Brentuximab vedotin (Adcetris) 1.8 mg/kg IV over 30 minutes once on day 1
21-day cycles
===References===
# SGN35-012CD30+B-NHL: Jacobsen ED, Sharman JP, Oki Y, Advani RH, Winter JN, Bello CM, Spitzer G, Palanca-Wessels MC, Kennedy DA, Levine P, Yang J, Bartlett NL. Brentuximab vedotin demonstrates objective responses in a phase 2 study of relapsed/refractory DLBCL with variable CD30 expression. Blood. 2015 Feb 26;125(9):1394-402. Epub 2015 Jan 8. link to original article dosing details in manuscript have been reviewed by our editors EuroPMC NCT01421667
Category:Mediastinal gray-zone lymphoma regimens
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Category:Aggressive non-Hodgkin lymphomas
Category:B-cell lymphomas