Table 5

Recommendations for the treatment (standard therapies) of CD30+ LPD

PCALCL
LYP
Solitary or grouped lesion(s)Multifocal lesionsExtracutaneous spreadLocalized/regional or few lesions*Numerous and/or generalized lesions
SE Methotrexate Single or multiagent chemotherapy Observation Observation 
RT Alternatives: retinoids, interferon  Phototherapy§ Phototherapy§ 
   Topical steroids Methotrexate 
    Topical steroids 
    Alternatives: retinoids, interferon 
PCALCL
LYP
Solitary or grouped lesion(s)Multifocal lesionsExtracutaneous spreadLocalized/regional or few lesions*Numerous and/or generalized lesions
SE Methotrexate Single or multiagent chemotherapy Observation Observation 
RT Alternatives: retinoids, interferon  Phototherapy§ Phototherapy§ 
   Topical steroids Methotrexate 
    Topical steroids 
    Alternatives: retinoids, interferon 
*

For larger (defined as > 2 cm in diameter) and persistent (defined as duration of lesion > 12 weeks) lesions, SE or RT may represent alternatives.

These therapies are of low-level evidence other than expert opinion.

In cases of skin and only local node involvement in PCALCL, one could consider addition of local nodal radiation

§

PUVA is best documented. Alternatively, treatment with other types of phototherapy (eg, UVB-narrow band) can be tried (evidence level 5).

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