title: Variations in clinical presentation, frequency of hemophagocytosis and clinical behavior of intravascular lymphoma diagnosed in different geographical regions.
abstract: OBJECTIVE
  This study explored variations in the clinical manifestations of intravascular lymphoma (IVL) on the bases of the association with hemophagocytosis and the country where the diagnosis was made.
  
  
  METHODS
  The clinical features of 50 Western patients with IVL were compared with those of 123 patients with IVL diagnosed in Eastern countries (87 diagnosed in Japan and 36 in other Asian countries), previously reported in English literature, and collected by an electronic bibliographic search.
  
  
  RESULTS
  Hemophagocytosis was absent in Western patients, but reported in 38 (44%) Japanese patients (p=0.00001) and in seven (19%) patients from other Asian countries (p=0.002). No clinical differences were evident between patients with hemophagocytosis-negative IVL diagnosed in Western countries, Japan and other Asian Countries. Conversely, Japanese and non-Japanese patients with hemophagocytosis-related IVL more frequently had stage IV disease, fever, hepato-splenic involvement, marrow infiltration, dyspnea, anemia, and thrombocytopenia, and rarely exhibited cutaneous or central nervous system involvement. Lymph node and peripheral blood involvement was uncommon in all subgroups. In Western patients, anthracycline-based chemotherapy was associated with a 52% remission rate, and a 2-year overall survival of 46%.
  
  
  CONCLUSIONS
  The clinical features of IVL vary according to the association with hemophagocytosis, regardless of the country in which the diagnosis is made. Western, Japanese and other Asian patients with hemophagocytosis-negative IVL display similar clinical characteristics and should be considered as having classical IVL. Patients with hemophagocytosis-related IVL show significantly different clinical features. Both forms have a poor prognosis. Extensive molecular studies are needed to explore whether these clinical differences might reflect discordant biological entities within IVL.
authors.affiliation: Medical Oncology Unit, Department of Oncology, San Raffaele Scientific Institute, Milan, Italy. andres.ferreri@hsr
authors.forename: Andrés J M
authors.initials: AJ
authors.lastname: Ferreri
authors.forename: Giuseppina P
authors.initials: GP
authors.lastname: Dognini
authors.forename: Elías
authors.initials: E
authors.lastname: Campo
authors.forename: Rein
authors.initials: R
authors.lastname: Willemze
authors.forename: John F
authors.initials: JF
authors.lastname: Seymour
authors.forename: Osnat
authors.initials: O
authors.lastname: Bairey
authors.forename: Maurizio
authors.initials: M
authors.lastname: Martelli
authors.forename: Amalia O
authors.initials: AO
authors.lastname: De Renz
authors.forename: Claudio
authors.initials: C
authors.lastname: Doglioni
authors.forename: Carlos
authors.initials: C
authors.lastname: Montalbán
authors.forename: Alberto
authors.initials: A
authors.lastname: Tedeschi
authors.forename: Astrid
authors.initials: A
authors.lastname: Pavlovsky
authors.forename: Sue
authors.initials: S
authors.lastname: Morgan
authors.forename: Lilj
authors.initials: L
authors.lastname: Uziel
authors.forename: Massimo
authors.initials: M
authors.lastname: Ferracci
authors.forename: Stefano
authors.initials: S
authors.lastname: Ascani
authors.forename: Umberto
authors.initials: U
authors.lastname: Gianelli
authors.forename: Carlo
authors.initials: C
authors.lastname: Patriarca
authors.forename: Fabio
authors.initials: F
authors.lastname: Facchetti
authors.forename: Alessio
authors.initials: A
authors.lastname: Dalla Libera
authors.forename: Barbara
authors.initials: B
authors.lastname: Pertoldi
authors.forename: Barbara
authors.initials: B
authors.lastname: Horváth
authors.forename: Arpad
authors.initials: A
authors.lastname: Szomor
authors.forename: Emanuele
authors.initials: E
authors.lastname: Zucca
authors.forename: Franco
authors.initials: F
authors.lastname: Cavalli
authors.forename: Maurilio
authors.initials: M
authors.lastname: Ponzoni
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D018943:Anthracyclines / Q000627:therapeutic use; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001208:Asia / Q000453:epidemiology; D044466:Asian People / Q000706:statistics & numerical data; D001932:Brain Neoplasms / Q000453:epidemiology / Q000208:ethnology / Q000473:pathology; D018450:Disease Progression; D005060:Europe / Q000453:epidemiology; D005260:Female; D005500:Follow-Up Studies; D005544:Forecasting; D006801:Humans; D007564:Japan / Q000208:ethnology; D051359:Lymphohistiocytosis, Hemophagocytic / Q000175:diagnosis / Q000453:epidemiology* / Q000208:ethnology / Q000209:etiology; D016403:Lymphoma, Large B-Cell, Diffuse / Q000145:classification / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology* / Q000208:ethnology / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009928:Organ Specificity; D010641:Phenotype; D011379:Prognosis; D012189:Retrospective Studies; D012878:Skin Neoplasms / Q000453:epidemiology / Q000208:ethnology / Q000473:pathology; D011795:Surveys and Questionnaires; D016896:Treatment Outcome; D019043:Vascular Neoplasms / Q000145:classification / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology* / Q000208:ethnology / Q000473:pathology; D044465:White People / Q000706:statistics & numerical data
chemical_list: D018943:Anthracyclines