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Chapter 115: Cutaneous Anaplastic Large Cell Lymphoma

Warren H. Chan; Madeleine Duvic

CUTANEOUS ANAPLASTIC LARGE CELL LYMPHOMA

Therapeutic Approach

Primary cutaneous anaplastic large cell lymphomas (PC-ALCL) represent 8% of all cutaneous T-cell lymphomas. PC-ALCL often follows an indolent course with an excellent prognosis, although relapses are common. Therapy is based on whether the patient presents with solitary or grouped lesions, multifocal lesions (20%), or PC-ALCL with extracutaneous, usually regional lymph node, involvement (10%). Surgical excision is used for solitary localized lesions only, while involved site radiation therapy (ISRT) is recommended for localized or grouped lesions. Systemic therapy, beginning with methotrexate, is given for multifocal lesions or PC-ALCL with extracutaneous disease. Relapses are treated with the same initial regimen unless refractory disease or intolerance develop. Treatment for refractory disease is not standardized; in these cases, other systemic therapies, including oral bexarotene and brentuximab vedotin, and referral to clinical trial should be considered. See Table 115-1.

Table 115-1Cutaneous Anaplastic Large Cell Lymphoma Treatment Table
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Table 115-1 Cutaneous Anaplastic Large Cell Lymphoma Treatment Table
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MEDICATION NAME INDICATION MECHANISM OF ACTION DOSING ADVERSE EFFECTS SUGGESTED MONITORING LEVEL OF EVIDENCE (REFERENCE)
Topical Therapy
Imiquimod Localized skin involvement Induces IFN-α, IFNf-γ, and IL-12 5% cream; apply 3×/wk for 6 wk Skin irritation None IV1
Retinoids Localized skin involvement Aids in cellular differentiation, proliferation, and apoptosis Bexarotene gel 1.0%; apply BID Skin irritation None IV2,3
Carmustine Localized skin involvement Alkylating agent 0.2% solution; apply QD Skin irritation (<10%), leukopenia, hyperpigmentation CBC IV4
Corticosteroids Localized or generalized skin involvement Anti-inflammatory Clobetasol 0.05% cream; apply BID Skin irritation, atrophy, striae None IV5
Mechlorethamine hydrochloride (nitrogen mustard) Localized or generalized skin involvement Alkylating agent 0.02% gel; apply QD Contact dermatitis None IV6
Systemic Therapy
Methotrexate Multifocal lesions, ≥N1 Inhibits DNA synthesis, repair, replication; anti-inflammatory 7.5 mg BID 1 d/wk, <50 mg weekly Nausea, stomach upset, headache, fatigue, hepatotoxicity, pulmonary fibrosis, myelosuppression CBC, CMP (every 1-3 mo), pregnancy test, HBV, HCV, CXR, PFT IIA7
Bexarotene Multifocal lesions Induces apoptosis (retinoid X receptor) 225 mg QD; max 300-400 mg QD Weakness, myalgia, arthralgia, headaches, hepatotoxicity, hyperlipidemia, teratogenic, skin irritation, hypothyroidismb CBC, CMP, pregnancy test (monthly), lipids (every 8 wk), free T4/TSH (every 1-2 mo), CK, HBV IV8,9
Brentuximab vedotina Multifocal lesions, ±ISRT or CHP for ≥N1 Anti-CD30 1.8 mg/kg (max dose 180 mg) IV every 3 wk; reduce to 1.2 mg/kg for low disease burden Peripheral neuropathy, PML, neutropenia, nausea, chest pain, DVT, transaminitis, arthralgias, fatigue, skin hypersensitivity rash CBC (prior to each dose), CMP, pregnancy test, HBV IIA9–11
Pralatrexate Multifocal lesions, ≥N1 Inhibits DNA, RNA, protein synthesis 15 mg/m2 IV weekly for 3 wk Mucositis, fatigue, nausea, edema, epistaxis, pyrexia, anorexia, skin toxicity, leukopenia, TLS CBC (weekly), CMP (prior to 1st and 4th doses in each cycle), pregnancy test, HBV IIA12
Interferon Multifocal lesions Th2 to Th1 cytokine profile shift

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