Huidlymfomen

Inhoudsopgave

B cell lymphoma in the skin
no evidence of extracutaneous disease at the time of diagnosis and after the completion of an initial staging evaluation

typically firm, erythematous, painless, nonpruritic papules, nodules or plaques with a predilection for the head, neck, and trunk; generally smooth and do not ulcerate
PA: epidermis-sparing B cell infiltrate comprised of centrocytes and centroblasts with a follicular or diffuse pattern, B cell lineage is confirmed by the presence of CD20 or CD79a and the absence of CD3

+/- 30% of patients initially treated with radiation therapy will relapse. Most relapses are confined to the skin and are treated with the same modalities as initial therapy. (See 'Treatment at relapse' above.)

PCFCL rare disorder, large trials are lacking
focal disease (a solitary lesion or lesions that can be contained in one radiation field):
- radiation therapy, 24 to 30 Gy to a field that includes a margin of at least 1 to 1.5 cm of uninvolved skin
- surgical excision or observation acceptable alternative for lesions that are not amenable to radiation therapy (eg, in a location at which radiation toxicity is increased), choice depends upon the morbidity associated with excision
a few scattered lesions not able to fit in one radiation field: low-dose radiation therapy to all visible lesions or initial observation
- choice depends upon the sites involved and expected toxicity of radiation to the area and the presence or absence of symptoms
extensive diffuse disease: single agent rituximab
extremely extensive symptomatic disease or those who fail to respond to initial rituximab: combination chemotherapy