title: A case of true malignant histiocytosis: identification of histiocytic origin with use of immunohistochemical and immunocytogenetic methods.
abstract: We report here an autopsy case of true malignant histiocytosis. The patient was a 67-year-old woman who exhibited fever, wasting, hepatosplenomegaly, and progressive pancytopenia. The bone marrow aspiration disclosed hemophagocytosing cells, which resembled histiocytes. The molecular analysis did not show the clonal gene rearrangement of T-cell receptor or immunoglobulin heavy chain. Although the patient had been started on methylprednisolone pulse therapy and chemotherapy with etoposide, she died from cerebral hemorrhage. The autopsy specimens of spleen and liver showed extensive infiltration of atypical cells, for which histiocytic origin was identified with an immunohistochemical method using monoclonal antibodies against CD11c, CD68, macrophage colony-stimulating factor (M-CSF), M-CSF receptor, lysozyme, antitrypsin and alpha1-antichymotrypsin. Recent investigations have disclosed that in most cases diagnosed as malignant histiocytosis, hemophagocytosis was reactive and not evoked by histiocytic malignancy. True malignant histiocytosis, for which histiocytic origin is confirmed, is extremely rare.
authors.affiliation: Department of Internal Medicine (Section 4), Sapporo Medical University, School of Medicine, South-1, West-16, Chuo-ku, Japan. ben_sato@hotmail.com
authors.forename: T
authors.initials: T
authors.lastname: Sato
authors.forename: T
authors.initials: T
authors.lastname: Terui
authors.forename: K
authors.initials: K
authors.lastname: Kogawa
authors.forename: Y
authors.initials: Y
authors.lastname: Nagaoka
authors.forename: Y
authors.initials: Y
authors.lastname: Sato
authors.forename: S
authors.initials: S
authors.lastname: Iyama
authors.forename: K
authors.initials: K
authors.lastname: Takada
authors.forename: S
authors.initials: S
authors.lastname: Hagiwara
authors.forename: S
authors.initials: S
authors.lastname: Takahashi
authors.forename: T
authors.initials: T
authors.lastname: Oku
authors.forename: T
authors.initials: T
authors.lastname: Matsunaga
authors.forename: M
authors.initials: M
authors.lastname: Takahashi
authors.forename: J
authors.initials: J
authors.lastname: Kato
authors.forename: S
authors.initials: S
authors.lastname: Sakamaki
authors.forename: T
authors.initials: T
authors.lastname: Torigoe
authors.forename: N
authors.initials: N
authors.lastname: Sato
authors.forename: M
authors.initials: M
authors.lastname: Hirayama
authors.forename: Y
authors.initials: Y
authors.lastname: Niitsu
mesh_terms: D000368:Aged; D000911:Antibodies, Monoclonal; D001853:Bone Marrow / Q000473:pathology; D002460:Cell Line; D020732:Cytogenetic Analysis; D017809:Fatal Outcome; D005260:Female; D054747:Histiocytic Sarcoma / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry / Q000379:methods*; D008099:Liver / Q000473:pathology; D013154:Spleen / Q000473:pathology
chemical_list: D000911:Antibodies, Monoclonal