Large granular lymphocytic leukemia

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*We have moved How I Treat articles to a dedicated page.
Last updated on 2024-07-23:3 regimens on this page
3 variants on this page
=Guidelines=
Given the rapid change in evidence in many areas of hematology/oncology, readers are encouraged to consider any guideline published 5+ years ago to be for historical purposes, only.
==NCCN==
*NCCN does not currently have guidelines at this granular level; please see NCCN Guidelines - T-cell Lymphomas.
=Initial therapy=
==Cyclophosphamide monotherapy== 
===Regimen===
Study 	Dates of enrollment	Evidence
Bareau et al. 2010	1999-2007 	Retrospective
Note: These are retrospective series, of which Bareau et al. 2010 is representative. The most common dose reported is 100 mg PO once per day; some authors mentions co-administration of steroids but details are not supplied.
====Immunosuppressive therapy====
*Cyclophosphamide (Cytoxan) 50 to 100 mg PO once per day
Lamy et al. 2011 recommends that patients who are responding are continued on treatment for 6 to 12 months; patients who do not respond within 4 months of therapy are discontinued
===References===
# Retrospective: Go RS, Li CY, Tefferi A, Phyliky RL. Acquired pure red cell aplasia associated with lymphoproliferative disease of granular T lymphocytes. Blood. 2001 Jul 15;98(2):483-5. link to original article EuroPMC
# Retrospective: Bareau B, Rey J, Hamidou M, Donadieu J, Morcet J, Reman O, Schleinitz N, Tournilhac O, Roussel M, Fest T, Lamy T. Analysis of a French cohort of patients with large granular lymphocyte leukemia: a report on 229 cases. Haematologica. 2010 Sep;95(9):1534-41. Epub 2010 Apr 7. link to original article link to PMC article EuroPMC content property of HemOnc.org
# Retrospective: Moignet A, Hasanali Z, Zambello R, Pavan L, Bareau B, Tournilhac O, Roussel M, Fest T, Awwad A, Baab K, Semenzato G, Houot R, Loughran TP Jr, Lamy T. Cyclophosphamide as a first-line therapy in LGL leukemia. Leukemia. 2014 May;28(5):1134-6. Epub 2013 Nov 27. link to original article link to PMC article EuroPMC
==Methotrexate & Prednisone==
===Regimen===
Study 	Dates of enrollment 	Evidence
Loughran et al. 2014 (ECOG E5998)	1999-07-16 to 2009-03-24	Phase 2
====Immunosuppressive therapy====
*Methotrexate (MTX) 10 mg/m2/day PO in divided doses on days 1, 8, 15, 22
**Details not described in ECOG E5998; Lamy et al. 2011 divides the 10 mg/m2 methotrexate dose into 5 mg/m2 in the morning and 5 mg/m2 in the evening
*Prednisone (Sterapred) 1 mg/kg PO once per day for 30 days, then tapered off over 24 days (details not described)
28-day cycles
====Subsequent treatment====
*ECOG E5998, patients with PR: continue up to one year
*ECOG E5998, patients with CR: continue for one month past documented CR
*ECOG E5998, non-responders: Salvage cyclophosphamide & prednisone
===References===
# ECOG E5998: Loughran TP Jr, Zickl L, Olson TL, Wang V, Zhang D, Rajala HL, Hasanali Z, Bennett JM, Lazarus HM, Litzow MR, Evens AM, Mustjoki S, Tallman MS. Immunosuppressive therapy of LGL leukemia: prospective multicenter phase II study by the Eastern Cooperative Oncology Group (E5998). Leukemia. 2015 Apr;29(4):886-94. Epub 2014 Sep 13. link to original article dosing details in manuscript have been reviewed by our editors link to PMC article EuroPMC
=Relapsed or refractory=
==Cyclophosphamide & Prednisone==
===Regimen===
Study 	Evidence	Efficacy
Loughran et al. 2014 (ECOG E5998)	Phase 2 	ORR: 64% (95% CI, 35-87)
Note: Patients with PR continue up to one year; patients with CR continue for one month past documented CR.
====Preceding treatment====
*Upfront methotrexate & prednisone, with treatment failure
====Immunosuppressive therapy====
*Cyclophosphamide (Cytoxan) 100 mg PO once per day on days 1 to 28
*Prednisone (Sterapred) 1 mg/kg PO once per day for 30 days, then tapered off over 24 days (details not described)
28-day cycles (see note)
===References===
# ECOG E5998: Loughran TP Jr, Zickl L, Olson TL, Wang V, Zhang D, Rajala HL, Hasanali Z, Bennett JM, Lazarus HM, Litzow MR, Evens AM, Mustjoki S, Tallman MS. Immunosuppressive therapy of LGL leukemia: prospective multicenter phase II study by the Eastern Cooperative Oncology Group (E5998). Leukemia. 2015 Apr;29(4):886-94. Epub 2014 Sep 13. link to original article dosing details in manuscript have been reviewed by our editors link to PMC article EuroPMC
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