Description

Primary cutaneous diffuse large B-cell lymphoma, leg type (PCLBCL-LT) is an aggressive form of cutaneous lymphoma.


 

Clinical features:

(1) affects middle aged to elderly adults (mean age 76 years)

(2) can affect any skin area but typically found on the legs

(3) relapses are common

(4) may show extracutaneous involvement in lymph nodes and/or viscera

(5) may show a poor clinical response to therapy

 

Pathologic features:

(1) diffuse large B-cell lymphoma with predominance of large cells with round nuclei (both centroblasts and immunoblasts)

(2) usually bcl-2 positive

 

Poor prognostic features:

(1) multiple skin lesions (2 or more) at diagnosis

(2) leg involvement

 

Additional factors affecting prognosis:

(1) age > 75 years (page 1147)

(2) poor response to therapy (complete response alone not significant, Table 1)

 

Short term prognosis may be improved if the patient is treated with rituximab and anthracyclines.

 


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