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Abstract

Lymphomatoid papulosis (LyP) is a low grade primary cutaneous indolent T cell lymphoproliferative disorder characterized by recurrent papules/nodules, potentially associated with lymphoma, preceding, concurrent or following the lymphoma diagnosis. We report two cases of LyP arising after nodal ALCL remission. Case 1 was a 46-year-old male with extracutaneous ALK-negative ALCL (left groin lymph node, tonsil) achieved complete remission after chemotherapy and bone marrow transplantation (BMT). Prior to BMT and following his BMT, the patient developed spontaneous regressing lesions, each < 3 cm. Biopsies from both sites revealed dermal and subcutaneous CD8+, CD30+, atypical large T lymphocytes and small atypical epidermotropic T cells diagnosed as LyP with a 6p25.3 rearrangement. The patient continues to be in remission from his ALCL (follow-up duration after BMT: 2 years and 7 months). Case 2 is a 79-year-old female with extracutaneous ALK-negative ALCL, localized to the left axillary lymph node, achieving remission after chemotherapy. She developed papulonodules on her neck 6 months later. Biopsies showed CD4+, CD30+ atypical large T lymphocytes infiltrating the dermis compatible with LyP.  In case 2, identical T-cell clones were identified in nodal and cutaneous lesions though the two forms of LPD are different categorically and prognostically (follow-up duration since remission: 3 months). There are two priors of treated ALCL followed by recurrent LyP without lymphoma recurrence. While therapy with chemotherapy and BMT can eradicate the lymphoma it may not eliminate the same neoplastic clone that manifests uniquely in the skin as recurrent LYP.