Addictive response of primary cutaneous diffuse large B cell lymphoma leg type to low-dose ibrutinib
Primary cutaneous diffuse large B cell lymphoma leg type (PCLBCL-LT) is a rare malignancy occurring predominantly below the knee, but may involve other cutaneous and extra-cutaneous sites . Lymphoma cells typically express CD20, CD79A, BCL2, BCL6, MUM-1, and FOXP1, but not CD10 [1, 2]. Gene expression of PCLBCL-LT resembled activated B cell diffuse large B cell lymphoma (ABC-DLBCL). Consequently, genes involved in NF-κB activation were frequently mutated , with the MYD88 L265P mutation most prevalent, found in 60–80% of cases .
Outcome of PCLBCL-LT is unfavorable. Pre-rituximab, the 5-year overall survival (OS) was about 50%, with few survivals beyond 10 years [2, 4]. With rituximab-containing regimens, 5-year survivals were improved to about 75% . Poor risks affecting 5-year OS included disseminated disease (solitary versus disseminated: 70% versus 0%) , BCL2 expression (positive versus negative: 41% versus 89%) , and MYD88status (L265P versus wildtype: 41% versus...
A. Pang: performed the investigations, wrote and approved the manuscript.
R. Au-Yeung: performed the pathologic analysis, wrote and approved the manuscript.
R. Leung: performed the pathologic analysis, wrote and approved the manuscript.
Y.L. Kwong: treated the patient, wrote and approved the manuscript.
Compliance with ethical standards
Conflict of interest
The authors declare that they have no conflict of interest.
Patient gave informed consent to treatment.
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