title: Primary cutaneous anaplastic large-cell lymphoma presenting with hemophagocytic syndrome: a case report and review of the literature.
abstract: Primary cutaneous anaplastic large-cell lymphoma (C-ALCL) is a rare entity of lymphoma. We report a case of C-ALCL presenting with hemophagocytic syndrome and skin lesion with giant ulcer. Histopathological examination of the skin biopsy specimens showed non-epidermotropic infiltrates with cohesive sheets of large tumor cells. The tumor cells showed CD4-, CD8+, CD30+, CD56-, ALK-, TIA-1+, and granzyme B+. C-ALCL is generally a disorder that progresses slowly and has a good prognosis. Manifestation of a giant ulcer and hemophagocytic syndrome, such as in the present case, is rare.
authors.affiliation: Department of Hematology, Comprehensive Cancer Center, International Medical Center, Saitama Medical University, 1397-1 Yamane, Hidaka, Saitama 350-1298, Japan.
authors.forename: Yoko
authors.initials: Y
authors.lastname: Shimizu
authors.forename: Ken
authors.initials: K
authors.lastname: Tanae
authors.forename: Naoki
authors.initials: N
authors.lastname: Takahashi
authors.forename: Mika
authors.initials: M
authors.lastname: Kohri
authors.forename: Eiichi
authors.initials: E
authors.lastname: Arai
authors.forename: Masami
authors.initials: M
authors.lastname: Bessho
authors.forename: Nozomi
authors.initials: N
authors.lastname: Niitsu
mesh_terms: D000328:Adult; D018572:Disease-Free Survival; D006801:Humans; D007150:Immunohistochemistry; D051359:Lymphohistiocytosis, Hemophagocytic / Q000209:etiology* / Q000532:radiotherapy; D017728:Lymphoma, Large-Cell, Anaplastic / Q000150:complications* / Q000532:radiotherapy; D008297:Male; D009361:Neoplasm Invasiveness; D012878:Skin Neoplasms / Q000209:etiology / Q000473:pathology; D012883:Skin Ulcer / Q000209:etiology*