title: Excellent Outcome of Immunomodulation or Bruton's Tyrosine Kinase Inhibition in Highly Refractory Primary Cutaneous Diffuse Large B-Cell Lymphoma, Leg Type.
abstract: Primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL-LT) is a rare diffuse large B-cell lymphoma confined to the skin of the legs. The typical presentation is characterized by solitary or multiple growing plaques, usually confined to one leg. We report a case of PCDLBCL-LT of activated B-cell subtype characterized by multiple local relapses in the legs, initially, and systemic relapses about seven years after the diagnosis. Local relapses were sensitive to radiation therapy. Cutaneous and systemic relapses responded well to immunomodulatory therapy with lenalidomide followed by Bruton's tyrosine kinase inhibition with ibrutinib. Ibrutinib is the only treatment that resulted in long-lasting complete remission. Lenalidomide and especially ibrutinib appear to have a significant activity against this lymphoma and should be incorporated in the treatment of this resistant and aggressive lymphoma. To our knowledge, this is the first case of PCDLBCL-LT reported in the literature exhibiting a complete response to ibrutinib.
authors.affiliation: Division of Hematology and Oncology, Mayo Clinic, Jacksonville , FL, USA.
authors.forename: Eva
authors.initials: E
authors.lastname: Gupta
authors.affiliation: Department of Diagnostic Radiology, Mayo Clinic, Jacksonville , FL, USA.
authors.forename: Joseph
authors.initials: J
authors.lastname: Accurso
authors.affiliation: Department of Dermatology, Mayo Clinic, Jacksonville , FL, USA.
authors.forename: Jason
authors.initials: J
authors.lastname: Sluzevich
authors.affiliation: Department of Pathology, Mayo Clinic, Jacksonville , FL, USA.
authors.forename: David M
authors.initials: DM
authors.lastname: Menke
authors.affiliation: Division of Hematology and Oncology, Mayo Clinic, Jacksonville , FL, USA.
authors.forename: Han W
authors.initials: HW
authors.lastname: Tun
keywords: Cutaneous diffuse large B-cell lym-phoma; ibrutinib; lenalidomide