Variations in clinical presentation, frequency of hemophagocytosis and clinical behavior of intravascular lymphoma diagnosed in different geographical regions.
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.
Ferreri
Andrés J M
AJ
Medical Oncology Unit, Department of Oncology, San Raffaele Scientific Institute, Milan, Italy. andres.ferreri@hsr
Dognini
Giuseppina P
GP
Campo
Elías
E
Willemze
Rein
R
Seymour
John F
JF
Bairey
Osnat
O
Martelli
Maurizio
M
De Renz
Amalia O
AO
Doglioni
Claudio
C
Montalbán
Carlos
C
Tedeschi
Alberto
A
Pavlovsky
Astrid
A
Morgan
Sue
S
Uziel
Lilj
L
Ferracci
Massimo
M
Ascani
Stefano
S
Gianelli
Umberto
U
Patriarca
Carlo
C
Facchetti
Fabio
F
Dalla Libera
Alessio
A
Pertoldi
Barbara
B
Horváth
Barbara
B
Szomor
Arpad
A
Zucca
Emanuele
E
Cavalli
Franco
F
Ponzoni
Maurilio
M
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
D018943:Anthracyclines