NHL: Primary Cutaneous B-Cell Lymphoma
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NON-HODGKIN LYMPHOMA TREATMENT REGIMENS: Primary Cutaneous B-Cell Lymphoma |
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Clinical Trials: The NCCN recommends cancer patient participation in clinical trials as the gold standard for treatment. |
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Cancer therapy selection, dosing, administration, and the management of related adverse events can be a complex process that should be handled by an experienced healthcare team. Clinicians must choose and verify treatment options based on the individual patient; drug dose modifications and supportive care interventions should be administered accordingly. The non-hodgkin lymphoma (primary cutaneous b-cell lymphoma) cancer treatment regimens below may include both U.S. Food and Drug Administration-approved and unapproved indications/regimens. These non-hodgkin lymphoma (primary cutaneous b-cell lymphoma) cancer treatment regimens are provided only to supplement the latest treatment strategies. These Cancer Treatment Guidelines are a work in progress that may be refined as often as new significant data becomes available. The NCCN Guidelines® are a consensus statement of its authors regarding their views of currently accepted approaches to treatment. Any clinician seeking to apply or consult any NCCN Guidelines® is expected to use independent medical judgment in the context of individual clinical circumstances to determine any patient’s care or treatment. The National Comprehensive Cancer Network makes no warranties of any kind whatsoever regarding their content, use, or application and disclaims any responsibility for their application or use in any way. |
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Primary Cutaneous Marginal Zone Lymphoma or Follicle Center Lymphoma Initial Therapy1 |
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Note: All recommendations are Category 2A unless otherwise indicated. |
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Solitary/Regional T1-2a |
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REGIMEN |
DOSING |
Topicals2–4 |
Topical nitrogen mustard Day 1–15: Topical clobetasol propionate 0.05% cream once daily. OR Day 1–15: Mechlorethamine 0.02% aqueous solution once daily. Topical bexarotene Topical bexarotene gel applied BID until lesions disappear, then use on a needed basis (use with intralesional triamcinolone and interferon alfa). Topical imiquimod Imiquimod 5% cream daily until an inflammatory response develops, then apply to all areas 2–4 times weekly until all inflammation had subsided fully. Then, maintain treatment for another 2 months. Treat for a total of 8 months. |
Intralesional steroids3 |
Triamcinolone 20mg/mL injected into lesions once monthly until lesions are gone or no further improvement is noted (use with interferon-alfa and topical bexarotene gel). OR Interferon-alfa 5 million units SC/IM every 4 weeks for 5 months. |
Generalized Disease (Skin Only), T3bc |
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Rituximab5–8 |
Rituximab 375mg/m2 IV once weekly for 4–8 weeks. OR Rituximab 3mL of stem solution (10mg/mL) injection into the stigmatizing nodules 3 times a week every 28 days for 6 months. |
Topicals2–4 |
Topical nitrogen mustard Day 1–15: Topical clobetasol propionate 0.05% cream once daily. OR Day 1–15: Mechlorethamine 0.02% aqueous solution once daily. Topical bexarotene Topical bexarotene gel applied BID until lesions disappear, then use on an a needed basis (use with intralesional triamcinolone and interferon alfa). Topical imiquimod Imiquimod 5% cream daily until an inflammatory response develops, then apply to all areas 2–4 times weekly until all inflammation subsides fully. Then, maintain treatment for another 2 months. Treat for a total of 8 months. |
Intralesional steroids3 |
Triamcinolone 20mg/mL injected into lesions once monthly until lesions are gone or no further improvement is noted (use with interferon-alfa and topical bexarotene gel). OR Interferon-alfa 5 million units SC/IM every 4 weeks for 5 months. |
Extracutaneous Disease |
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Manage as per Follicular Lymphoma (Grade 1–2) Stage I, II. |
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Primary Cutaneous Marginal Zone Lymphoma or Follicle Center Lymphoma Relapsed or Progressive Disease1 |
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Solitary/Regional T1-2a |
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Topicals2–4 |
Topical nitrogen mustard Day 1–15: Topical clobetasol propionate 0.05% cream once daily. OR Day 1–15: Mechlorethamine 0.02% aqueous solution once daily. Topical bexarotene Topical bexarotene gel applied BID until lesions disappear, then use on an a needed basis (use with intralesional triamcinolone and interferon-alfa) Topical imiquimod Imiquimod 5% cream daily until an inflammatory response develops, then apply to all areas 2–4 times weekly until all inflammation had subsided fully. Then, maintain treatment for another 2 months. Treat for a total of 8 months. |
Intralesional steroids3 |
Triamcinolone 20mg/mL injected into lesions once monthly until lesions are gone or no further improvement is noted (use with interferon-alfa and topical bexarotene gel). OR Interferon-alfa 5 million units SC/IM every 4 weeks for 5 months. |
