Variations in clinical presentation, frequency of hemophagocytosis and clinical behavior of intravascular lymphoma diagnosed in different geographical regions

Medical Oncology Unit, San Raffaele Scientific Institute, Milan, Italy (AJMF, GPD); Div. of Pathology, Hospital Clínic, Barcelona, Spain (EC); Dutch Cutaneous Lymphoma Group, The Netherlands (RW); Australasian Leukaemia and Lymphoma Group and Univ. of Melbourne, Australia (JFS); Div. Of Hematology,...

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Published in:	Haematologica (Roma) Vol. 92; no. 4; pp. 486 - 492
Main Authors:	Ferreri, Andres J.M, Dognini, Giuseppina P, Campo, Elias, Willemze, Rein, Seymour, John F, Bairey, Osnat, Martelli, Maurizio, De Renzo, Amalia, Doglioni, Claudio, Montalban, Carlos, Tedeschi, Alberto, Pavlovsky, Astrid, Morgan, Sue, Uziel, Lilj, Ferracci, Massimo, Ascani, Stefano, Gianelli, Umberto, Patriarca, Carlo, Facchetti, Fabio, Dalla Libera, Alessio, Pertoldi, Barbara, Horvath, Barbara, Szomor, Arpad, Zucca, Emanuele, Cavalli, Franco, Ponzoni, Maurilio, on behalf of International Extranodal Lymphoma Study Group (IELSG)
Format:	Journal Article
Language:	English
Published:	Pavia Haematologica 01-04-2007 Ferrata Storti Foundation
Subjects:	Adolescent Adult Aged Aged, 80 and over Anthracyclines - therapeutic use Antineoplastic Combined Chemotherapy Protocols - therapeutic use Asia - epidemiology Asian Continental Ancestry Group - statistics & numerical data Biological and medical sciences Brain Neoplasms - epidemiology Brain Neoplasms - ethnology Brain Neoplasms - pathology Disease Progression Europe - epidemiology European Continental Ancestry Group - statistics & numerical data Female Follow-Up Studies Forecasting Hematologic and hematopoietic diseases Humans Japan - ethnology Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis Lymphohistiocytosis, Hemophagocytic - diagnosis Lymphohistiocytosis, Hemophagocytic - epidemiology Lymphohistiocytosis, Hemophagocytic - ethnology Lymphohistiocytosis, Hemophagocytic - etiology Lymphoma, Large B-Cell, Diffuse - classification Lymphoma, Large B-Cell, Diffuse - complications Lymphoma, Large B-Cell, Diffuse - diagnosis Lymphoma, Large B-Cell, Diffuse - drug therapy Lymphoma, Large B-Cell, Diffuse - epidemiology Lymphoma, Large B-Cell, Diffuse - ethnology Lymphoma, Large B-Cell, Diffuse - pathology Male Medical sciences Middle Aged Organ Specificity Phenotype Prognosis Retrospective Studies Skin Neoplasms - epidemiology Skin Neoplasms - ethnology Skin Neoplasms - pathology Surveys and Questionnaires Treatment Outcome Vascular Neoplasms - classification Vascular Neoplasms - complications Vascular Neoplasms - diagnosis Vascular Neoplasms - drug therapy Vascular Neoplasms - epidemiology Vascular Neoplasms - ethnology Vascular Neoplasms - pathology Asia Europe Japan Intravascular lymphoma Hematology Central nervous system Malignant hemopathy Lymphoid neoplasm Histiocytosis Encephalon brain lymphoma Hemophagocytic syndrome hemophagocytosis Lymphoproliferative syndrome Frequency Skin Behavior Diagnosis cutaneous lymphoma
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Summary:	Medical Oncology Unit, San Raffaele Scientific Institute, Milan, Italy (AJMF, GPD); Div. of Pathology, Hospital Clínic, Barcelona, Spain (EC); Dutch Cutaneous Lymphoma Group, The Netherlands (RW); Australasian Leukaemia and Lymphoma Group and Univ. of Melbourne, Australia (JFS); Div. Of Hematology, Rabin Medical Center, Beilinson Hospital, Petah Tiqwa, Israel (OB); Div. of Hematology, Università La Sapienza, Rome, Italy (MM); Div. of Hematology, Università Federico II, Napoli, Italy (ADR); Pathology Unit, San Raffaele Scientific Institute, Milan, Italy (CD, MP); Servicio de Medicina Interna, Hospital Ramón y Cajal, Madrid, Spain (CM); Div. of Internal Medicine, Ospedale Maggiore, Milan, Italy (AT); Div. of Hematology, Fundaleu, Buenos Aires, Argentina (AP); Div. of Hematology, Alfred Hospital, Melbourne, Australia (SM); Div. of Hematology, Ospedale San Paolo, Milan, Italy (LU); Pathology Unit, Ospedale di Belluno, Belluno, Italy (MF); Div. of Pathology, Ospedale SantOrsola, Bologna, Italy (SA); Div. of Pathology, Ospedale San Paolo, Milan, Italy (UG); Div. of Pathology, Ospedale Vizzolo Predabissi, Melegnano, Italy (CP); Div. of Pathology, Spedali Civili di Brescia, Italy (FF); Neurology Division, "Boldrini" Hospital, Thiene, Italy (ADL); Div. of Pathology, Ospedale di Thiene, Italy (BP); Dept. of Dermatovenereology and Dermatooncology, Semmelweis University, Budapest, Hungary (BH); First Dept. Medicine Univ. of Pecs, Pecs, Hungary (AS); Div. of Medical Oncology, Ist. Oncologico Svizzera Italiana, Switzerland (EZ, FC). Correspondence: Andrés JM Ferreri, M.D., Medical Oncology Unit, Department of Oncology, San Raffaele Scientific Institute, via Olgettina 60, 20132, Milan, Italy. E-mail: andres.ferreri{at}hsr.it Background and Objectives: 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. Design and 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%. Interpretation and 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. Key words: intravascular lymphoma, hemophagocytosis, cutaneous lymphoma, brain lymphoma. Related Article Intravascular large B-cell lymphoma: the heterogeneous clinical manifestations of its classical and hemophagocytosis-related forms Shigeo Nakamura, Takuhei Murase, Tomohiro Kinoshita Haematologica 2007 92: 434-436. [Full Text] [PDF]
Bibliography:	ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23
ISSN:	0390-6078 1592-8721
DOI:	10.3324/haematol.10829