Haematologica, Vol 92, Issue 4, 486-492 doi:10.3324/haematol.10829
Copyright © 2007 by Ferrata Storti Foundation

This Article Full Text Full Text (PDF) Supplementary Data Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Ferreri, A. J.M. Search for Related Content PubMed PubMed Citation Articles by Ferreri, A. J.M. Related Collections Related Article

Malignant Lymphomas

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

Andrés J.M. Ferreri, Giuseppina P. Dognini, Elías Campo, Rein Willemze, John F. Seymour, Osnat Bairey, Maurizio Martelli, Amalia De Renzo, Claudio Doglioni, Carlos Montalbán, Alberto Tedeschi, Astrid Pavlovsky, Sue Morgan, Lilj Uziel, Massimo Ferracci, Stefano Ascani, Umberto Gianelli, Carlo Patriarca, Fabio Facchetti, Alessio Dalla Libera, Barbara Pertoldi, Barbara Horváth, Arpad Szomor, Emanuele Zucca, Franco Cavalli, Maurilio Ponzoni on behalf of the International Extranodal Lymphoma Study Group (IELSG)

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 Sant’Orsola, 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]

This article has been cited by other articles:

				A. J.M. Ferreri, G. P. Dognini, S. Govi, R. Crocchiolo, M. Bouzani, C. R. Bollinger, M. D'Incan, E. Delaporte, M. Hamadani, F. Jardin, et al. Can Rituximab Change the Usually Dismal Prognosis of Patients With Intravascular Large B-Cell Lymphoma? J. Clin. Oncol., November 1, 2008; 26(31): 5134 - 5136. [Full Text] [PDF]