Generalized Disease (Skin Only), T3bc |
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Rituximab5–8 |
Rituximab 375mg/m2 IV once weekly for 4–8 weeks. OR Rituximab 3mL of stem solution (10mg/mL) injection into the stigmatizing nodules 3 times a week every 28 days for 6 months. |
Topicals2–4 |
Topical nitrogen mustard Day 1–15: Topical clobetasol propionate 0.05% cream once daily. OR Day 1–15: Mechlorethamine 0.02% aqueous solution once daily. Topical bexarotene Topical bexarotene gel applied BID until lesions disappear, then use on an a needed basis (use with intralesional triamcinolone and interferon-alfa). Topical imiquimod Imiquimod 5% cream daily until an inflammatory response develops, then apply to all areas 2–4 times weekly until all inflammation had subsided fully. Then, maintain treatment for another 2 months. Treat for a total of 8 months. |
Intralesional steroids3 |
Triamcinolone 20mg/mL injected into lesions once monthly until lesions are gone or no further improvement is noted (use with interferon-alfa and topical bexarotene gel). OR Interferon-alfa 5 million units SC/IM every 4 weeks for 5 months. |
Extracutaneous Disease |
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Manage as per Follicular Lymphoma (Grade 1–2) Stage I, II. |
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Primary Cutaneous Diffuse Large B-Cell Lymphoma, Leg Type1 |
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Solitary Regional, T1-2de |
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RCHOP + local RT9 |
Day 0: Rituximab 375mg/m2 IV Day 1: Cyclophosphamide 750mg/m2 IV Day 1: Doxorubicin 50mg/m2 IV Day 1: Vincristine 1.4mg/m2 (max 2mg) IV Days 1–5: Prednisone 100mg/m2 orally daily. Repeat every 3 weeks for 6–8 cycles (6 cycles if complete response is achieved after 4 cycles, all others received 8 cycles). Long-term interferon-alfa maintenance initiated at a dose of 3 х 5 million U/week and reduced according to observed adverse effects. Interferon-alfa maintenance therapy was given until lymphoma progression or the development of intolerable adverse effects. |
Generalized Disease (Skin Only), T3ef |
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RCHOP ± local RT9 |
Day 0: Rituximab 375mg/m2 IV Day 1: Cyclophosphamide 750mg/m2 IV Day 1: Doxorubicin 50mg/m2 IV Day 1: Vincristine 1.4mg/m2 (max 2mg) IV Days 1–5: Prednisone 100mg/m2 orally daily. Repeat every 3 weeks for 6–8 cycles (6 cycles if complete response is achieved after 4 cycles, all others received 8 cycles). |
Extracutaneous Disease |
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Manage as per Diffuse Large B-Cell Lymphoma induction therapy. |
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a Local RT is the preferred initial treatment, but not necessarily the preferred treatment for relapse. When RT or surgical treatment is neither feasible nor desired, observation may be appropriate. b In rare circumstances for very extensive or refractory disease, other combination chemotherapy regimens used for the treatment of follicular lymphoma (grade 1-2) stage I, II are used. c Treatment may consist of local radiotherapy for palliation of symptoms or observation d Treatment may consist of local radiotherapy or clinical trial e For patients who cannot tolerate anthracyclines, see first-line therapy for patients with diffuse large B-cell lymphoma who have poor left ventricular function. f Treatment may consist of clinical trial |
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References |
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1. Referenced with permission from the NCCN Clinical Practice Guidelines in Oncology (NCCNGuidelines®) for Non-Hodgkin’s Lymphomas. V3.2016. Available at: https://www.nccn.org. Accessed September 19, 2016. 2. Bachmeyer C, Orlandini V, Aractingi S. Topical mechlorethamine and clobetasol in multifocal primary cutaneous marginal zone-B cell lymphoma. BrJDermatol. 2006;154:1207–1209. 3. Trent JT, Romanelli P, Kerdel FA. Topical Targretin and Intralesional Interferon Alfa for Cutaneous Lymphoma of the Scalp. Arch Dermatol. 2002;138:1421–1423. 4. Stavrakoglou A, Brown VL, Coutts I. Successful treatment of primary cutaneous follicle centre lymphoma with topical 5% imiquimod. Br J Dermatol. 2007;157:620–622. 5. Morales AV, Advani R, Horwitz SM, et al. Indolent primary cutaneous B-cell lymphoma: experience using systemic rituximab. J Am Acad Dermatol. 2008;59:953–957. 6. Valencak J, Weihsengruber F, Rappersberger K, et al. Rituximab monotherapy for primary cutaneous B-cell lymphoma: Response and follow-up in 16 patients. Ann Oncol. 2009;20:326–330. 7. Senff NJ, Noordijk EM, Kim YH, et al. European Organization for Research and Treatment of Cancer and International Society for Cutaneous Lymphoma consensus recommendations for the management of cutaneous B-cell lymphomas. Blood. 2008;112:1600–1609. 8. Heinzerling L, Dummer R, Kempf W, Schmid MH, Burg G. Intralesional therapy with anti-CD20 monoclonal antibody rituximab in primary cutaneous B-cell lymphoma. Arch Dermatol. 2000;136:374–378. 9. Senff NJ, Noordijk EM, Kim YH, et al. European Organization for Research and Treatment of Cancer International Society of Cutaneous Lymphoma consensus recommendations for the management of cutaneous B-cell lymphomas. Blood. 2008;112:1600–1609. |
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(Revised 10/2016) © 2016 by Haymarket Media, Inc. |