title: Autopsy case of CD4/CD8 cutaneous T-cell lymphoma presenting disseminated pagetoid reticulosis with aggressive granulomatous invasion to the lungs and pancreas.
abstract: Pagetoid reticulosis is a rare cutaneous T-cell lymphoma with striking epidermotropism similar to that present in Paget's disease. There are two forms of pagetoid reticulosis: localized and disseminated. Reported herein is an autopsy case of disseminated pagetoid reticulosis with CD4(-)/CD8(-) phenotype T cells and massive invasion of the lungs and pancreas. The abnormal cells in the epidermis expressed a protein derived from a rearranged T-cell receptor beta gene, and this feature was used to confirm the monoclonality of these cells by polymerase chain reaction. At present, the World Health Organization (WHO) classification system considers pagetoid reticulosis to be an indolent form of primary cutaneous T-cell lymphoma and a variant of mycosis fungoides/Sezary syndrome with prominent epidermotropism. Some differences have been observed between pagetoid reticulosis and mycosis fungoides in terms of clinical course, tumor cell phenotype, and genetic findings; and these differences are highlighted in the present case. The relation between disseminated pagetoid reticulosis, CD4(-)/CD8(-) cutaneous T-cell lymphoma, and gammadelta T-cell lymphoma, including whether pagetoid reticulosis is a variant of mycosis fungoides, remains unclear.
authors.affiliation: Second Department of Pathology, Showa University School of Medicine, 1-5-8 Hatanodai, Shinagawa-ku, Tokyo 142-8555, Japan. eshiozawa@aol.com
authors.forename: Eisuke
authors.initials: E
authors.lastname: Shiozawa
authors.forename: Akira
authors.initials: A
authors.lastname: Shiokawa
authors.forename: Mari
authors.initials: M
authors.lastname: Shibata
authors.forename: Tokio
authors.initials: T
authors.lastname: Nakada
authors.forename: Toshiko
authors.initials: T
authors.lastname: Yamochi-Onizuka
authors.forename: Bungo
authors.initials: B
authors.lastname: Saito
authors.forename: Emi
authors.initials: E
authors.lastname: Takaba
authors.forename: Masafumi
authors.initials: M
authors.lastname: Iijima
authors.forename: Masafumi
authors.initials: M
authors.lastname: Takimoto
authors.forename: Hidekazu
authors.initials: H
authors.lastname: Ota
mesh_terms: D001344:Autopsy; D015704:CD4 Antigens / Q000378:metabolism*; D016827:CD8 Antigens / Q000378:metabolism*; D005260:Female; D006099:Granuloma; D006801:Humans; D007150:Immunohistochemistry; D017403:In Situ Hybridization; D008175:Lung Neoplasms / Q000473:pathology; D016410:Lymphoma, T-Cell, Cutaneous / Q000145:classification / Q000378:metabolism / Q000473:pathology*; D008875:Middle Aged; D010190:Pancreatic Neoplasms / Q000473:pathology*; D016133:Polymerase Chain Reaction; D012878:Skin Neoplasms / Q000145:classification / Q000378:metabolism / Q000473:pathology*
chemical_list: D015704:CD4 Antigens; D016827:CD8 Antigens