title: Secondary ALK negative anaplastic large cell lymphoma in a patient with lymphomatoid papulosis of 40 years duration.
abstract: A 49 year-old man presented to our clinic. He had a history of lymphomatoid papulosis since childhood. At age 44, regional lymph node manifestation of anaplastic lymphoma kinase (ALK) anaplastic large cell lymphoma (ALCL) developed. Chemotherapy resulted in complete remission of the lymphadenopathy. Four years later, systemic relapse was detected which was refractory to therapy. Histology and immunohistochemistry showed congruent characteristics of multiple skin and lymph node biopsies: diffuse mixed infiltrate with large, anaplastic CD30 cells. Immunophenotype and microscopic morphology suggested a common origin of the different manifestations-however, this could not be proven due to lack of T-cell receptor (TCR) gamma gene rearrangement in most of the samples. The diagnosis of ALK-negative systemic ALCL with cutaneous symptoms was set up at the second flare up, however, the possibility of primary cutaneous ALCL was not excluded steadily. Lymphomatoid papulosis, primary cutaneous ALCL, and systemic ALK ALCL are 3 different entities but the separation of them cannot be solved without distinctive diagnostic tools.
authors.affiliation: Department of Dermatology, Venereology and Dermatooncology, Semmelweis University, Budapest, Hungary. marmar@bor.sote.hu
authors.forename: Márta
authors.initials: M
authors.lastname: Marschalkó
authors.forename: Nóra
authors.initials: N
authors.lastname: Eros
authors.forename: Péter
authors.initials: P
authors.lastname: Holló
authors.forename: Judit
authors.initials: J
authors.lastname: Hársing
authors.forename: Gyula
authors.initials: G
authors.lastname: Bottlik
authors.forename: Arpád
authors.initials: A
authors.lastname: Bátai
authors.forename: Zoltán
authors.initials: Z
authors.lastname: Csukly
authors.forename: Tamás
authors.initials: T
authors.lastname: Masszi
authors.forename: Zoltán
authors.initials: Z
authors.lastname: Szentirmai
authors.forename: János
authors.initials: J
authors.lastname: Fodor
authors.forename: Sarolta
authors.initials: S
authors.lastname: Kárpáti
authors.forename: András
authors.initials: A
authors.lastname: Matolcsy
authors.forename: Judit
authors.initials: J
authors.lastname: Csomor
mesh_terms: D000077548:Anaplastic Lymphoma Kinase; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D017809:Fatal Outcome; D006801:Humans; D017728:Lymphoma, Large-Cell, Anaplastic / Q000188:drug therapy / Q000473:pathology*; D017731:Lymphomatoid Papulosis / Q000188:drug therapy / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D016609:Neoplasms, Second Primary / Q000188:drug therapy / Q000473:pathology*; D011505:Protein-Tyrosine Kinases / Q000378:metabolism; D020794:Receptor Protein-Tyrosine Kinases; D012878:Skin Neoplasms / Q000188:drug therapy / Q000473:pathology*
chemical_list: C000626173:ALK protein, human; D000077548:Anaplastic Lymphoma Kinase; D011505:Protein-Tyrosine Kinases; D020794:Receptor Protein-Tyrosine Kinases